WorldWideScience

Sample records for huntington canyon generating

  1. Public response to the Diablo Canyon Nuclear Generating Station

    International Nuclear Information System (INIS)

    Pijawka, K.D.

    1982-01-01

    The authors examine the nature of the public response to the Diablo Canyon Nuclear Generating Station located in San Luis Obispo, California, from the early 1960s to the present. Four distinct phases of public intervention were discerned, based on change in both plant-related issues and in the nature of the antinuclear constituencies in the region. The level of public concern varied both geographically and temporally and is related to the area's social structure, environmental predispositions, and distribution of plant-related economic benefits. External events, such as the prolonged debate over the risk assessment of the seismic hazard and the Three Mile Island accident were found to be important factors in explaining variation in public concern and political response

  2. Public response to the Diablo Canyon Nuclear Generating Station

    International Nuclear Information System (INIS)

    Pijawka, K.D.

    1982-01-01

    We examine the nature of the public response to the Diablo Canyon Nuclear Generating Station located in San Luis Obispo, California, from the early 1960s to the present. Four distinct phases of public intervention were discerned, based on change in both plant-related issues and in the nature of the antinuclear constituencies in the region. The level of public concern varied both geographically and temporally and is related to the area's social structure, environmental predispositions, and distribution of plant-related economic benefits. External events, such as the prolonged debate over the risk assessment of the seismic hazard and the Three Mile Island accident were found to be important factors in explaining variation in public concern and political response. (author)

  3. Public response to the Diablo Canyon Nuclear Generating Station

    Energy Technology Data Exchange (ETDEWEB)

    Pijawka, K D [Arizona State Univ., Tempe (USA)

    1982-08-01

    We examine the nature of the public response to the Diablo Canyon Nuclear Generating Station located in San Luis Obispo, California, from the early 1960s to the present. Four distinct phases of public intervention were discerned, based on change in both plant-related issues and in the nature of the antinuclear constituencies in the region. The level of public concern varied both geographically and temporally and is related to the area's social structure, environmental predispositions, and distribution of plant-related economic benefits. External events, such as the prolonged debate over the risk assessment of the seismic hazard and the Three Mile Island accident were found to be important factors in explaining variation in public concern and political response.

  4. Co-segregation of Huntington disease and hereditary spastic paraplegia in 4 generations.

    Science.gov (United States)

    Panas, Marios; Karadima, Georgia; Kalfakis, Nikolaos; Vassilopoulos, Dimitris

    2011-07-01

    Huntington disease (HD) is an autosomal dominant neurodegenerative disease characterized by choreic hyperkinesias, cognitive decline, and psychiatric manifestations, caused by an increased number of CAG repeats in the IT15 gene on chromosome 4p16.3. Silver syndrome is a rare autosomal dominant form of complicated hereditary spastic paraplegia, characterized by lower limb spasticity in addition to amyotrophy of the small muscles of the hands. In addition to the previously identified locus SPG17 on chromosome 11q12-q14, a new locus (SPG38) on chromosome 4p16-p15 has been recently identified, a region that includes the HD gene. We present a Greek family with 5 members diagnosed with HD in 4 generations. All affected members also presented with clinical features of Silver syndrome showing severe spastic paraplegia and prominent atrophy of all small hand muscles bilaterally. None of the other family members showed features of either HD or spastic paraplegia. The reported coexistence of Silver syndrome with HD in 4 generations is not fortuitous, suggesting that these 2 distinct genetic disorders are in linkage disequilibrium. Although rare, it is reasonable to expect additional similar cases. Clinical neurologists should perhaps investigate this possibility in cases combining features of HD and involvement of the upper and lower motor neurons.

  5. Huntington's disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth; Law, Ian; Jønch, Aia

    2011-01-01

    In this open-label pilot study, the authors evaluated the effect of memantine on the distribution of brain glucose metabolism in four Huntington's disease (HD) patients as determined by serial 18-fluoro-deoxyglucose [F(18)]FDG-PET scans over a period of 3-4 months (90-129 days, with one patient...

  6. Numerical modeling of landslides and generated seismic waves: The Bingham Canyon Mine landslides

    Science.gov (United States)

    Miallot, H.; Mangeney, A.; Capdeville, Y.; Hibert, C.

    2016-12-01

    Landslides are important natural hazards and key erosion processes. They create long period surface waves that can be recorded by regional and global seismic networks. The seismic signals are generated by acceleration/deceleration of the mass sliding over the topography. They consist in a unique and powerful tool to detect, characterize and quantify the landslide dynamics. We investigate here the processes at work during the two massive landslides that struck the Bingham Canyon Mine on the 10th April 2013. We carry a combined analysis of the generated seismic signals and the landslide processes computed with a 3D modeling on a complex topography. Forces computed by broadband seismic waveform inversion are used to constrain the study and particularly the force-source and the bulk dynamic. The source time function are obtained by a 3D model (Shaltop) where rheological parameters can be adjusted. We first investigate the influence of the initial shape of the sliding mass which strongly affects the whole landslide dynamic. We also see that the initial shape of the source mass of the first landslide constrains pretty well the second landslide source mass. We then investigate the effect of a rheological parameter, the frictional angle, that strongly influences the resulted computed seismic source function. We test here numerous friction laws as the frictional Coulomb law and a velocity-weakening friction law. Our results show that the force waveform fitting the observed data is highly variable depending on these different choices.

  7. Generation of induced pluripotent stem cell line, CSSi004-A (2962, from a patient diagnosed with Huntington's disease at the presymptomatic stage

    Directory of Open Access Journals (Sweden)

    Eris Bidollari

    2018-04-01

    Full Text Available Huntington's disease (HD is an incurable, autosomal dominant, hereditary neurodegenerative disorder that typically manifests itself in midlife. This pathology is linked to the deregulation of multiple, as yet unknown, cellular processes starting before HD onset. A human iPS cell line was generated from skin fibroblasts of a subject at the presymptomatic life stage, carrying a polyglutamine expansion in HTT gene codifying Huntingtin protein. The iPSC line contained the expected CAG expansion, expressed the expected pluripotency markers, displayed in vivo differentiation potential to the three germ layers and had a normal karyotype.

  8. Psychopathology in Huntington's disease

    NARCIS (Netherlands)

    Duijn, Erik van

    2010-01-01

    Dit proefschrift begint met een overzichtsartikel van oorspronkelijke onderzoek naar psychopathologie bij mutatiedragers voor de ziekte van Huntington. Aansluitend worden de resultaten van een cohortstudie naar de aanwezigheid en ernst van psychopathologie bij mensen met de ziekte van Huntington in

  9. Lung cancer risk assessment due to traffic-generated particles exposure in urban street canyons: A numerical modelling approach.

    Science.gov (United States)

    Scungio, M; Stabile, L; Rizza, V; Pacitto, A; Russi, A; Buonanno, G

    2018-08-01

    Combustion-generated nanoparticles are responsible for negative health effects due to their ability to penetrate in the lungs, carrying toxic compounds with them. In urban areas, the coexistence of nanoparticle sources and particular street-building configurations can lead to very high particle exposure levels. In the present paper, an innovative approach for the evaluation of lung cancer incidence in street canyon due to exposure to traffic-generated particles was proposed. To this end, the literature-available values of particulate matter, PAHs and heavy metals emitted from different kind of vehicles were used to calculate the Excess Lifetime Cancer Risk (ELCR) at the tailpipe. The estimated ELCR was then used as input data in a numerical CFD (Computational Fluid Dynamics) model that solves the mass, momentum, turbulence and species transport equations, in order to evaluate the cancer risk in every point of interest inside the street canyon. Thus, the influence of wind speed and street canyon geometry (H/W, height of building, H and width of the street, W) on the ELCR at street level was evaluated by means of a CFD simulation. It was found that the ELCR calculated on the leeward and windward sides of the street canyon at a breathable height of 1.5 m, for people exposed 15 min per day for 20 years, is equal to 1.5 × 10 -5 and 4.8 × 10 -6 , respectively, for wind speed of 1 m/s and H/W equal to 1. The ELCR at street level results higher on the leeward side for aspect ratios equal to 1 and 3, while for aspect ratio equal to 2 it is higher on the windward side. In addition, the simulations showed that with the increasing of wind speed the ELCR becomes lower everywhere in the street canyon, due to the increased in dispersion. Copyright © 2018 Elsevier B.V. All rights reserved.

  10. Diablo Canyon

    International Nuclear Information System (INIS)

    Bindon, F.J.L.

    1986-01-01

    The paper traces the history of Diablo Canyon nuclear power station, California, which took 18 years to reach full-power testing from the planning stage. The major delays during the construction are outlined, as well as the costs of Diablo Canyon. (UK)

  11. Socio-economic impacts of nuclear generating stations: Diablo Canyon case study

    International Nuclear Information System (INIS)

    Pijawka, K.D.; Yaquinto, G.

    1982-07-01

    This report documents a case study of the socioeconomic impacts of the construction and operation of the Diablo Canyon nuclear power station. It is part of a major post-licensing study of the socioeconomic impacts at twelve nuclear power stations. The case study covers the period beginning with the announcement of plans to construct the reactor and ending in the period, 1980 to 1981. The case study deals with changes in the economy, population, settlement patterns and housing, local government and public services, social structure, and public response in the study area during the construction/operation of the reactor. A regional modeling approach is used to trace the impact of construction/operation on the local economy, labor market, and housing market. Emphasis in the study is on the attribution of socioeconomic impacts to the reactor or other causal factors. As part of the study of local public response to the construction/operation of the reactor, the effects of the Three Mile Island accident are examined

  12. Identification of genetic variants associated with Huntington's disease progression

    DEFF Research Database (Denmark)

    Hensman Moss, Davina J; Pardiñas, Antonio F; Langbehn, Douglas

    2017-01-01

    indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008-11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers...... in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression......BACKGROUND: Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate...

  13. Learning about Huntington's Disease

    Science.gov (United States)

    Skip to main content Learning About Huntington's Disease Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research ...

  14. Hazard assessment for a submarine landslide generated local-source tsunami from Kaikoura Canyon

    Science.gov (United States)

    DuBois, J.

    2012-04-01

    The Kaikoura Canyon, sediment sink for the Canterbury rivers north of Christchurch, comes to within 500 meters of shore at Goose Bay and accumulates approximately 1.5x106 m3 of sediment each year (Lewis and Barnes, 1999). This sediment, which has accumulated to about seventy meters in thickness (Walters et al., 2006), exhibits tensional fractures, is located in a tectonically active area and could result in catastrophic failure and potentially a local-source tsunami (Lewis and Banes, 1999; Lewis, 1998; Walters et al, 2006). Evidence suggests that this may have happened in the last two hundred years (Lewis, 1998; Lewis and Barnes 1999) and with a return period on the nearby Alpine and Hope faults also in the range of a one to two hundred years (Walters et al, 2006) could happen again relatively soon. A review of the historical record and oral traditions for Kaikoura shows that historically Kaikoura has been affected by 11 events of which 10 are from distant sources and one, though debatable, is possibly from a local source. There are some preserved traditions for the Kaikoura area. These taniwha stories from near Oaro and from the Lyell Creek have been repeated and changed though time though the general essence remains the same. These taniwha legends, though not conclusive, indicate a dangerous shoreline where people have been killed in the past, possibly by flooding or tsunami. Archaeological investigations at Kaikoura found evidence of a Maori occupational layers interrupted by water-worn stones, a "lens of clean gravel between occupation layers" and in other areas of the excavation, the gravels separate discontinuous periods of occupation (Fomison 1963; Foster, 2006). Additionally "pea-gravel" sized greywacke pebbles were found dispersed throughout sections of the South Bay shore platforms, though they were attributesd to slopewash (Duckmanton, 1974) this is less likely since the nearby hills are limestone. A geological investigation along the Kaikoura Coast, at

  15. VT Data - Zoning 20120709, Huntington

    Data.gov (United States)

    Vermont Center for Geographic Information — Zoning district data for the Town of Huntington, Vermont. For details regarding each zoning district refer to the current zoning regulations on town of Huntington's...

  16. Geomorphic process fingerprints in submarine canyons

    Science.gov (United States)

    Brothers, Daniel S.; ten Brink, Uri S.; Andrews, Brian D.; Chaytor, Jason D.; Twichell, David C.

    2013-01-01

    Submarine canyons are common features of continental margins worldwide. They are conduits that funnel vast quantities of sediment from the continents to the deep sea. Though it is known that submarine canyons form primarily from erosion induced by submarine sediment flows, we currently lack quantitative, empirically based expressions that describe the morphology of submarine canyon networks. Multibeam bathymetry data along the entire passive US Atlantic margin (USAM) and along the active central California margin near Monterey Bay provide an opportunity to examine the fine-scale morphology of 171 slope-sourced canyons. Log–log regression analyses of canyon thalweg gradient (S) versus up-canyon catchment area (A) are used to examine linkages between morphological domains and the generation and evolution of submarine sediment flows. For example, canyon reaches of the upper continental slope are characterized by steep, linear and/or convex longitudinal profiles, whereas reaches farther down canyon have distinctly concave longitudinal profiles. The transition between these geomorphic domains is inferred to represent the downslope transformation of debris flows into erosive, canyon-flushing turbidity flows. Over geologic timescales this process appears to leave behind a predictable geomorphic fingerprint that is dependent on the catchment area of the canyon head. Catchment area, in turn, may be a proxy for the volume of sediment released during geomorphically significant failures along the upper continental slope. Focused studies of slope-sourced submarine canyons may provide new insights into the relationships between fine-scale canyon morphology and down-canyon changes in sediment flow dynamics.

  17. Huntington\\'s disease: Genetic heterogeneity in black African patients

    African Journals Online (AJOL)

    Objective. Huntington's disease (HD) has been reported to occur rarely in black patients. A new genetic variant– Huntington's disease-like 2 (HDL2) – occurring more frequently in blacks, has recently been described. The absence of an expanded trinucleotide repeat at the chromosome 4 HD locus was previously regarded ...

  18. Clinical neurogenetics: huntington disease.

    Science.gov (United States)

    Bordelon, Yvette M

    2013-11-01

    Huntington disease (HD) is an autosomal dominant, adult-onset, progressive neurodegenerative disease characterized by the triad of abnormal movements (typically chorea), cognitive impairment, and psychiatric problems. It is caused by an expanded CAG repeat in the gene encoding the protein huntingtin on chromosome 4 and causes progressive atrophy of the striatum as well as cortical and other extrastriatal structures. Genetic testing has been available since 1993 to confirm diagnosis in affected adults and for presymptomatic testing in at-risk individuals. This review covers HD signs, symptoms, and pathophysiology; current genetic testing issues; and current and future treatment strategies. Copyright © 2013 Elsevier Inc. All rights reserved.

  19. Socioeconomic impacts of nuclear generating stations - Diablo Canyon case study. Technical report for 1 Oct 78-4 Jan 82

    International Nuclear Information System (INIS)

    Pijawka, K.D.; Yaquinto, G.

    1982-07-01

    This report documents a case study of the socioeconomic impacts of the construction and operation of the Diablo Canyon nuclear power station. It is part of a major post-licensing study of the socioeconomic impacts at twelve nuclear power stations. The case study covers the period beginning with the announcement of plans to construct the reactor and ending in the period, 1980-81. The case study deals with changes in the economy, population, settlemeent patterns and housing, local government and public services, social structure, and public response in the study during the construction/operation of the reactor. A regional modeling approach is used to trace the impact of construction/operation on the local economy, labor market, and housing market. Emphasis in the study is on the attribution of socioeconomic impacts to the reactor or other causal factors. As part of the study of local public response to the construction/operation of the reactor, the effects of the Three Mile Island accident are examined

  20. Huntington's disease presenting as amyotrophic lateral sclerosis.

    LENUS (Irish Health Repository)

    Phukan, Julie

    2010-08-01

    We present the clinical, electrophysiological and molecular genetic findings of a 58-year-old male with genetically confirmed Huntington\\'s disease (HD) and concurrent clinically definite ALS by El Escorial criteria. The patient presented with asymmetric upper limb amyotrophy and weakness, and subsequently developed chorea and cognitive change. Genetic testing confirmed the presence of expanded trinucleotide repeats in huntingtin, consistent with a diagnosis of Huntington\\'s disease. This case confirms the rare coexistence of Huntington\\'s disease and motor neuron degeneration.

  1. Flow dynamics around downwelling submarine canyons

    Directory of Open Access Journals (Sweden)

    J. M. Spurgin

    2014-10-01

    Full Text Available Flow dynamics around a downwelling submarine canyon were analysed with the Massachusetts Institute of Technology general circulation model. Blanes Canyon (northwestern Mediterranean was used for topographic and initial forcing conditions. Fourteen scenarios were modelled with varying forcing conditions. Rossby and Burger numbers were used to determine the significance of Coriolis acceleration and stratification (respectively and their impacts on flow dynamics. A new non-dimensional parameter (χ was introduced to determine the significance of vertical variations in stratification. Some simulations do see brief periods of upwards displacement of water during the 10-day model period; however, the presence of the submarine canyon is found to enhance downwards advection of density in all model scenarios. High Burger numbers lead to negative vorticity and a trapped anticyclonic eddy within the canyon, as well as an increased density anomaly. Low Burger numbers lead to positive vorticity, cyclonic circulation, and weaker density anomalies. Vertical variations in stratification affect zonal jet placement. Under the same forcing conditions, the zonal jet is pushed offshore in more uniformly stratified domains. The offshore jet location generates upwards density advection away from the canyon, while onshore jets generate downwards density advection everywhere within the model domain. Increasing Rossby values across the canyon axis, as well as decreasing Burger values, increase negative vertical flux at shelf break depth (150 m. Increasing Rossby numbers lead to stronger downwards advection of a passive tracer (nitrate, as well as stronger vorticity within the canyon. Results from previous studies are explained within this new dynamic framework.

  2. Identification of phenylbutyrate-generated metabolites in Huntington disease patients using parallel liquid chromatography/electrochemical array/mass spectrometry and off-line tandem mass spectrometry.

    Science.gov (United States)

    Ebbel, Erika N; Leymarie, Nancy; Schiavo, Susan; Sharma, Swati; Gevorkian, Sona; Hersch, Steven; Matson, Wayne R; Costello, Catherine E

    2010-04-15

    Oral sodium phenylbutyrate (SPB) is currently under investigation as a histone deacetylation (HDAC) inhibitor in Huntington disease (HD). Ongoing studies indicate that symptoms related to HD genetic abnormalities decrease with SPB therapy. In a recently reported safety and tolerability study of SPB in HD, we analyzed overall chromatographic patterns from a method that employs gradient liquid chromatography with series electrochemical array, ultraviolet (UV), and fluorescence (LCECA/UV/F) for measuring SPB and its metabolite phenylacetate (PA). We found that plasma and urine from SPB-treated patients yielded individual-specific patterns of approximately 20 metabolites that may provide a means for the selection of subjects for extended trials of SPB. The structural identification of these metabolites is of critical importance because their characterization will facilitate understanding the mechanisms of drug action and possible side effects. We have now developed an iterative process with LCECA, parallel LCECA/LCMS, and high-performance tandem MS for metabolite characterization. Here we report the details of this method and its use for identification of 10 plasma and urinary metabolites in treated subjects, including indole species in urine that are not themselves metabolites of SPB. Thus, this approach contributes to understanding metabolic pathways that differ among HD patients being treated with SPB. Copyright 2010 Elsevier Inc. All rights reserved.

  3. Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

    OpenAIRE

    Hensman Moss, Davina J; Pardinas, Antonio; Langbehn, Douglas; Lo, Kitty; Leavitt, Blair R; Roos, Raymund; Durr, Alexandra; Mead, Simon; Holmans, Peter; Jones, Lesley; Tabrizi, Sarah J; Coleman, A; Santos, R Dar; Decolongon, J; Sturrock, A

    2017-01-01

    Background\\ud \\ud Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure.\\ud \\ud Methods\\ud \\ud We generated a progression score on the basis of principal ...

  4. Music therapy in Huntington's disease

    NARCIS (Netherlands)

    Bruggen-Rufi, van C.H.M.

    2018-01-01

    The thesis reports about the effects of music therapy with patients in the late stage of Huntington's disease. A literature review, a focus group study, a randomized controlled trial, an evaluation for complex interventions and a case report study are integrated in the thesis. The beneficial

  5. Semantic, phonologic, and verb fluency in Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Jardim Azambuja

    Full Text Available Abstract Verbal fluency tasks have been identified as important indicators of executive functioning impairment in patients with frontal lobe dysfunction. Although the usual evaluation of this ability considers phonologic and semantic criteria, there is some evidence that fluency of verbs would be more sensitive in disclosing frontostriatal physiopathology since frontal regions primarily mediate retrieval of verbs. Huntington's disease usually affects these circuitries. Objective: To compare three types of verbal fluency task in the assessment of frontal-striatal dysfunction in HD subjects. Methods: We studied 26 Huntington's disease subjects, divided into two subgroups: mild (11 and moderate (15 along with 26 normal volunteers matched for age, gender and schooling, for three types of verbal fluency: phonologic fluency (F-A-S, semantic fluency and fluency of verbs. Results: Huntington's disease subjects showed a significant reduction in the number of words correctly generated in the three tasks when compared to the normal group. Both controls and Huntington's disease subjects showed a similar pattern of decreasing task performance with the greatest number of words being generated by semantic elicitation followed by verbs and lastly phonologic criteria. We did not find greater production of verbs compared with F-A-S and semantic conditions. Moreover, the fluency of verbs distinguished only the moderate group from controls. Conclusion: Our results indicated that phonologic and semantic fluency can be used to evaluate executive functioning, proving more sensitive than verb fluency. However, it is important to point out that the diverse presentations of Huntington's disease means that an extended sample is necessary for more consistent analysis of this issue.

  6. Tectonic activity and the evolution of submarine canyons: The Cook Strait Canyon system, New Zealand

    Science.gov (United States)

    Micallef, Aaron; Mountjoy, Joshu; Barnes, Philip; Canals, Miquel; Lastras, Galderic

    2016-04-01

    Submarine canyons are Earth's most dramatic erosional features, comprising steep-walled valleys that originate in the continental shelf and slope. They play a key role in the evolution of continental margins by transferring sediments into deep water settings and are considered important biodiversity hotspots, pathways for nutrients and pollutants, and analogues of hydrocarbon reservoirs. Although comprising only one third of continental margins worldwide, active margins host more than half of global submarine canyons. We still lack of thorough understanding of the coupling between active tectonics and submarine canyon processes, which is necessary to improve the modelling of canyon evolution in active margins and derive tectonic information from canyon morphology. The objectives of this study are to: (i) understand how tectonic activity influences submarine canyon morphology, processes, and evolution in an active margin, and (2) formulate a generalised model of canyon development in response to tectonic forcing based on morphometric parameters. We fulfil these objectives by analysing high resolution geophysical data and imagery from Cook Strait Canyon system, offshore New Zealand. Using these data, we demonstrate that tectonic activity, in the form of major faults and structurally-generated tectonic ridges, leaves a clear topographic signature on submarine canyon location and morphology, in particular their dendritic and sinuous planform shapes, steep and linear longitudinal profiles, and cross-sectional asymmetry and width. We also report breaks/changes in canyon longitudinal slope gradient, relief and slope-area regression models at the intersection with faults. Tectonic activity gives rise to two types of knickpoints in the Cook Strait Canyon. The first type consists of low slope gradient, rounded and diffusive knickpoints forming as a result of short wavelength folds or fault break outs and being restored to an equilibrium profile by upstream erosion and

  7. Flow in bedrock canyons.

    Science.gov (United States)

    Venditti, Jeremy G; Rennie, Colin D; Bomhof, James; Bradley, Ryan W; Little, Malcolm; Church, Michael

    2014-09-25

    Bedrock erosion in rivers sets the pace of landscape evolution, influences the evolution of orogens and determines the size, shape and relief of mountains. A variety of models link fluid flow and sediment transport processes to bedrock incision in canyons. The model components that represent sediment transport processes are increasingly well developed. In contrast, the model components being used to represent fluid flow are largely untested because there are no observations of the flow structure in bedrock canyons. Here we present a 524-kilometre, continuous centreline, acoustic Doppler current profiler survey of the Fraser Canyon in western Canada, which includes 42 individual bedrock canyons. Our observations of three-dimensional flow structure reveal that, as water enters the canyons, a high-velocity core follows the bed surface, causing a velocity inversion (high velocities near the bed and low velocities at the surface). The plunging water then upwells along the canyon walls, resulting in counter-rotating, along-stream coherent flow structures that diverge near the bed. The resulting flow structure promotes deep scour in the bedrock channel floor and undercutting of the canyon walls. This provides a mechanism for channel widening and ensures that the base of the walls is swept clear of the debris that is often deposited there, keeping the walls nearly vertical. These observations reveal that the flow structure in bedrock canyons is more complex than assumed in the models presently used. Fluid flow models that capture the essence of the three-dimensional flow field, using simple phenomenological rules that are computationally tractable, are required to capture the dynamic coupling between flow, bedrock erosion and solid-Earth dynamics.

  8. Effects of canyon geometry on the distribution of traffic-related air pollution in a large urban area: Implications of a multi-canyon air pollution dispersion model

    Science.gov (United States)

    Fu, Xiangwen; Liu, Junfeng; Ban-Weiss, George A.; Zhang, Jiachen; Huang, Xin; Ouyang, Bin; Popoola, Olalekan; Tao, Shu

    2017-09-01

    Street canyons are ubiquitous in urban areas. Traffic-related air pollutants in street canyons can adversely affect human health. In this study, an urban-scale traffic pollution dispersion model is developed considering street distribution, canyon geometry, background meteorology, traffic assignment, traffic emissions and air pollutant dispersion. In the model, vehicle exhausts generated from traffic flows first disperse inside street canyons along the micro-scale wind field generated by computational fluid dynamics (CFD) model. Then, pollutants leave the street canyon and further disperse over the urban area. On the basis of this model, the effects of canyon geometry on the distribution of NOx and CO from traffic emissions were studied over the center of Beijing. We found that an increase in building height leads to heavier pollution inside canyons and lower pollution outside canyons at pedestrian level, resulting in higher domain-averaged concentrations over the area. In addition, canyons with highly even or highly uneven building heights on each side of the street tend to lower the urban-scale air pollution concentrations at pedestrian level. Further, increasing street widths tends to lead to lower pollutant concentrations by reducing emissions and enhancing ventilation simultaneously. Our results indicate that canyon geometry strongly influences human exposure to traffic pollutants in the populated urban area. Carefully planning street layout and canyon geometry while considering traffic demand as well as local weather patterns may significantly reduce inhalation of unhealthy air by urban residents.

  9. Huntington's disease: a perplexing neurological disease ...

    African Journals Online (AJOL)

    Huntington's disease is an inherited intricate brain illness. It is a neurodegenerative, insidious disorder; the onset of the disease is very late to diagnose. It is caused by an expanded CAG repeat in the Huntingtin gene, which encodes an abnormally long polyglutamine repeat in the Huntingtin protein. Huntington's disease ...

  10. Huntington's disease : Psychological aspects of predictive testing

    NARCIS (Netherlands)

    Timman, Reinier

    2005-01-01

    Predictive testing for Huntington's disease appears to have long lasting psychological effects. The predictive test for Huntington's disease (HD), a hereditary disease of the nervous system, was introduced in the Netherlands in the late eighties. As adverse consequences of the test were

  11. Psychiatric symptoms and CAG expansion in Huntington`s disease

    Energy Technology Data Exchange (ETDEWEB)

    Weber, M.W.; Schmid, W.; Spiegel, R. [Univ. of Zuerich (Switzerland)

    1996-02-16

    The mutation responsible for Huntington`s disease (HD) is an elongated CAG repeat in the coding region of the IT15 gene. A PCR-based test with high sensitivity and accuracy is now available to identify asymptomatic gene carriers and patients. An inverse correlation between CAG copy number and age at disease onset has been found in a large number of affected individuals. The influence of the CAG repeat expansion on other phenotypic manifestations, especially specific psychiatric symptoms has not been studied intensively. In order to elucidate this situation we investigated the relation between CAG copy number and distinct psychiatric phenotypes found in 79 HD-patients. None of the four differentiated categories (personality change, psychosis, depression, and nonspecific alterations) showed significant differences in respect to size of the CAG expansion. In addition, no influence of individual sex on psychiatric presentation could be found. On the other hand in patients with personality changes maternal transmission was significantly more frequent compared with all other groups. Therefore we suggest that clinical severity of psychiatric features in HD is not directly dependent on the size of the dynamic mutation involved. The complex pathogenetic mechanisms leading to psychiatric alterations are still unknown and thus genotyping does not provide information about expected psychiatric symptoms in HD gene carriers. 40 refs., 1 fig., 2 tabs.

  12. Separations canyon decontamination facilities

    International Nuclear Information System (INIS)

    Hershey, J.H.

    1975-01-01

    Highly radioactive process equipment is decontaminated at the Savannah River Plant in specially equipped areas of the separations canyon building so that direct mechanical repairs or alterations can be made. Using these facilities it is possible to decontaminate and repair equipment such as 10- x 11-ft storage tanks, 8- x 8-ft batch evaporator pots and columns, 40-in. Bird centrifuges, canyon pumps and agitators, and various canyon piping systems or ''jumpers.'' For example, centrifuge or evaporator pots can be decontaminated and rebuilt for about 60 percent of the 1974 replacement cost. The combined facilities can decontaminate and repair 6 to 10 pieces of major equipment per year. Decontamination time varies with type of equipment and radioactivity levels encountered

  13. Separations canyon decontamination facilities

    International Nuclear Information System (INIS)

    Hershey, J.H.

    1975-05-01

    Highly radioactive process equipment is decontaminated at the Savannah River Plant in specially equipped areas of the separations canyon buildings so that direct mechanical repairs or alterations can be made. Using these facilities it is possible to decontaminate and repair equipment such as 10- x 11-ft storage tanks, 8- x 8-ft batch evaporator pots and columns, 40-in. Bird centrifuges, canyon pumps and agitators, and various canyon piping systems or ''jumpers.'' For example, centrifuge or evaporator pots can be decontaminated and rebuilt for about 60 percent of the 1974 replacement cost. The combined facilities can decontaminate and repair 6 to 10 pieces of major equipment per year. Decontamination time varies with type of equipment and radioactivity levels encountered. (U.S.)

  14. Generation of induced pluripotent stem cells from transgenic minipigs expressing the N-terminal part of the human mutant huntingtin - a new way to study pathogenesis of Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Lišková, Irena; Vodička, P.; Juhás, Štefan; Klempíř, J.; Motlík, Jan

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 18-19 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * huntingtin * induced pluripotent stem cells Subject RIV: FH - Neurology

  15. Geologic Investigation of a Potential Site for a Next-Generation Reactor Neutrino Oscillation Experiment -- Diablo Canyon, San Luis Obispo County, CA

    International Nuclear Information System (INIS)

    Onishi, Celia Tiemi; Dobson, Patrick; Nakagawa, Seiji; Glaser, Steven; Galic, Dom

    2004-01-01

    This report provides information on the geology and selected physical and mechanical properties of surface rocks collected at Diablo Canyon, San Luis Obispo County, California as part of the design and engineering studies towards a future reactor neutrino oscillation experiment. The main objective of this neutrino project is to study the process of neutrino flavor transformation or neutrino oscillation by measuring neutrinos produced in the fission reactions of a nuclear power plant. Diablo Canyon was selected as a candidate site because it allows the detectors to be situated underground in a tunnel close to the source of neutrinos (i.e., at a distance of several hundred meters from the nuclear power plant) while having suitable topography for shielding against cosmic rays. The detectors have to be located underground to minimize the cosmic ray-related background noise that can mimic the signal of reactor neutrino interactions in the detector. Three Pliocene-Miocene marine sedimentary units dominate the geology of Diablo Canyon: the Pismo Formation, the Monterey Formation, and the Obispo Formation. The area is tectonically active, located east of the active Hosgri Fault and in the southern limb of the northwest trending Pismo Syncline. Most of the potential tunnel for the neutrino detector lies within the Obispo Formation. Review of previous geologic studies, observations from a field visit, and selected physical and mechanical properties of rock samples collected from the site provided baseline geological information used in developing a preliminary estimate for tunneling construction cost. Gamma-ray spectrometric results indicate low levels of radioactivity for uranium, thorium, and potassium. Grain density, bulk density, and porosity values for these rock samples range from 2.37 to 2.86 g/cc, 1.41 to 2.57 g/cc, and 1.94 to 68.5 percent respectively. Point load, unconfined compressive strength, and ultrasonic velocity tests were conducted to determine rock

  16. Geologic Investigation of a Potential Site for a Next-Generation Reactor Neutrino Oscillation Experiment -- Diablo Canyon, San Luis Obispo County, CA

    International Nuclear Information System (INIS)

    Onishi, Celia Tiemi; Dobson, Patrick; Nakagawa, Seiji; Glaser, Steven; Galic, Dom

    2004-01-01

    This report provides information on the geology and selected physical and mechanical properties of surface rocks collected at Diablo Canyon, San Luis Obispo County, California as part of the design and engineering studies towards a future reactor neutrino oscillation experiment. The main objective of this neutrino project is to study the process of neutrino flavor transformation--or neutrino oscillation--by measuring neutrinos produced in the fission reactions of a nuclear power plant. Diablo Canyon was selected as a candidate site because it allows the detectors to be situated underground in a tunnel close to the source of neutrinos (i.e., at a distance of several hundred meters from the nuclear power plant) while having suitable topography for shielding against cosmic rays. The detectors have to be located underground to minimize the cosmic ray-related background noise that can mimic the signal of reactor neutrino interactions in the detector. Three Pliocene-Miocene marine sedimentary units dominate the geology of Diablo Canyon: the Pismo Formation, the Monterey Formation, and the Obispo Formation. The area is tectonically active, located east of the active Hosgri Fault and in the southern limb of the northwest trending Pismo Syncline. Most of the potential tunnel for the neutrino detector lies within the Obispo Formation. Review of previous geologic studies, observations from a field visit, and selected physical and mechanical properties of rock samples collected from the site provided baseline geological information used in developing a preliminary estimate for tunneling construction cost. Gamma-ray spectrometric results indicate low levels of radioactivity for uranium, thorium, and potassium. Grain density, bulk density, and porosity values for these rock samples range from 2.37 to 2.86 g/cc, 1.41 to 2.57 g/cc, and 1.94 to 68.5% respectively. Point load, unconfined compressive strength, and ultrasonic velocity tests were conducted to determine rock mechanical

  17. Fourmile Canyon Fire Findings

    Science.gov (United States)

    Russell Graham; Mark Finney; Chuck McHugh; Jack Cohen; Dave Calkin; Rick Stratton; Larry Bradshaw; Ned Nikolov

    2012-01-01

    The Fourmile Canyon Fire burned in the fall of 2010 in the Rocky Mountain Front Range adjacent to Boulder, Colorado. The fire occurred in steep, rugged terrain, primarily on privately owned mixed ponderosa pine and Douglas-fir forests. The fire started on September 6 when the humidity of the air was very dry (¡Ö

  18. Personality Traits in Huntington's Disease

    DEFF Research Database (Denmark)

    Larsen, Ida Unmack; Mortensen, Erik Lykke; Vinther-Jensen, Tua

    2016-01-01

    Huntington's disease (HD) is associated with risk for developing psychiatric symptoms. Vulnerability or resilience to psychiatric symptoms may be associated with personality traits. This exploratory study, aimed to investigate personality traits in a large cohort of HD carriers and at risk gene......-expansion negative individuals (HD non-carriers), exploring whether carrying the HD gene or growing up in an HD family influences personality traits. Forty-seven HD carriers, Thirty-nine HD non-carriers, and 121 healthy controls answered the Danish version of the revised NEO personality inventory. Comparisons...... symptoms. Our findings suggest that, there is no direct effect of the HD gene on personality traits, but that personality assessment may be relevant to use when identifying individuals from HD families who are vulnerable to develop psychiatric symptoms....

  19. Clinical presentation of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Ruocco Heloísa H.

    2006-01-01

    Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

  20. Huntington's disease: a clinical review

    Directory of Open Access Journals (Sweden)

    Roos Raymund AC

    2010-12-01

    Full Text Available Abstract Huntington disease (HD is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD. The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which

  1. Canyons off northwest Puerto Rico

    International Nuclear Information System (INIS)

    Gardner, W.D.; Glover, L.K.; Hollister, C.D.

    1980-01-01

    The Nuclear-Research Submarine NR-1 was used to study morphoplogy, sediment, and sediment-water interactions off the northwest coast of Puerto Rico. New detailed bathymetry from the surface-support ship, USS Portland, shows several submarine canyons in the area, some of them unreported previously. The north coast canyons, Arecibo, Tiberones and Quebradillas, are primarily erosional features although no recent turbidity-current evidence is seen. The canyons are presently filling with river-transported sediments. (orig./ME)

  2. Discrepancies in reporting the CAG repeat lengths for Huntington's disease

    DEFF Research Database (Denmark)

    Quarrell, Oliver W; Handley, Olivia; O'Donovan, Kirsty

    2011-01-01

    Huntington's disease results from a CAG repeat expansion within the Huntingtin gene; this is measured routinely in diagnostic laboratories. The European Huntington's Disease Network REGISTRY project centrally measures CAG repeat lengths on fresh samples; these were compared with the original...

  3. The Whittard Canyon - A case study of submarine canyon processes

    Science.gov (United States)

    Amaro, T.; Huvenne, V. A. I.; Allcock, A. L.; Aslam, T.; Davies, J. S.; Danovaro, R.; De Stigter, H. C.; Duineveld, G. C. A.; Gambi, C.; Gooday, A. J.; Gunton, L. M.; Hall, R.; Howell, K. L.; Ingels, J.; Kiriakoulakis, K.; Kershaw, C. E.; Lavaleye, M. S. S.; Robert, K.; Stewart, H.; Van Rooij, D.; White, M.; Wilson, A. M.

    2016-08-01

    Submarine canyons are large geomorphological features that incise continental shelves and slopes around the world. They are often suggested to be biodiversity and biomass hotspots, although there is no consensus about this in the literature. Nevertheless, many canyons do host diverse faunal communities but owing to our lack of understanding of the processes shaping and driving this diversity, appropriate management strategies have yet to be developed. Here, we integrate all the current knowledge of one single system, the Whittard Canyon (Celtic Margin, NE Atlantic), including the latest research on its geology, sedimentology, geomorphology, oceanography, ecology, and biodiversity in order to address this issue. The Whittard Canyon is an active system in terms of sediment transport. The net suspended sediment transport is mainly up-canyon causing sedimentary overflow in some upper canyon areas. Occasionally sediment gravity flow events do occur, some possibly the result of anthropogenic activity. However, the role of these intermittent gravity flows in transferring labile organic matter to the deeper regions of the canyon appears to be limited. More likely, any labile organic matter flushed downslope in this way becomes strongly diluted with bulk material and is therefore of little food value for benthic fauna. Instead, the fresh organic matter found in the Whittard Channel mainly arrives through vertical deposition and lateral transport of phytoplankton blooms that occur in the area during spring and summer. The response of the Whittard Canyon fauna to these processes is different in different groups. Foraminiferal abundances are higher in the upper parts of the canyon and on the slope than in the lower canyon. Meiofaunal abundances in the upper and middle part of the canyon are higher than on adjacent slopes, but lower in the deepest part. Mega- and macrofauna abundances are higher in the canyon compared with the adjacent slope and are higher in the eastern than

  4. Perceptions of genetic discrimination among people at risk for Huntington?s disease: a cross sectional survey

    OpenAIRE

    Bombard, Yvonne; Veenstra, Gerry; Friedman, Jan M; Creighton, Susan; Currie, Lauren; Paulsen, Jane S; Bottorff, Joan L; Hayden, Michael R

    2009-01-01

    Objective To assess the nature and prevalence of genetic discrimination experienced by people at risk for Huntington?s disease who had undergone genetic testing or remained untested. Design Cross sectional, self reported survey. Setting Seven genetics and movement disorders clinics servicing rural and urban communities in Canada. Participants 233 genetically tested and untested asymptomatic people at risk for Huntington?s disease (response rate 80%): 167 underwent testing (83 had the Huntingt...

  5. Mapping energy poverty in Huntington, West Virginia

    Science.gov (United States)

    Callicoat, Elizabeth Anne

    Energy poverty is a growing phenomenon culminating from the combination of low to mid household income, deteriorating housing structures and rising household energy costs. Energy prices are increasing for all households, but the burden is proportionally larger for those with low to mid income. These groups must sacrifice to afford energy, and are often unable or do not have the autonomy to make structural improvements, especially if they rent their home. Data on residential dwellings from the Cabell County Tax Assessor's Office was used within a geographic information system to map where energy poverty likely exists within the city limits of Huntington, WV. It was found that one fifth of Huntington households are at a high risk of energy poverty, primarily located across the northern section of the city and in the center, surrounding Marshall University, Downtown and Cabell Huntington Hospital.

  6. New York Canyon Stimulation

    Energy Technology Data Exchange (ETDEWEB)

    Raemy, Bernard

    2012-06-21

    The New York Canyon Stimulation Project was to demonstrate the commercial application of Enhanced Geothermal System techniques in Buena Vista Valley area of Pershing County, Nevada. From October 2009 to early 2012, TGP Development Company aggressively implemented Phase I of Pre-Stimulation and Site/Wellbore readiness. This included: geological studies; water studies and analyses and procurement of initial permits for drilling. Oversubscription of water rights and lack of water needed for implementation of EGS were identified and remained primary obstacles. Despite extended efforts to find alternative solutions, the water supply circumstances could not be overcome and led TGP to determine a "No Go" decision and initiate project termination in April 2012.

  7. Best Practices for Mudweight Window Generation and Accuracy Assessment between Seismic Based Pore Pressure Prediction Methodologies for a Near-Salt Field in Mississippi Canyon, Gulf of Mexico

    Science.gov (United States)

    Mannon, Timothy Patrick, Jr.

    Improving well design has and always will be the primary goal in drilling operations in the oil and gas industry. Oil and gas plays are continuing to move into increasingly hostile drilling environments, including near and/or sub-salt proximities. The ability to reduce the risk and uncertainly involved in drilling operations in unconventional geologic settings starts with improving the techniques for mudweight window modeling. To address this issue, an analysis of wellbore stability and well design improvement has been conducted. This study will show a systematic approach to well design by focusing on best practices for mudweight window projection for a field in Mississippi Canyon, Gulf of Mexico. The field includes depleted reservoirs and is in close proximity of salt intrusions. Analysis of offset wells has been conducted in the interest of developing an accurate picture of the subsurface environment by making connections between depth, non-productive time (NPT) events, and mudweights used. Commonly practiced petrophysical methods of pore pressure, fracture pressure, and shear failure gradient prediction have been applied to key offset wells in order to enhance the well design for two proposed wells. For the first time in the literature, the accuracy of the commonly accepted, seismic interval velocity based and the relatively new, seismic frequency based methodologies for pore pressure prediction are qualitatively and quantitatively compared for accuracy. Accuracy standards will be based on the agreement of the seismic outputs to pressure data obtained while drilling and petrophysically based pore pressure outputs for each well. The results will show significantly higher accuracy for the seismic frequency based approach in wells that were in near/sub-salt environments and higher overall accuracy for all of the wells in the study as a whole.

  8. H Canyon Processing In Correlation With FH Analytical Labs

    International Nuclear Information System (INIS)

    Weinheimer, E.

    2012-01-01

    Management of radioactive chemical waste can be a complicated business. H Canyon and F/H Analytical Labs are two facilities present at the Savannah River Site in Aiken, SC that are at the forefront. In fact H Canyon is the only large-scale radiochemical processing facility in the United States and this processing is only enhanced by the aid given from F/H Analytical Labs. As H Canyon processes incoming materials, F/H Labs provide support through a variety of chemical analyses. Necessary checks of the chemical makeup, processing, and accountability of the samples taken from H Canyon process tanks are performed at the labs along with further checks on waste leaving the canyon after processing. Used nuclear material taken in by the canyon is actually not waste. Only a small portion of the radioactive material itself is actually consumed in nuclear reactors. As a result various radioactive elements such as Uranium, Plutonium and Neptunium are commonly found in waste and may be useful to recover. Specific processing is needed to allow for separation of these products from the waste. This is H Canyon's specialty. Furthermore, H Canyon has the capacity to initiate the process for weapons-grade nuclear material to be converted into nuclear fuel. This is one of the main campaigns being set up for the fall of 2012. Once usable material is separated and purified of impurities such as fission products, it can be converted to an oxide and ultimately turned into commercial fuel. The processing of weapons-grade material for commercial fuel is important in the necessary disposition of plutonium. Another processing campaign to start in the fall in H Canyon involves the reprocessing of used nuclear fuel for disposal in improved containment units. The importance of this campaign involves the proper disposal of nuclear waste in order to ensure the safety and well-being of future generations and the environment. As processing proceeds in the fall, H Canyon will have a substantial

  9. Evolution and Submarine Landslide Potential of Monterey Canyon Head, Offshore Central California

    Science.gov (United States)

    Maier, K. L.; Johnson, S. Y.; Hart, P. E.; Hartwell, S. R.

    2016-12-01

    Monterey Canyon, offshore central California, incises the shelf from near the shoreline to 30 km seaward where axial water depths approach 2,000 m. It is one of the world's most studied submarine canyons, yet debate continues concerning its age, formation, and associated geologic hazards. To address these issues, the USGS, with partial support from the California Seafloor Mapping Program, collected hundreds of kilometers of high-resolution, mini-sparker, single-channel (2009 and 2011 surveys) and multichannel (2015 survey) seismic-reflection profiles near the canyon head. The seismic data were combined with multibeam bathymetry to generate a geologic map of the proximal canyon, which delineates numerous faults and compound submarine landslide headwall scarps (covering up to 4 km2) along canyon walls. Seismic-reflection data reveal a massive ( 100 km2 lateral extent) paleochannel cut-and-fill complex underlying the proximal canyon. These subsurface cut-and-fill deposits span both sides of the relatively narrow modern canyon head, crop out in canyon walls, and incise into Purisima Formation (late Miocene and Pliocene) bedrock to depths of up to 0.3 s two-way travel time ( 240 m) below the modern shelf. We propose that the paleochannel complex represents previous locations of a migrating canyon head, and attribute its origin to multiple alternating cycles of fluvial and submarine canyon erosion and deposition linked to fluctuating sea levels. Thus, the canyon head imaged in modern bathymetry is a relatively young feature, perhaps forming in the last 20,000 years of sea-level rise. The paleocanyon deposits are significantly less consolidated than bedrock in deeper canyon walls, and therefore, are probably more prone to submarine landsliding. Nearby mapped faults occur within the active, distributed, San Andreas fault system, and earthquake-generated strong ground motions are likely triggers for past and future submarine landslides and potential associated tsunamis.

  10. 75 FR 27550 - Electrical Interconnection of the Juniper Canyon I Wind Project

    Science.gov (United States)

    2010-05-17

    ... DEPARTMENT OF ENERGY Bonneville Power Administration Electrical Interconnection of the Juniper Canyon I Wind Project AGENCY: Bonneville Power Administration (BPA), Department of Energy (DOE). ACTION... would be generated from their proposed Juniper Canyon I Wind Energy Project (Wind Project) in Klickitat...

  11. 236-Z canyon utilization study

    International Nuclear Information System (INIS)

    Dixon, D.R.

    1977-01-01

    The 236-Z canyon contains equipment for repurification of plutonium and recovery of plutonium from scrap material. To meet production requirements of Fast Flux Test Facility/Clinch River Breeder Reactor oxide with the existing plant, several new pieces of equipment will be needed in the future. More storage space and a better accountability system are needed to support this increased production. The available canyon space needs to be utilized to its fullest in order to accommodate the new equipment. The purpose of this document is to identify the new pieces of equipment, show how they fit into the flowsheet, and locate them in the canyon

  12. Kas Huntington oli prohvet? / Priit Simson

    Index Scriptorium Estoniae

    Simson Priit, 1977-

    2008-01-01

    Autor käsitleb Samuel Huntingtoni teese ning leiab, et tegelikult Huntington ei pakkunud õigustust islamiriikide ründamisele, vaid pigem hoiatas tsivilisatsioonide siseasjusse sekkumise, tekkida võiva ahelreaktsiooni eest, kus üks tsivilisatsiooni liige tõmbab sõtta ka teise

  13. Destination and source memory in Huntington's disease

    NARCIS (Netherlands)

    El Haj, M.; Caillaud, M.; Verny, C.; Fasotti, L.; Allain, P.

    2016-01-01

    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults

  14. 2010 Pacific Gas and Electric Diablo Canyon Power Plant (DCPP): Diablo Canyon, CA Central Coast

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Diablo Canyon Power Plant (DCPP) LiDAR and Imagery datasets are comprised of three separate LiDAR surveys: Diablo Canyon (2010), Diablo Canyon (2010), and San...

  15. A Metabolic Study of Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Rajasree Nambron

    Full Text Available Huntington's disease patients have a number of peripheral manifestations suggestive of metabolic and endocrine abnormalities. We, therefore, investigated a number of metabolic factors in a 24-hour study of Huntington's disease gene carriers (premanifest and moderate stage II/III and controls.Control (n = 15, premanifest (n = 14 and stage II/III (n = 13 participants were studied with blood sampling over a 24-hour period. A battery of clinical tests including neurological rating and function scales were performed. Visceral and subcutaneous adipose distribution was measured using magnetic resonance imaging. We quantified fasting baseline concentrations of glucose, insulin, cholesterol, triglycerides, lipoprotein (a, fatty acids, amino acids, lactate and osteokines. Leptin and ghrelin were quantified in fasting samples and after a standardised meal. We assessed glucose, insulin, growth hormone and cortisol concentrations during a prolonged oral glucose tolerance test.We found no highly significant differences in carbohydrate, protein or lipid metabolism markers between healthy controls, premanifest and stage II/III Huntington's disease subjects. For some markers (osteoprotegerin, tyrosine, lysine, phenylalanine and arginine there is a suggestion (p values between 0.02 and 0.05 that levels are higher in patients with premanifest HD, but not moderate HD. However, given the large number of statistical tests performed interpretation of these findings must be cautious.Contrary to previous studies that showed altered levels of metabolic markers in patients with Huntington's disease, our study did not demonstrate convincing evidence of abnormalities in any of the markers examined. Our analyses were restricted to Huntington's disease patients not taking neuroleptics, anti-depressants or other medication affecting metabolic pathways. Even with the modest sample sizes studied, the lack of highly significant results, despite many being tested, suggests that

  16. A Numerical Simulation of Traffic-Related Air Pollution Exposures in Urban Street Canyons

    Science.gov (United States)

    Liu, J.; Fu, X.; Tao, S.

    2016-12-01

    Urban street canyons are usually associated with intensive vehicle emissions. However, the high buildings successively along both sides of a street block the dispersion of traffic-generated air pollutants, which enhances human exposure and adversely affects human health. In this study, an urban scale traffic pollution dispersion model is developed with the consideration of street distribution, canyon geometry, background meteorology, traffic assignment, traffic emissions and air pollutant dispersion. Vehicle exhausts generated from traffic flows will first disperse inside a street canyon along the micro-scale wind field (generated by computational fluid dynamics (CFD) model) and then leave the street canyon and further disperse over the urban area. On the basis of this model, the effects of canyon geometry on the distribution of NOx and CO from traffic emissions were studied over the center of Beijing, China. We found that an increase of building height along the streets leads to higher pollution levels inside streets and lower pollution levels outside, resulting in higher domain-averaged concentrations over the area. In addition, street canyons with equal (or highly uneven) building heights on two sides of a street tend to lower the urban-scale air pollution concentrations at pedestrian level. Our results indicate that canyon geometry strongly influences human exposure to traffic pollutants in the populated urban area. Carefully planning street layout and canyon geometry in consideration of traffic demand as well as local weather pattern may significantly reduce the chances of unhealthy air being inhaled by urban residents.

  17. Characteristics of flow and reactive pollutant dispersion in urban street canyons

    Science.gov (United States)

    Park, Soo-Jin; Kim, Jae-Jin; Kim, Minjoong J.; Park, Rokjin J.; Cheong, Hyeong-Bin

    2015-05-01

    In this study, the effects of aspect ratio defined as the ratio of building height to street width on the dispersion of reactive pollutants in street canyons were investigated using a coupled CFD-chemistry model. Flow characteristics for different aspect ratios were analyzed first. For each aspect ratio, six emission scenarios with different VOC-NOX ratios were considered. One vortex was generated when the aspect ratio was less than 1.6 (shallow street canyon). When the aspect ratio was greater than 1.6 (deep street canyon), two vortices were formed in the street canyons. Comparing to previous studies on two-dimensional street canyons, the vortex center is slanted toward the upwind building and reverse and downward flows are dominant in street canyons. Near the street bottom, there is a marked difference in flow pattern between in shallow and deep street canyons. Near the street bottom, reverse and downward flows are dominant in shallow street canyon and flow convergence exists near the center of the deep street canyons, which induces a large difference in the NOX and O3 dispersion patterns in the street canyons. NOX concentrations are high near the street bottom and decreases with height. The O3 concentrations are low at high NO concentrations near the street bottom because of NO titration. At a low VOC-NOX ratio, the NO concentrations are sufficiently high to destroy large amount of O3 by titration, resulting in an O3 concentration in the street canyon much lower than the background concentration. At high VOC-NOX ratios, a small amount of O3 is destroyed by NO titration in the lower layer of the street canyons. However, in the upper layer, O3 is formed through the photolysis of NO2 by VOC degradation reactions. As the aspect ratio increases, NOX (O3) concentrations averaged over the street canyons decrease (increase) in the shallow street canyons. This is because outward flow becomes strong and NOX flux toward the outsides of the street canyons increases

  18. Mineral resources of the Desolation Canyon, Turtle Canyon, and Floy Canyon Wilderness Study Areas, Carbon Emery, and Grand counties, Utah

    International Nuclear Information System (INIS)

    Cashion, W.B.; Kilburn, J.E.; Barton, H.N.; Kelley, K.D.; Kulik, D.M.; McDonnell, J.R.

    1990-09-01

    This paper reports on the Desolation Canyon, Turtle Canyon, and Floy Canyon Wilderness Study Areas which include 242,000 acres, 33,690 acres, and 23,140 acres. Coal deposits underlie all three study areas. Coal zones in the Blackhawk and Nelsen formations have identified bituminous coal resources of 22 million short tons in the Desolation Canyon Study Area, 6.3 million short tons in the Turtle Canyon Study Area, and 45 million short tons in the Floy Canyon Study Area. In-place inferred oil shale resources are estimated to contain 60 million barrels in the northern part of the Desolation Canyon area. Minor occurrences of uranium have been found in the southeastern part of the Desolation Canyon area and in the western part of the Floy Canyon area. Mineral resource potential for the study areas is estimated to be for coal, high for all areas, for oil and gas, high for the northern tract of the Desolation Canyon area and moderate for all other tracts, for bituminous sandstone, high for the northern part of the Desolation Canyon area, and low for all other tracts, for oil shale, low in all areas, for uranium, moderate for the Floy Canyon area and the southeastern part of the Desolation Canyon area and low for the remainder of the areas, for metals other than uranium, bentonite, zeolites, and geothermal energy, low in all areas, and for coal-bed methane unknown in all three areas

  19. Formative flow in bedrock canyons

    Science.gov (United States)

    Venditti, J. G.; Kwoll, E.; Rennie, C. D.; Church, M. A.

    2017-12-01

    In alluvial channels, it is widely accepted that river channel configuration is set by a formative flow that represents a balance between the magnitude and frequency of flood flows. The formative flow is often considered to be one that is just capable of filling a river channel to the top of its banks. Flows much above this formative flow are thought to cause substantial sediment transport and rearrange the channel morphology to accommodate the larger flow. This idea has recently been extended to semi-alluvial channels where it has been shown that even with bedrock exposed, the flows rarely exceed that required to entrain the local sediment cover. What constitutes a formative flow in a bedrock canyon is not clear. By definition, canyons have rock walls and are typically incised vertically, removing the possibility of the walls being overtopped, as can occur in an alluvial channel at high flows. Canyons are laterally constrained, have deep scour pools and often have width to maximum depth ratios approaching 1, an order of magnitude lower than alluvial channels. In many canyons, there are a sequence of irregularly spaced scour pools. The bed may have intermittent or seasonal sediment cover, but during flood flows the sediment bed is entrained leaving a bare bedrock channel. It has been suggested that canyons cut into weak, well-jointed rock may adjust their morphology to the threshold for block plucking because the rock bed is labile during exceptionally large magnitude flows. However, this hypothesis does not apply to canyons cut into massive crystalline rock where abrasion is the dominant erosion process. Here, we argue that bedrock canyon morphology is adjusted to a characteristic flow structure developed in bedrock canyons. We show that the deeply scoured canyon floor is adjusted to a velocity inversion that is present at low flows, but gets stronger at high flows. The effect is to increase boundary shear stresses along the scour pool that forms in constricted

  20. Insulin and IGF-1 improve mitochondrial function in a PI-3K/Akt-dependent manner and reduce mitochondrial generation of reactive oxygen species in Huntington's disease knock-in striatal cells.

    Science.gov (United States)

    Ribeiro, Márcio; Rosenstock, Tatiana R; Oliveira, Ana M; Oliveira, Catarina R; Rego, A Cristina

    2014-09-01

    Oxidative stress and mitochondrial dysfunction have been described in Huntington's disease, a disorder caused by expression of mutant huntingtin (mHtt). IGF-1 was previously shown to protect HD cells, whereas insulin prevented neuronal oxidative stress. In this work we analyzed the role of insulin and IGF-1 in striatal cells derived from HD knock-in mice on mitochondrial production of reactive oxygen species (ROS) and related antioxidant and signaling pathways influencing mitochondrial function. Insulin and IGF-1 decreased mitochondrial ROS induced by mHtt and normalized mitochondrial SOD activity, without affecting intracellular glutathione levels. IGF-1 and insulin promoted Akt phosphorylation without changing the nuclear levels of phosphorylated Nrf2 or Nrf2/ARE activity. Insulin and IGF-1 treatment also decreased mitochondrial Drp1 phosphorylation, suggesting reduced mitochondrial fragmentation, and ameliorated mitochondrial function in HD cells in a PI-3K/Akt-dependent manner. This was accompanied by increased total and phosphorylated Akt, Tfam, and mitochondrial-encoded cytochrome c oxidase II, as well as Tom20 and Tom40 in mitochondria of insulin- and IGF-1-treated mutant striatal cells. Concomitantly, insulin/IGF-1-treated mutant cells showed reduced apoptotic features. Hence, insulin and IGF-1 improve mitochondrial function and reduce mitochondrial ROS caused by mHtt by activating the PI-3K/Akt signaling pathway, in a process independent of Nrf2 transcriptional activity, but involving enhanced mitochondrial levels of Akt and mitochondrial-encoded complex IV subunit. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Diablo Canyon ECCS enhancements

    International Nuclear Information System (INIS)

    Lin, A.; Lee, T.P.; Walter, L.E.

    2004-01-01

    Diablo Canyon Power Plant (DCPP) operated by Pacific Gas and Electric Co. (PG and E) is a Westinghouse designed four loop plant. In recent years, several issues were identified regarding the compliance of the Emergency Core Cooling System (ECCS) surveillance tests to the ECCS analyses assumptions. These concerns are related mostly to the High Head Safety Injection (HHSI) and the Intermediate Head Safety Injection (IHSI) systems where the injection line throttle valves are adjusted during outage surveillance testing to ensure compliance with the Technical Specifications (TS). To resolve all of the identified issues PG and E performed an ECCS reanalysis and upgraded the ECCS surveillance test program and also had Westinghouse perform a containment reanalysis using their latest model. As a result of these plant specific enhancement efforts, DCPP widened the operating window for TS surveillance testing, lowered the ECCS pumps' acceptance performance curves, and re-gained Peak Clad Temperature (PCT) and containment peak pressure margins. These enhancements are generically applicable to other plants and are addressed in this paper. (author)

  2. The Wilson films--Huntington's chorea.

    Science.gov (United States)

    Klein, Christine

    2011-12-01

    Wilson's Queen Square Case 9 with Huntington's chorea shows a 68-year-old man with mild to moderate generalized chorea, impaired fixation, and probable cognitive decline in keeping with a diagnosis of Huntington's disease (HD). An age of onset in the late sixties and a negative family history suggest a relatively small expanded trinucleotide repeat in the HTT gene in the patient and reduced penetrance of an even shorter repeat allele in one of his parents. A highly sensitive and specific gene test has been offered worldwide for diagnostic testing of HD for almost two decades. This test, obviously unavailable at Wilson's times, became the historic frontrunner for guidelines of symptomatic, presymptomatic, and prenatal testing for an adult-onset neurodegenerative disorder. Regarding treatment of HD, however, we are still awaiting the successful translation of research results into the development of effective cause-directed, neuropreventive and neurorestaurative therapies. Copyright © 2011 Movement Disorder Society.

  3. [Sporadic juvenile forms of Huntington's chorea].

    Science.gov (United States)

    Zinchenko, A P; Goncharov, V D; Burtianskii, D L; Zakhar'ev, Iu M

    1980-01-01

    Six patients with Huntington's chorea in the age of 15-24 years old, suffered from diffusive choreic hyperkynesis with slowly progressive dementia. The development of this disease in childhood and adolescence was atypical, as nobody in the family and in kin sufferred from it and it was difficult to diagnose the disease. Recognition of the disease was promoted by pneumoencephalography, electromyography and memory investigation.

  4. Earliest functional declines in Huntington disease

    Science.gov (United States)

    Beglinger, Leigh J.; O'Rourke, Justin J.F.; Wang, Chiachi; Langbehn, Douglas R.; Duff, Kevin; Paulsen, Jane S.

    2013-01-01

    We examined the gold standard for Huntington disease (HD) functional assessment, the Unified Huntington's Disease Rating Scale (UHDRS), in a group of at-risk participants not yet diagnosed but who later phenoconverted to manifest HD. We also sought to determine which skill domains first weaken and the clinical correlates of declines. Using the UHDRS Total Functional Capacity (TFC) and Functional Assessment Scale (FAS), we examined participants from Huntington Study Group clinics who were not diagnosed at their baseline visit but were diagnosed at a later visit (N = 265). Occupational decline was the most common with 65.1% (TFC) and 55.6% (FAS) reporting some loss of ability to engage in their typical work. Inability to manage finances independently (TFC 49.2%, FAS 35.1%) and drive safely (FAS 33.5%) were also found. Functional decline was significantly predicted by motor, cognitive, and depressive symptoms. The UHDRS captured early functional losses in individuals with HD prior to formal diagnosis, however, fruitful areas for expanded assessment of early functional changes are performance at work, ability to manage finances, and driving. These are also important areas for clinical monitoring and treatment planning as up to 65% experienced loss in at least one area prior to diagnosis. PMID:20471695

  5. Deepwater Canyons 2013: Pathways to the Abyss

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Leg I focused on biological objectives in Norfolk Canyon, with some sampling in Baltimore Canyon. Leg II focused on archaeological targets in and around the Norfolk...

  6. 78 FR 48670 - Boulder Canyon Project

    Science.gov (United States)

    2013-08-09

    ... DEPARTMENT OF ENERGY Western Area Power Administration Boulder Canyon Project AGENCY: Western Area... Canyon Project (BCP) electric service provided by the Western Area Power Administration (Western). The... INFORMATION: Hoover Dam, authorized by the Boulder Canyon Project Act (45 Stat. 1057, December 21, 1928), sits...

  7. 77 FR 48151 - Boulder Canyon Project

    Science.gov (United States)

    2012-08-13

    ... DEPARTMENT OF ENERGY Western Area Power Administration Boulder Canyon Project AGENCY: Western Area... Canyon Project (BCP) electric service provided by the Western Area Power Administration (Western). The... INFORMATION: Hoover Dam, authorized by the Boulder Canyon Project Act (45 Stat. 1057, December 21, 1928), sits...

  8. Clinical and genetic study of a juvenile-onset Huntington disease

    Directory of Open Access Journals (Sweden)

    HAO Ying

    2012-06-01

    Full Text Available Background Huntington's disease (HD is an autosomal dominant hereditary progressive neurodegenerative disorder with a distinct phenotype characterized by chorea, dementia, cognitive and affective impairment. There are selective neural cell loss and atrophy in the caudate and putamen. Dr. George Huntington firstly described the disease accurately and insightfully, which led to a widespread recognition of the inherited chorea that now bears his name. Huntington disease gene (IT15 locus on chromosome 4p16.3, and encompasses 67 exons with a trinucleotide repeat (CAG in the first exon. The CAG repeat length is highly polymorphic in the population and expanded on at least one chromosome of individuals with HD. Clinically, patient with HD are often onset in adulthood. Juvenile-onset HD is relatively rare. Adult-onset HD patients usually have a CAG expansion from 40 to 55 whereas those with juvenile-onset greater than 60 which are often inherited from the father. We investigated the clinical features of a juvenile-onset case with Huntington disease and dynamic mutation of his family. Methods The CAG repeats of IT15 gene were detected using polymerase chain reaction and capillary electrophoresis in 115 individuals with preliminary diagnosis as Huntington disease. The repeat numbers of some samples carried expanded or intermediate alleles were verified by the pMD18-T vector clone sequencing. Results Fragment analysis showed that one juvenile-onset case presenting with cognitive dysfunction and hypokinesis carried 15/68 CAG repeats of IT15. His father carried 17/37 and mother carried 15/17. Conclusion 1 The juvenile-onset case of HD presented with different clinical features compared with adult-onset cases. The typical signs of adult-onset cases include progressive chorea, rigidity and dementia. The most common sign of juvenile-onset Huntington disease is cognitive decline. 2 The dynamic mutation of IT15 gene expansion of the CAG repeats in the

  9. Clinical and genetic data of Huntington disease in Moroccan patients

    African Journals Online (AJOL)

    Background: Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10 /100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Methods: Clinical ...

  10. Regional economic impacts of Grand Canyon river runners.

    Science.gov (United States)

    Hjerpe, Evan E; Kim, Yeon-Su

    2007-10-01

    Economic impact analysis (EIA) of outdoor recreation can provide critical social information concerning the utilization of natural resources. Outdoor recreation and other non-consumptive uses of resources are viewed as environmentally friendly alternatives to extractive-type industries. While outdoor recreation can be an appropriate use of resources, it generates both beneficial and adverse socioeconomic impacts on rural communities. The authors used EIA to assess the regional economic impacts of rafting in Grand Canyon National Park. The Grand Canyon region of northern Arizona represents a rural US economy that is highly dependent upon tourism and recreational expenditures. The purpose of this research is twofold. The first is to ascertain the previously unknown regional economic impacts of Grand Canyon river runners. The second purpose is to examine attributes of these economic impacts in terms of regional multipliers, leakage, and types of employment created. Most of the literature on economic impacts of outdoor recreation has focused strictly on the positive economic impacts, failing to illuminate the coinciding adverse and constraining economic impacts. Examining the attributes of economic impacts can highlight deficiencies and constraints that limit the economic benefits of recreation and tourism. Regional expenditure information was obtained by surveying non-commercial boaters and commercial outfitters. The authors used IMPLAN input-output modeling to assess direct, indirect, and induced effects of Grand Canyon river runners. Multipliers were calculated for output, employment, and income. Over 22,000 people rafted on the Colorado River through Grand Canyon National Park in 2001, resulting in an estimated $21,100,000 of regional expenditures to the greater Grand Canyon economy. However, over 50% of all rafting-related expenditures were not captured by the regional economy and many of the jobs created by the rafting industry are lower-wage and seasonal. Policy

  11. Durable terrestrial bedrock predicts submarine canyon formation

    Science.gov (United States)

    Smith, Elliot; Finnegan, Noah J.; Mueller, Erich R.; Best, Rebecca J.

    2017-01-01

    Though submarine canyons are first-order topographic features of Earth, the processes responsible for their occurrence remain poorly understood. Potentially analogous studies of terrestrial rivers show that the flux and caliber of transported bedload are significant controls on bedrock incision. Here we hypothesize that coarse sediment load could exert a similar role in the formation of submarine canyons. We conducted a comprehensive empirical analysis of canyon occurrence along the West Coast of the contiguous United States which indicates that submarine canyon occurrence is best predicted by the occurrence of durable crystalline bedrock in adjacent terrestrial catchments. Canyon occurrence is also predicted by the flux of bed sediment to shore from terrestrial streams. Surprisingly, no significant correlation was observed between canyon occurrence and the slope or width of the continental shelf. These findings suggest that canyon incision is promoted by greater yields of durable terrestrial clasts to the shore.

  12. Submarine canyons off Madras Coast

    Digital Repository Service at National Institute of Oceanography (India)

    Setty, M.G.A.P.

    Submarine canyons off the coast of Madras, Tamil Nadu, India were studied during cruise of @iINS Kistna@@ as part of the IIOE programme They consist of hill-like projections and V-shaped valleys Their other features are also reported...

  13. Big Canyon Creek Ecological Restoration Strategy.

    Energy Technology Data Exchange (ETDEWEB)

    Rasmussen, Lynn; Richardson, Shannon

    2007-10-01

    then used data collected from the District's stream assessment and inventory, utilizing the Stream Visual Assessment Protocol (SVAP), to determine treatment necessary to bring 90% of reaches ranked Poor or Fair through the SVAP up to good or excellent. In 10 year's time, all reaches that were previously evaluated with SVAP will be reevaluated to determine progress and to adapt methods for continued success. Over 400 miles of stream need treatment in order to meet identified restoration goals. Treatments include practices which result in riparian habitat improvements, nutrient reductions, channel condition improvements, fish habitat improvements, invasive species control, water withdrawal reductions, improved hydrologic alterations, upland sediment reductions, and passage barrier removal. The Nez Perce Soil and Water Conservation District (District) and the Nez Perce Tribe Department of Fisheries Resource Management Watershed Division (Tribe) developed this document to guide restoration activities within the Big Canyon Creek watershed for the period of 2008-2018. This plan was created to demonstrate the ongoing need and potential for anadromous fish habitat restoration within the watershed and to ensure continued implementation of restoration actions and activities. It was developed not only to guide the District and the Tribe, but also to encourage cooperation among all stakeholders, including landowners, government agencies, private organizations, tribal governments, and elected officials. Through sharing information, skills, and resources in an active, cooperative relationships, all concerned parties will have the opportunity to join together to strengthen and maintain a sustainable natural resource base for present and future generations within the watershed. The primary goal of the strategy is to address aquatic habitat restoration needs on a watershed level for resident and anadromous fish species, promoting quality habitat within a self

  14. Near-inertial motions in the DeSoto Canyon during Hurricane Georges

    Science.gov (United States)

    Jordi, Antoni; Wang, Dong-Ping; Hamilton, Peter

    2016-09-01

    Hurricane Georges passed directly over an array of 13 moorings deployed in the DeSoto Canyon in the northern Gulf of Mexico on 27-28 September 1998. Current velocity data from the mooring array were analyzed together with a primitive-equation model simulation with realistic hurricane forcing, to characterize the generation and propagation of the hurricane-generated near-inertial waves. The model successfully reproduces the observed mean (sub-inertial) and near-inertial motions. The upper ocean response is strongly impacted by the canyon 'wall': a strong jet is formed along the slope, and the near-inertial motions on the shelf are rapidly suppressed. The model results moreover suggest that strong near-inertial waves in the mixed layer are mostly trapped in an energy flux recirculating gyre around the canyon. This gyre retains the near-inertial energy in the canyon region and enhances the transfer of near-inertial energy below the mixed layer. Additional simulations with idealized topographies show that the presence of a steep slope rather than the canyon is fundamental for the generation of this recirculating gyre. The near-inertial wave energy budget shows that during the study period the wind generated an input of 6.79 × 10-2 Wm-2 of which about 1/3, or 2.43 × 10-2 Wm-2, was transferred below the mixed layer. The horizontal energy flux into and out of the canyon region, in contrast, was relatively weak.

  15. High Protein Diet and Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Chiung-Mei Chen

    Full Text Available Huntington's disease (HD is a neurodegenerative disorder caused by the huntingtin (HTT gene with expanded CAG repeats. In addition to the apparent brain abnormalities, impairments also occur in peripheral tissues. We previously reported that mutant Huntingtin (mHTT exists in the liver and causes urea cycle deficiency. A low protein diet (17% restores urea cycle activity and ameliorates symptoms in HD model mice. It remains unknown whether the dietary protein content should be monitored closely in HD patients because the normal protein consumption is lower in humans (~15% of total calories than in mice (~22%. We assessed whether dietary protein content affects the urea cycle in HD patients. Thirty HD patients were hospitalized and received a standard protein diet (13.7% protein for 5 days, followed by a high protein diet (HPD, 26.3% protein for another 5 days. Urea cycle deficiency was monitored by the blood levels of citrulline and ammonia. HD progression was determined by the Unified Huntington's Disease Rating Scale (UHDRS. The HPD increased blood citrulline concentration from 15.19 μmol/l to 16.30 μmol/l (p = 0.0378 in HD patients but did not change blood ammonia concentration. A 2-year pilot study of 14 HD patients found no significant correlation between blood citrulline concentration and HD progression. Our results indicated a short period of the HPD did not markedly compromise urea cycle function. Blood citrulline concentration is not a reliable biomarker of HD progression.

  16. Huntington disease: Experimental models and therapeutic perspectives

    International Nuclear Information System (INIS)

    Serrano Sanchez, Teresa; Blanco Lezcano, Lisette; Garcia Minet, Rocio; Alberti Amador, Esteban; Diaz Armesto, Ivan and others

    2011-01-01

    Huntington's disease (HD) is a degenerative dysfunction of hereditary origin. Up to date there is not, an effective treatment to the disease which having lapsed 15 or 20 years advances inexorably, in a slow form, toward the total inability or death. This paper reviews the clinical and morphological characteristics of Huntington's disease as well as the experimental models more commonly used to study this disease, having as source the articles indexed in Medline data base, published in the last 20 years. Advantages and disadvantages of all experimental models to reproduce the disease as well as the perspectives to therapeutic assay have been also considered. the consent of outline reported about the toxic models, those induced by neurotoxins such as quinolinic acid, appears to be the most appropriate to reproduce the neuropathologic characteristic of the disease, an genetic models contributing with more evidence to the knowledge of the disease etiology. Numerous treatments ameliorate clinical manifestations, but none of them has been able to stop or diminish the affectations derived from neuronal loss. At present time it is possible to reproduce, at least partially, the characteristics of the disease in experimentation animals that allow therapy evaluation in HD. from the treatment view point, the more promissory seems to be transplantation of no neuronal cells, taking into account ethical issues and factibility. On the other hand the new technology of interference RNA emerges as a potential therapeutic tool for treatment in HD, and to respond basic questions on the development of the disease.

  17. Evaluation of turbulence from traffic using experimental data obtained in a street canyon

    Energy Technology Data Exchange (ETDEWEB)

    Mazzeo, N.A.; Venegas, L.E. [Univ. of Buenos Aires, Buenos Aires (Argentina). Dept. of Atmospheric and Oceanic Sciences, National Scientific and Technological Research Council

    2004-07-01

    High air pollution levels have been observed in street canyons. Within these streets, pedestrians, cyclists, drivers and residents are likely to be exposed to pollutant concentrations exceeding current air quality standards. Airflow and dispersion in street canyons are very complicated. Depending on the synoptic wind three main dispersion conditions can be identified: a) low wind conditions, b) perpendicular or near perpendicular flow for winds over 1.5-2.0 m/s blowing at an angle of more than 30 to the canyon axes, c) parallel or near parallel flow for winds over 1.5-2.0 m/s blowing from all other directions. Under condition b), airflow in canyons with H/W{approx}1 (H is the height and W is the width of the canyon) is characterised by the formation of a single vortex within the canyon. The dispersion of gaseous pollutants in a street canyon depends generally on the rate at which the street exchanges air vertically with the above roof-level atmosphere and laterally with connecting streets. There is evidence that when the synoptic wind speed is low, the mechanical traffic-produced turbulence (TPT) might place a significant role in dispersion of traffic-generated pollutants. In this paper, we analyse interactions between wind and traffic induced dispersive air motions. Data from full-scale measurements in Goettinger Strasse (Hannover, Germany) are used for application of parameterisation proposed by Di Sabatino, S. et al. (2003) and Kastner-Klein, P. et al. (2003). (orig.)

  18. O desenvolvimento político em Huntington e Fukuyama Huntington and Fukuyama on political development

    Directory of Open Access Journals (Sweden)

    Natália Nóbrega de Mello

    2010-01-01

    Full Text Available O artigo contrasta as teses de Huntington e Fukuyama sobre desenvolvimento político. As obras analisadas, Ordem política nas sociedades em mudança e O fim da história, inscrevem-se entre duas conjunturas decisivas - 1968 e 1989. Huntington desmontou a equivalência entre desenvolvimento político e modernização e Fukuyama reafirmou a democracia como o destino de todos os países e, desse modo, como o fim da história. Nesta comparação, dois eixos se sobressaem: o contexto de produção das obras e a alternância entre os polos teóricos da democracia e da estabilidade. Procura-se demonstrar como, apesar de reinserir a democracia no desenvolvimento político, a instabilidade continua a ser um foco privilegiado de análise no pensamento de Fukuyama.The article contrasts the theories of Huntington and Fukuyama on political development. The analyzed works, Political order in changing societies and The end of history, fall between two decisive historical moments - in 1968 and 1989. Huntington disassembled the equivalence between political development and modernization; Fukuyama reaffirmed democracy as the destiny of all countries and, as such, it is the end of history. In this comparison, two axes call our attention: the production context of these works and the alternation between the theoreticals poles of democracy and stability. The article shows how, although reenters democracy in the political development theory, instablility remains a prime focus of analysis in Fukuyama's thought.

  19. Outage risk reduction at Diablo Canyon

    International Nuclear Information System (INIS)

    Burnett, Tobias W.T.; Eugene Newman, C.

    2004-01-01

    A formal risk reduction program was conducted at the Diablo Canyon Nuclear Generating plant as part of EPRI's Outage Risk Assessment and Management Program. The program began with a probabilistic and deterministic assessment of the frequency of core coolant boiling and core uncovery during shutdown operations. This step identified important contributors to risk, periods of high vulnerability, and potential mechanisms for reducing risk. Next, recovery strategies were evaluated and procedures, training, and outage schedules modified. Twelve risk reduction enhancements were developed and implemented. These enhancements and their impact are described in this paper. These enhancements reduced the calculated risk of core uncovery by about a factor of four for a refueling outage without lengthening the outage schedule; increased the outage efficiency, contributing to completing 11 days ahead of schedule; and helped to earn the highest achievable SALP rating from the NRC. (author)

  20. Marble Canyon spring sampling investigation

    International Nuclear Information System (INIS)

    McCulley, B.

    1985-10-01

    The Mississippian Leadville Limestone is the most permeable formation in the lower hydrostratigraphic unit underlying the salt beds of the Paradox Formation in Gibson Dome, Paradox Basin, Utah, which is being considered as a potential nuclear waste repository site. The closest downgradient outcrop of the Mississippian limestone is along the Colorado River in Marble Canyon, Arizona. This report describes the sampling and interpretation of springs in that area to assess the relative contribution of Gibson Dome-type Leadville Limestone ground water to that spring discharge. The high-volume (hundreds of liters per second or thousands of gallons per minute) springs discharging from fault zones in Marble Canyon are mixtures of water recharged west of the Colorado River on the Kaibab Plateau and east of the river in the Kaiparowits basin. No component of Gibson Dome-type Leadville Limestone ground water is evident in major and trace element chemistry or isotopic composition of the Marble Canyon Springs. A low-volume (0.3 liters per second or 5 gallons per minute) spring with some chemical and isotopic characteristics of Gibson Dome-type Leadville Limestone water diluted by Kaiparowits basin-type water issues from a travertine mound in the Bright Angel Shale on the Little Colorado River. However, the stable isotopic composition and bromide levels of that spring discharge, in addition to probable ground-water flow paths, contradict the dilution hypothesis

  1. Américo Negrette and Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Moscovich

    2011-08-01

    Full Text Available The authors present a historical review of the seminal clinical contribution of Professor Américo Negrette, a Venezuelan neurologist, to the evolution of scientific knowledge about Huntington's disease.

  2. Unravelling and Exploiting Astrocyte Dysfunction in Huntington's Disease

    DEFF Research Database (Denmark)

    Khakh, Baljit S.; Beaumont, Vahri; Cachope, Roger

    2017-01-01

    Astrocytes are abundant within mature neural circuits and are involved in brain disorders. Here, we summarize our current understanding of astrocytes and Huntington's disease (HD), with a focus on correlative and causative dysfunctions of ion homeostasis, calcium signaling, and neurotransmitter...

  3. Contribution of Neuroepigenetics to Huntington's Disease.

    Science.gov (United States)

    Francelle, Laetitia; Lotz, Caroline; Outeiro, Tiago; Brouillet, Emmanuel; Merienne, Karine

    2017-01-01

    Unbalanced epigenetic regulation is thought to contribute to the progression of several neurodegenerative diseases, including Huntington's disease (HD), a genetic disorder considered as a paradigm of epigenetic dysregulation. In this review, we attempt to address open questions regarding the role of epigenetic changes in HD, in the light of recent advances in neuroepigenetics. We particularly discuss studies using genome-wide scale approaches that provide insights into the relationship between epigenetic regulations, gene expression and neuronal activity in normal and diseased neurons, including HD neurons. We propose that cell-type specific techniques and 3D-based methods will advance knowledge of epigenome in the context of brain region vulnerability in neurodegenerative diseases. A better understanding of the mechanisms underlying epigenetic changes and of their consequences in neurodegenerative diseases is required to design therapeutic strategies more effective than current strategies based on histone deacetylase (HDAC) inhibitors. Researches in HD may play a driving role in this process.

  4. Destination and source memory in Huntington's disease.

    Science.gov (United States)

    El Haj, Mohamad; Caillaud, Marie; Verny, Christophe; Fasotti, Luciano; Allain, Philippe

    2016-03-01

    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults had to put 12 items in a black or a white box (destination task), and to extract another 12 items from a blue or a red box (source task). Afterwards, they had to decide in which box each item had previously been deposited (destination memory), and from which box each item had previously been extracted (source memory). HD participants showed poorer source as well as destination recall performance than healthy adults in the proposed tasks. Correlation analysis showed that destination recall was significantly correlated with episodic recall in HD participants. Destination memory impairment in HD participants seems to be considerably influenced by their episodic memory performance. © 2014 The British Psychological Society.

  5. Magnetic biomineralisation in Huntington's disease transgenic mice

    International Nuclear Information System (INIS)

    Beyhum, W; Hautot, D; Dobson, J; Pankhurst, Q A

    2005-01-01

    The concentration levels of biogenic magnetite nanoparticles in transgenic R6/2 Huntington's disease (HD) mice have been investigated, using seven control and seven HD mice each from an 8 week-old litter and from a 12 week-old litter. Hysteresis and isothermal remnant magnetisation data were collected on a SQUID magnetometer, and analysed using a model comprising dia/paramagnetic, ferrimagnetic and superparamagnetic contributions, to extract the magnetite and ferritin concentrations present. It was found that magnetite was present in both superparamagnetic and blocked states. A larger spread and higher concentration of magnetite levels was found in the diseased mice for both the 8 week-old and 12 week-old batches, compared to the controls

  6. The impact of traffic-flow patterns on air quality in urban street canyons

    International Nuclear Information System (INIS)

    Thaker, Prashant; Gokhale, Sharad

    2016-01-01

    We investigated the effect of different urban traffic-flow patterns on pollutant dispersion in different winds in a real asymmetric street canyon. Free-flow traffic causes more turbulence in the canyon facilitating more dispersion and a reduction in pedestrian level concentration. The comparison of with and without a vehicle-induced-turbulence revealed that when winds were perpendicular, the free-flow traffic reduced the concentration by 73% on the windward side with a minor increase of 17% on the leeward side, whereas for parallel winds, it reduced the concentration by 51% and 29%. The congested-flow traffic increased the concentrations on the leeward side by 47% when winds were perpendicular posing a higher risk to health, whereas reduced it by 17–42% for parallel winds. The urban air quality and public health can, therefore, be improved by improving the traffic-flow patterns in street canyons as vehicle-induced turbulence has been shown to contribute significantly to dispersion. - Highlights: • CFD is used to study impact of traffic-flow patterns on urban air quality. • Facilitating free-flow patterns induce more turbulence in street canyons. • Traffic-generated turbulence alters pollutant levels in urban street canyons. - This study investigates the effect of vehicle-induced-turbulence generated during free-flow traffic pattern in reduction of air pollutant concentrations in urban street canyons.

  7. Progressive Impairment of Lactate-based Gluconeogenesis in the Huntington?s Disease Mouse Model R6/2

    OpenAIRE

    Nielsen, Signe Marie Borch; Hasholt, Lis; N?rrem?lle, Anne; Josefsen, Knud

    2015-01-01

    Huntington?s disease (HD) is a neurodegenerative illness, where selective neuronal loss in the brain caused by expression of mutant huntingtin protein leads to motor dysfunction and cognitive decline in addition to peripheral metabolic changes. In this study we confirm our previous observation of impairment of lactate-based hepatic gluconeogenesis in the transgenic HD mouse model R6/2 and determine that the defect manifests very early and progresses in severity with disease development, indic...

  8. 78 FR 7775 - Boulder Canyon Project

    Science.gov (United States)

    2013-02-04

    ... DEPARTMENT OF ENERGY Western Area Power Administration Boulder Canyon Project AGENCY: Western Area...), is proposing an adjustment to the Boulder Canyon Project (BCP) electric service base charge and rates... subsequent laws, particularly section 9(c) of the Reclamation Project Act of 1939 (43 U.S.C. 485h(c)); and...

  9. 77 FR 2533 - Boulder Canyon Project

    Science.gov (United States)

    2012-01-18

    ... DEPARTMENT OF ENERGY Western Area Power Administration Boulder Canyon Project AGENCY: Western Area...), is proposing an adjustment to the Boulder Canyon Project (BCP) electric service base charge and rates...) of the Reclamation Project Act of 1939 (43 U.S.C. 485h(c)); and other acts that specifically apply to...

  10. 76 FR 56430 - Boulder Canyon Project

    Science.gov (United States)

    2011-09-13

    ... DEPARTMENT OF ENERGY Western Area Power Administration Boulder Canyon Project AGENCY: Western Area... Project (BCP) electric service provided by the Western Area Power Administration (Western). The Rates will... by the Boulder Canyon Project Act (45 Stat. 1057, December 21, 1928), sits on the Colorado River...

  11. 76 FR 8359 - Boulder Canyon Project

    Science.gov (United States)

    2011-02-14

    ... DEPARTMENT OF ENERGY Western Area Power Administration Boulder Canyon Project AGENCY: Western Area... Western Area Power Administration (Western) is proposing an adjustment to the Boulder Canyon Project (BCP... Reclamation Project Act of 1939 (43 U.S.C. 485h(c)), and other acts that specifically apply to the project...

  12. Grand Canyon Monitoring and Research Center

    Science.gov (United States)

    Hamill, John F.

    2009-01-01

    The Grand Canyon of the Colorado River, one of the world's most spectacular gorges, is a premier U.S. National Park and a World Heritage Site. The canyon supports a diverse array of distinctive plants and animals and contains cultural resources significant to the region's Native Americans. About 15 miles upstream of Grand Canyon National Park sits Glen Canyon Dam, completed in 1963, which created Lake Powell. The dam provides hydroelectric power for 200 wholesale customers in six western States, but it has also altered the Colorado River's flow, temperature, and sediment-carrying capacity. Over time this has resulted in beach erosion, invasion and expansion of nonnative species, and losses of native fish. Public concern about the effects of Glen Canyon Dam operations prompted the passage of the Grand Canyon Protection Act of 1992, which directs the Secretary of the Interior to operate the dam 'to protect, mitigate adverse impacts to, and improve values for which Grand Canyon National Park and Glen Canyon National Recreation Area were established...' This legislation also required the creation of a long-term monitoring and research program to provide information that could inform decisions related to dam operations and protection of downstream resources.

  13. Environmental assessment: Davis Canyon site, Utah

    Energy Technology Data Exchange (ETDEWEB)

    none,

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high- level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. These evaluations were reported in draft environmental assessments (EAs), which were issued for public review and comment. After considering the comments received on the draft EAs, the DOE prepared the final EA. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. This setting contains one other potentially acceptable site -- the Lavender Canyon site. Although the Lavender Canyon site is suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. Furthermore, the DOE has found that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of the five sites suitable for characterization.

  14. Environmental assessment: Davis Canyon site, Utah

    Energy Technology Data Exchange (ETDEWEB)

    none,

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. These evaluations were reported in draft environmental assessments (EAs), which were issued for public review and comment. After considering the comments received on the draft EAs, the DOE prepared the final EA. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considering for the first repository. This setting contains one other potentially acceptable site -- the Lavender Canyon site. Although the Lavender Canyon site is suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. Furthermore, the DOE has found that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of five sites suitable for characterization.

  15. Environmental assessment: Davis Canyon site, Utah

    International Nuclear Information System (INIS)

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. These evaluations were reported in draft environmental assessments (EAs), which were issued for public review and comment. After considering the comments received on the draft EAs, the DOE prepared the final EA. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considering for the first repository. This setting contains one other potentially acceptable site -- the Lavender Canyon site. Although the Lavender Canyon site is suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. Furthermore, the DOE has found that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of five sites suitable for characterization

  16. Environmental assessment: Davis Canyon site, Utah

    International Nuclear Information System (INIS)

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high- level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. These evaluations were reported in draft environmental assessments (EAs), which were issued for public review and comment. After considering the comments received on the draft EAs, the DOE prepared the final EA. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. This setting contains one other potentially acceptable site -- the Lavender Canyon site. Although the Lavender Canyon site is suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. Furthermore, the DOE has found that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of the five sites suitable for characterization

  17. Bell Canyon test summary report

    International Nuclear Information System (INIS)

    Christensen, C.L.; Peterson, E.W.

    1981-04-01

    The Bell Canyon Test was an in situ evaluation of the ability of a cement grout plug to seal boreholes. It consisted of a 2-m-long, 20-cm-diameter grout plug in an anhydrite formation at a depth of 1370 m, directly above an aquifer that provided a 12.4 MPa (1800 psi) differential pressure. The aquifer had a production capability of 38,000 l/day (240 bbl/day, 10 4 gal/day). The observed leakage after plug installation was 0.6 l/day, which is equivalent to a 50 microdarcy flow path assuming all flow occurred through the plug cross-sectional area. Laboratory results and analysis of field data indicate that the bulk of the flow occurred through a microstructure at the interface between the plug and the host rock. The Bell Canyon Test demonstrated that a plug could be formulated, emplaced, and tested under actual conditions and provide acceptable performance. When these results are related to the WIPP performance assessment models, they provide additional confidence that borehole plugging can be accomplished satisfactorily. The Bell Canyon results can also be used as basis for future activities in the generic repository sealing program for similar emplacements and performance assessment evaluations. If the observed leakage rates are not acceptable at other sites, the BCT results would indicate that the first step in improving such emplacements should deal with improved bonding of the plug to the rock at these sites. The results obtained from the BCT, when coupled with results from long-term durability assessments, form a plug performance data basis for repository designers at other proposed waste repository sites

  18. H-Canyon Recovery Crawler

    Energy Technology Data Exchange (ETDEWEB)

    Kriikku, E. M. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL); Hera, K. R. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL); Marzolf, A. D. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL); Phillips, M. H. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL)

    2015-08-01

    The Nuclear Material Disposition Project group asked the Savannah River National Lab (SRNL) Research and Development Engineering (R&DE) department to help procure, test, and deploy a remote crawler to recover the 2014 Inspection Crawler (IC) that tipped over in the H-Canyon Air Exhaust Tunnel. R&DE wrote a Procurement Specification for a Recovery Crawler (RC) and SRNS Procurement Department awarded the contract to Power Equipment Manufacturing Inc. (PEM). The PEM RC was based on their standard sewer inspection crawler with custom arms and forks added to the front. The arms and forks would be used to upright the 2014 Inspection Crawler. PEM delivered the RC and associated cable reel, 2014 Inspection Crawler mockup, and manuals in late April 2015. R&DE and the team tested the crawler in May of 2015 and made modifications based on test results and Savannah River Site (SRS) requirements. R&DE delivered the RC to H-Area at the end of May. The team deployed the RC on June 9, 10, and 11, 2015 in the H-Canyon Air Exhaust Tunnel. The RC struggled with some obstacles in the tunnel, but eventually made it to the IC. The team spent approximately five hours working to upright the IC and eventually got it on its wheels. The IC travelled approximately 20 feet and struggled to drive over debris on the air tunnel floor. Unfortunately the IC tripped over trying to pass this obstacle. The team decided to leave the IC in this location and inspect the tunnel with the RC. The RC passed the IC and inspected the tunnel as it travelled toward H-Canyon. The team turned the RC around when it was about 20 feet from the H-Canyon crossover tunnel. From that point, the team drove the RC past the manway towards the new sand filter and stopped approximately 20 feet from the new sand filter. The team removed the RC from the tunnel, decontaminated the RC, and stored it the manway building, 294-2H. The RC deployment confirmed the IC was not in a condition to perform useful tunnel inspections and

  19. Environmental assessment overview, Davis Canyon site, Utah

    International Nuclear Information System (INIS)

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of five sites suitable for characterization. 3 figs

  20. Total recognition discriminability in Huntington's and Alzheimer's disease.

    Science.gov (United States)

    Graves, Lisa V; Holden, Heather M; Delano-Wood, Lisa; Bondi, Mark W; Woods, Steven Paul; Corey-Bloom, Jody; Salmon, David P; Delis, Dean C; Gilbert, Paul E

    2017-03-01

    Both the original and second editions of the California Verbal Learning Test (CVLT) provide an index of total recognition discriminability (TRD) but respectively utilize nonparametric and parametric formulas to compute the index. However, the degree to which population differences in TRD may vary across applications of these nonparametric and parametric formulas has not been explored. We evaluated individuals with Huntington's disease (HD), individuals with Alzheimer's disease (AD), healthy middle-aged adults, and healthy older adults who were administered the CVLT-II. Yes/no recognition memory indices were generated, including raw nonparametric TRD scores (as used in CVLT-I) and raw and standardized parametric TRD scores (as used in CVLT-II), as well as false positive (FP) rates. Overall, the patient groups had significantly lower TRD scores than their comparison groups. The application of nonparametric and parametric formulas resulted in comparable effect sizes for all group comparisons on raw TRD scores. Relative to the HD group, the AD group showed comparable standardized parametric TRD scores (despite lower raw nonparametric and parametric TRD scores), whereas the previous CVLT literature has shown that standardized TRD scores are lower in AD than in HD. Possible explanations for the similarity in standardized parametric TRD scores in the HD and AD groups in the present study are discussed, with an emphasis on the importance of evaluating TRD scores in the context of other indices such as FP rates in an effort to fully capture recognition memory function using the CVLT-II.

  1. Therapeutic approaches to preventing cell death in Huntington disease.

    Science.gov (United States)

    Kaplan, Anna; Stockwell, Brent R

    2012-12-01

    Neurodegenerative diseases affect the lives of millions of patients and their families. Due to the complexity of these diseases and our limited understanding of their pathogenesis, the design of therapeutic agents that can effectively treat these diseases has been challenging. Huntington disease (HD) is one of several neurological disorders with few therapeutic options. HD, like numerous other neurodegenerative diseases, involves extensive neuronal cell loss. One potential strategy to combat HD and other neurodegenerative disorders is to intervene in the execution of neuronal cell death. Inhibiting neuronal cell death pathways may slow the development of neurodegeneration. However, discovering small molecule inhibitors of neuronal cell death remains a significant challenge. Here, we review candidate therapeutic targets controlling cell death mechanisms that have been the focus of research in HD, as well as an emerging strategy that has been applied to developing small molecule inhibitors-fragment-based drug discovery (FBDD). FBDD has been successfully used in both industry and academia to identify selective and potent small molecule inhibitors, with a focus on challenging proteins that are not amenable to traditional high-throughput screening approaches. FBDD has been used to generate potent leads, pre-clinical candidates, and has led to the development of an FDA approved drug. This approach can be valuable for identifying modulators of cell-death-regulating proteins; such compounds may prove to be the key to halting the progression of HD and other neurodegenerative disorders. Copyright © 2012 Elsevier Ltd. All rights reserved.

  2. Validation of a two-dimensional pollutant dispersion model in an isolated street canyon

    Energy Technology Data Exchange (ETDEWEB)

    Chan, T.L.; Dong, G.; Leung, C.W.; Cheung, C.S. [The Hong Kong Polytechnic University, Kowloon (Hong Kong). Research Centre for Combustion and Pollution Control, Department of Mechanical Engineering; Hung, W.T. [The Hong Kong Polytechnic University, Kowloon (Hong Kong). Department of Civil and Structural Engineering

    2002-07-01

    A two-dimensional numerical model based on Reynolds-averaged Navier-Stokes equations coupled with a series of standard, Renormalization Group (RNG) and realizable k-{epsilon} turbulence models was developed to simulate the fluid-flow development and pollutant dispersion within an isolated street canyon using the FLUENT code. In the present study, the validation of the numerical model was evaluated using an extensive experimental database obtained from the atmospheric boundary layer wind tunnel at the Meteorological Institute of Hamburg University, Germany (J. Wind Eng. Ind. Aerodyn. 62 (1996) 37). Among the studied turbulence models, the RNG k-{epsilon} turbulence model was found to be the most optimum turbulence model coupled with the two-dimensional street canyon model developed in the present study. Both the calculated and measured dimensionless pollutant concentrations have been shown to be less dependent on the variation of wind speed and source strength conditions for the studied street canyon aspect ratio of the B/H=1 case. However, the street canyon configuration has significant influence on the pollutant dispersion. The wider street and lower height of the buildings are favorable to pollutant dilution within the street canyon. The fluid-flow development has demonstrated that the rotative vortex or vortices generated within the urban street canyon can transport the pollutants from a line source to the wall surfaces of the buildings. (author)

  3. Prehistoric deforestation at Chaco Canyon?

    Science.gov (United States)

    Wills, W H; Drake, Brandon L; Dorshow, Wetherbee B

    2014-08-12

    Ancient societies are often used to illustrate the potential problems stemming from unsustainable land-use practices because the past seems rife with examples of sociopolitical "collapse" associated with the exhaustion of finite resources. Just as frequently, and typically in response to such presentations, archaeologists and other specialists caution against seeking simple cause-and effect-relationships in the complex data that comprise the archaeological record. In this study we examine the famous case of Chaco Canyon, New Mexico, during the Bonito Phase (ca. AD 860-1140), which has become a prominent popular illustration of ecological and social catastrophe attributed to deforestation. We conclude that there is no substantive evidence for deforestation at Chaco and no obvious indications that the depopulation of the canyon in the 13th century was caused by any specific cultural practices or natural events. Clearly there was a reason why these farming people eventually moved elsewhere, but the archaeological record has not yet produced compelling empirical evidence for what that reason might have been. Until such evidence appears, the legacy of Ancestral Pueblo society in Chaco should not be used as a cautionary story about socioeconomic failures in the modern world.

  4. Carbon transport in Monterey Submarine Canyon

    Science.gov (United States)

    Barry, J.; Paull, C. K.; Xu, J. P.; Clare, M. A.; Gales, J. A.; Buck, K. R.; Lovera, C.; Gwiazda, R.; Maier, K. L.; McGann, M.; Parsons, D. R.; Simmons, S.; Rosenberger, K. J.; Talling, P. J.

    2017-12-01

    Submarine canyons are important conduits for sediment transport from continental margins to the abyss, but the rate, volume, and time scales of material transport have been measured only rarely. Using moorings with current meters, sediment traps (10 m above bottom) and optical backscatter sensors, we measured near-bottom currents, suspended sediment concentrations, and sediment properties at 1300 m depth in Monterey Canyon and at a non-canyon location on the continental slope at the same depth. Flow and water column backscatter were used to characterize "ambient" conditions when tidal currents dominated the flow field, and occasional "sediment transport events" when anomalously high down-canyon flow with sediment-laden waters arrived at the canyon mooring. The ambient sediment flux measured in sediment traps in Monterey Canyon was 350 times greater than measured at the non-canyon location. Although the organic carbon content of the canyon sediment flux during ambient periods was low (1.8 %C) compared to the slope location (4.9 %C), the ambient carbon transport in the canyon was 130 times greater than at the non-canyon site. Material fluxes during sediment transport events were difficult to measure owing to clogging of sediment traps, but minimal estimates indicate that mass transport during events exceeds ambient sediment fluxes through the canyon by nearly 3 orders of magnitude, while carbon transport is 380 times greater. Estimates of the instantaneous and cumulative flux of sediment and carbon from currents, backscatter, and sediment properties indicated that: 1) net flux is down-canyon, 2) flux is dominated by sediment transport events, and 3) organic carbon flux through 1300 m in Monterey Canyon was ca. 1500 MT C per year. The injection of 1500 MTCy-1 into the deep-sea represents ca. 260 km2 of the sediment C flux measured at the continental slope station (5.8 gCm-2y-1) and is sufficient to support a benthic community carbon demand of 5 gCm-2y-1 over 300 km2.

  5. Internal tides affect benthic community structure in an energetic submarine canyon off SW Taiwan

    Science.gov (United States)

    Liao, Jian-Xiang; Chen, Guan-Ming; Chiou, Ming-Da; Jan, Sen; Wei, Chih-Lin

    2017-07-01

    other hand, rebounded more rapidly due to their fast growth rate and short generation time and thus did not display bathymetric pattern in the canyon. To our knowledge, this is the first benthic ecological study in a submarine canyon connected to a high-sediment-yield SMR. The biological responses to extreme physical conditions in the GPSC could have broad implications on understanding the anthropogenic and climate change impacts in the deep-sea ecosystems.

  6. Megabenthic assemblages at the Hudson Canyon head (NW Atlantic margin): Habitat-faunal relationships

    Science.gov (United States)

    Pierdomenico, Martina; Gori, Andrea; Guida, Vincent G.; Gili, Josep-Maria

    2017-09-01

    The distribution of megabenthic communities at the head of Hudson Canyon and adjacent continental shelf was studied by means of underwater video transects and still photo imagery collected using a towed camera system. The goal was to explore the relationships between faunal distribution and physical seafloor conditions and to test the hypothesis that increased seafloor heterogeneity in the Hudson Canyon supports a larger diversity of benthic communities, compared with the adjacent continental shelf. Hierarchical cluster analysis was performed to identify benthic assemblages as defined in imagery. The BIO-ENV procedure and the Canonical Correspondence Analysis were carried out to elucidate species groupings in relation to terrain variables extracted from bathymetric data. Species accumulation curves were generated to evaluate species turn over in and out of Hudson Canyon. The results indicate that seafloor morphology is the main physical factor related to benthic community composition and distribution. Assemblages dominated by sponges, zoanthids and cup corals colonized the canyon margins and flanks, and were associated with coarse-grained sediments, while sea pen assemblages were observed along muddy seafloor within the thalweg. An assemblage dominated by sea stars occurred on the shelf, associated with a sandy seafloor. Some assemblages were exclusively observed in the canyon area, suggesting that the increased variability of seafloor composition, together with the oceanographic processes specific to the canyon area, enhance beta diversity. The colonization by benthic suspension feeders within the canyon, in contrast to shelf assemblages, mainly composed of carnivores and detritus feeders could be favored the intense hydrodynamics at the canyon head that increase the availability of suspended organic matter. From the perspective of management and conservation of marine resources, the results obtained support the relevance of Hudson Canyon as a biodiversity hotspot

  7. 75 FR 13454 - Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY

    Science.gov (United States)

    2010-03-22

    ...-AA08 Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY AGENCY: Coast... navigable waters of Huntington Bay, New York due to the annual Fran Schnarr Open Water Championships. The..., ``Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY'' (Docket number USCG...

  8. 75 FR 38710 - Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY

    Science.gov (United States)

    2010-07-06

    ...-AA08 Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY AGENCY: Coast... Regulation on the navigable waters of Huntington Bay, New York due to the annual Fran Schnarr Open Water... ``Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY'' in the Federal...

  9. Changes in mental state and behaviour in Huntington's disease.

    Science.gov (United States)

    Eddy, Clare M; Parkinson, Ellice G; Rickards, Hugh E

    2016-11-01

    Changes in mental state and behaviour have been acknowledged in Huntington's disease since the original monograph in 1872 provided evidence of disinhibition and impaired social cognition. Behavioural problems can manifest before obvious motor symptoms and are frequently the most disabling part of the illness. Although pharmacological treatments are used routinely for psychiatric difficulties in Huntington's disease, the scientific evidence base for their use is somewhat sparse. Moreover, effective treatments for apathy and cognitive decline do not currently exist. Understanding the social cognitive impairments associated with Huntington's disease can assist management, but related therapeutic interventions are needed. Future research should aim to design rating scales for behaviour and mental state in Huntington's disease that can detect change in clinical trials. Generally, communication and understanding of behaviour and mental state in Huntington's would be enhanced by a clear conceptual framework that unifies ideas around movement, cognition, emotion, behaviour, and mental state, reflecting both the experience of the patient and their underlying neuropathology. Copyright © 2016 Elsevier Ltd. All rights reserved.

  10. Street canyon aerosol pollutant transport measurements.

    Science.gov (United States)

    Longley, I D; Gallagher, M W; Dorsey, J R; Flynn, M; Bower, K N; Allan, J D

    2004-12-01

    Current understanding of dispersion in street canyons is largely derived from relatively simple dispersion models. Such models are increasingly used in planning and regulation capacities but are based upon a limited understanding of the transport of substances within a real canyon. In recent years, some efforts have been made to numerically model localised flow in idealised canyons (e.g., J. Appl. Meteorol. 38 (1999) 1576-89) and stepped canyons (Assimakopoulos V. Numerical modelling of dispersion of atmospheric pollution in and above urban canopies. PhD thesis, Imperial College, London, 2001) but field studies in real canyons are rare. To further such an understanding, a measurement campaign has been conducted in an asymmetric street canyon with busy one-way traffic in central Manchester in northern England. The eddy correlation method was used to determine fluxes of size-segregated accumulation mode aerosol. Measurements of aerosol at a static location were made concurrently with measurements on a platform lift giving vertical profiles. Size-segregated measurements of ultrafine and coarse particle concentrations were also made simultaneously at various heights. In addition, a small mobile system was used to make measurements of turbulence at various pavement locations within the canyon. From this data, various features of turbulent transport and dispersion in the canyon will be presented. The concentration and the ventilation fluxes of vehicle-related aerosol pollutants from the canyon will be related to controlling factors. The results will also be compared with citywide ventilation data from a separate measurement campaign conducted above the urban canopy.

  11. Haplotype-based stratification of Huntington's disease.

    Science.gov (United States)

    Chao, Michael J; Gillis, Tammy; Atwal, Ranjit S; Mysore, Jayalakshmi Srinidhi; Arjomand, Jamshid; Harold, Denise; Holmans, Peter; Jones, Lesley; Orth, Michael; Myers, Richard H; Kwak, Seung; Wheeler, Vanessa C; MacDonald, Marcy E; Gusella, James F; Lee, Jong-Min

    2017-11-01

    Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by expansion of a CAG trinucleotide repeat in HTT, resulting in an extended polyglutamine tract in huntingtin. We and others have previously determined that the HD-causing expansion occurs on multiple different haplotype backbones, reflecting more than one ancestral origin of the same type of mutation. In view of the therapeutic potential of mutant allele-specific gene silencing, we have compared and integrated two major systems of HTT haplotype definition, combining data from 74 sequence variants to identify the most frequent disease-associated and control chromosome backbones and revealing that there is potential for additional resolution of HD haplotypes. We have used the large collection of 4078 heterozygous HD subjects analyzed in our recent genome-wide association study of HD age at onset to estimate the frequency of these haplotypes in European subjects, finding that common genetic variation at HTT can distinguish the normal and CAG-expanded chromosomes for more than 95% of European HD individuals. As a resource for the HD research community, we have also determined the haplotypes present in a series of publicly available HD subject-derived fibroblasts, induced pluripotent cells, and embryonic stem cells in order to facilitate efforts to develop inclusive methods of allele-specific HTT silencing applicable to most HD patients. Our data providing genetic guidance for therapeutic gene-based targeting will significantly contribute to the developments of rational treatments and implementation of precision medicine in HD.

  12. Diablo Canyon refueling outage program

    International Nuclear Information System (INIS)

    McLane, W.B.; Irving, T.L.

    1991-01-01

    Management of outages has become one of the most talked about subjects in the nuclear power industry in the past several years. Many utilities do not perform refueling outages very well or in the past have had some outages that they would not like to repeat and in some cases do not even like to think about. With the growing cost of energy and the demands placed on utilities to improve capacity factors, it is very easy for management to focus on shortening refueling outage durations as a prime objective in improving overall corporate performance. So it is with Pacific Gas and Electric Company and the Diablo Canyon power plant. A review of their refueling outage performance reflects a utility that is responding to the nuclear industry's call for improved outage performance

  13. Environmental assessment: Davis Canyon site, Utah

    International Nuclear Information System (INIS)

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. These evaluations were reported in draft environmental assessments (EAs), which were issued for public review and comment. After considering the comments received on the draft EAs, the DOE prepared the final EA. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. This setting contains one other potentially acceptable site -- the Lavender Canyon site. Although the Lavender Canyon site is suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. Furthermore, the DOE has fond that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of five sites suitable for characterization. 181 figs., 175 tabs

  14. Environmental assessment: Davis Canyon site, Utah

    Energy Technology Data Exchange (ETDEWEB)

    none,

    1986-05-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah as one of the nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for the Nuclear Waste Repositories. These evaluations were reported in draft environmental assessments (EAs), which were issued for public review and comment. After considering the comments received on the draft EAs, the DOE prepared the final EA. The Davis Canyon site is in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. This setting contains one other potentially acceptable site -- the Lavender Canyon site. Although the Lavender Canyon site is suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of the evaluations reported in this EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. Furthermore, the DOE has fond that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is nominating the Davis Canyon site as one of five sites suitable for characterization. 181 figs., 175 tabs.

  15. Flow and Pollutant Transport in Urban Street Canyons of Different Aspect Ratios with Ground Heating: Large-Eddy Simulation

    Science.gov (United States)

    Li, Xian-Xiang; Britter, Rex E.; Norford, Leslie K.; Koh, Tieh-Yong; Entekhabi, Dara

    2012-02-01

    A validated large-eddy simulation model was employed to study the effect of the aspect ratio and ground heating on the flow and pollutant dispersion in urban street canyons. Three ground-heating intensities (neutral, weak and strong) were imposed in street canyons of aspect ratio 1, 2, and 0.5. The detailed patterns of flow, turbulence, temperature and pollutant transport were analyzed and compared. Significant changes of flow and scalar patterns were caused by ground heating in the street canyon of aspect ratio 2 and 0.5, while only the street canyon of aspect ratio 0.5 showed a change in flow regime (from wake interference flow to skimming flow). The street canyon of aspect ratio 1 does not show any significant change in the flow field. Ground heating generated strong mixing of heat and pollutant; the normalized temperature inside street canyons was approximately spatially uniform and somewhat insensitive to the aspect ratio and heating intensity. This study helps elucidate the combined effects of urban geometry and thermal stratification on the urban canyon flow and pollutant dispersion.

  16. A Numerical Study on the Effects of Street‒canyon Aspect‒ratio on Reactive Pollutant Dispersion

    Science.gov (United States)

    Park, S. J.; Kim, J.

    2014-12-01

    In this study, the effects of street‒canyon aspect‒ratio on reactive pollutant dispersion were investigated using the coupled CFD‒chemistry model. For this, flow characteristics were analyzed first in street canyons with different aspect ratios and flow regimes were classified according to the building height. For each flow regime, dispersion characteristics were investigated in views of reactive pollutant concentration and VOCs‒NOX ratio. Finally, the relations between pollutant concentration and aspect ratio in urban street canyons were investigated. In the case of H/S = 1.0 (H is building height and S is street width), one clockwise‒rotating vortex appeared vertically and the reverse and outward flows were dominant near the street bottom. In the case of H/S = 2.0, two counter‒rotating vortices appeared vertically in the street canyon. The primary (secondary) vortex rotating clockwise (counterclockwise) was formed in upper (lower) layer. The flow patterns affected the reactive pollutant concentration in street canyons. As building height increased, mean concentration of NO decreased when one vortex was generated in street canyons and increased when two vortexes appeared in street canyons. O3 concentration showed almost contrasted tendency with those of NO because O3 was depleted by the NO titration.

  17. Embodied emotion impairment in Huntington's Disease.

    Science.gov (United States)

    Trinkler, Iris; Devignevielle, Sévérine; Achaibou, Amal; Ligneul, Romain V; Brugières, Pierre; Cleret de Langavant, Laurent; De Gelder, Beatrice; Scahill, Rachael; Schwartz, Sophie; Bachoud-Lévi, Anne-Catherine

    2017-07-01

    Theories of embodied cognition suggest that perceiving an emotion involves somatovisceral and motoric re-experiencing. Here we suggest taking such an embodied stance when looking at emotion processing deficits in patients with Huntington's Disease (HD), a neurodegenerative motor disorder. The literature on these patients' emotion recognition deficit has recently been enriched by some reports of impaired emotion expression. The goal of the study was to find out if expression deficits might be linked to a more motoric level of impairment. We used electromyography (EMG) to compare voluntary emotion expression from words to emotion imitation from static face images, and spontaneous emotion mimicry in 28 HD patients and 24 matched controls. For the latter two imitation conditions, an underlying emotion understanding is not imperative (even though performance might be helped by it). EMG measures were compared to emotion recognition and to the capacity to identify and describe emotions using alexithymia questionnaires. Alexithymia questionnaires tap into the more somato-visceral or interoceptive aspects of emotion perception. Furthermore, we correlated patients' expression and recognition scores to cerebral grey matter volume using voxel-based morphometry (VBM). EMG results replicated impaired voluntary emotion expression in HD. Critically, voluntary imitation and spontaneous mimicry were equally impaired and correlated with impaired recognition. By contrast, alexithymia scores were normal, suggesting that emotion representations on the level of internal experience might be spared. Recognition correlated with brain volume in the caudate as well as in areas previously associated with shared action representations, namely somatosensory, posterior parietal, posterior superior temporal sulcus (pSTS) and subcentral sulcus. Together, these findings indicate that in these patients emotion deficits might be tied to the "motoric level" of emotion expression. Such a double

  18. Wintertime Boundary Layer Structure in the Grand Canyon.

    Science.gov (United States)

    Whiteman, C. David; Zhong, Shiyuan; Bian, Xindi

    1999-08-01

    Wintertime temperature profiles in the Grand Canyon exhibit a neutral to isothermal stratification during both daytime and nighttime, with only rare instances of actual temperature inversions. The canyon warms during daytime and cools during nighttime more or less uniformly through the canyon's entire depth. This weak stability and temperature structure evolution differ from other Rocky Mountain valleys, which develop strong nocturnal inversions and exhibit convective and stable boundary layers that grow upward from the valley floor. Mechanisms that may be responsible for the different behavior of the Grand Canyon are discussed, including the possibility that the canyon atmosphere is frequently mixed to near-neutral stratification when cold air drains into the top of the canyon from the nearby snow-covered Kaibab Plateau. Another feature of canyon temperature profiles is the sharp inversions that often form near the canyon rims. These are generally produced when warm air is advected over the canyon in advance of passing synoptic-scale ridges.Wintertime winds in the main canyon are not classical diurnal along-valley wind systems. Rather, they are driven along the canyon axis by the horizontal synoptic-scale pressure gradient that is superimposed along the canyon's axis by passing synoptic-scale weather disturbances. They may thus bring winds into the canyon from either end at any time of day.The implications of the observed canyon boundary layer structure for air pollution dispersion are discussed.

  19. Tremor in neurodegenerative ataxias, Huntington disease and tic disorder.

    Science.gov (United States)

    Rudzińska, M; Krawczyk, M; Wójcik-Pędziwiatr, M; Szczudlik, A; Tomaszewski, T

    2013-01-01

    Tremor is the most prevalent movement disorder, defined as rhythmic oscillations of a body part, caused by alternating or synchronic contractions of agonistic or antagonistic muscles. The aim of the study was to assess prevalence and to characterize parameters of tremor accompanying de-generative ataxias, Huntington disease (HD) and tic disorders in comparison with a control group. Forty-three patients with degenerative ataxias, 28 with HD and 26 with tic disorders together with 51 healthy controls were included in the study. For each participant, clinical and instrumental assessment (accelerometer, electromyography [EMG], graphic tablet) of hand tremor was performed. Frequency and severity of tremor were assessed in three positions: at rest (rest tremor), with hands extended (postural tremor), during the 'finger-to-nose' test and during Archimedes spiral drawing (kinetic tremor). Based on the mass load test, the type of tremor was determined as essential tremor type or enhanced physiological tremor type. The incidence of tremor in the accelerometry in patients with degenerative ataxia (50%) significantly differs from controls (10%) (p = 0.001). The dominant tremor was postural, low-intense, with 7-Hz frequency, essential tremor (23%) or other tremor type (23%), while enhanced physiological tremor was the least frequent (2%). Tremor in patients with HD and tic disorders was found in 10% and 20% of patients, respectively, similarly to the control group. Tremor was mild, postural and of essential tremor type, less frequently of enhanced physiological tremor type. No correlation between severity of tremor and severity of disease was found. The prevalence of tremor is considerably higher among patients with degenerative ataxias compared with HD, tic disorder and the control group. The most common type of tremor accompanying ataxias, HD and tic disorders is essential tremor type.

  20. An improved assay for the determination of Huntington`s disease allele size

    Energy Technology Data Exchange (ETDEWEB)

    Reeves, C.; Klinger, K.; Miller, G. [Intergrated Genetics, Framingham, MA (United States)

    1994-09-01

    The hallmark of Huntington`s disease (HD) is the expansion of a polymorphic (CAG)n repeat. Several methods have been published describing PCR amplification of this region. Most of these assays require a complex PCR reaction mixture to amplify this GC-rich region. A consistent problem with trinucleotide repeat PCR amplification is the presence of a number of {open_quotes}stutter bands{close_quotes} which may be caused by primer or amplicon slippage during amplification or insufficient polymerase processivity. Most assays for HD arbitrarily select a particular band for diagnostic purposes. Without a clear choice for band selection such an arbitrary selection may result in inconsistent intra- or inter-laboratory findings. We present an improved protocol for the amplification of the HD trinucleotide repeat region. This method simplifies the PCR reaction buffer and results in a set of easily identifiable bands from which to determine allele size. HD alleles were identified by selecting bands of clearly greater signal intensity. Stutter banding was much reduced thus permitting easy identification of the most relevant PCR product. A second set of primers internal to the CCG polymorphism was used in selected samples to confirm allele size. The mechanism of action of N,N,N trimethylglycine in the PCR reaction is not clear. It may be possible that the minimal isostabilizing effect of N,N,N trimethylglycine at 2.5 M is significant enough to affect primer specificity. The use of N,N,N trimethylglycine in the PCR reaction facilitated identification of HD alleles and may be appropriate for use in other assays of this type.

  1. Compilation of PRF Canyon Floor Pan Sample Analysis Results

    Energy Technology Data Exchange (ETDEWEB)

    Pool, Karl N. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Minette, Michael J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Wahl, Jon H. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Greenwood, Lawrence R. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Coffey, Deborah S. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); McNamara, Bruce K. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Bryan, Samuel A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Scheele, Randall D. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Delegard, Calvin H. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Sinkov, Sergey I. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Soderquist, Chuck Z. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Fiskum, Sandra K. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Brown, Garrett N. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Clark, Richard A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2016-06-30

    On September 28, 2015, debris collected from the PRF (236-Z) canyon floor, Pan J, was observed to exhibit chemical reaction. The material had been transferred from the floor pan to a collection tray inside the canyon the previous Friday. Work in the canyon was stopped to allow Industrial Hygiene to perform monitoring of the material reaction. Canyon floor debris that had been sealed out was sequestered at the facility, a recovery plan was developed, and drum inspections were initiated to verify no additional reactions had occurred. On October 13, in-process drums containing other Pan J material were inspected and showed some indication of chemical reaction, limited to discoloration and degradation of inner plastic bags. All Pan J material was sealed back into the canyon and returned to collection trays. Based on the high airborne levels in the canyon during physical debris removal, ETGS (Encapsulation Technology Glycerin Solution) was used as a fogging/lock-down agent. On October 15, subject matter experts confirmed a reaction had occurred between nitrates (both Plutonium Nitrate and Aluminum Nitrate Nonahydrate (ANN) are present) in the Pan J material and the ETGS fixative used to lower airborne radioactivity levels during debris removal. Management stopped the use of fogging/lock-down agents containing glycerin on bulk materials, declared a Management Concern, and initiated the Potential Inadequacy in the Safety Analysis determination process. Additional drum inspections and laboratory analysis of both reacted and unreacted material are planned. This report compiles the results of many different sample analyses conducted by the Pacific Northwest National Laboratory on samples collected from the Plutonium Reclamation Facility (PRF) floor pans by the CH2MHill’s Plateau Remediation Company (CHPRC). Revision 1 added Appendix G that reports the results of the Gas Generation Rate and methodology. The scope of analyses requested by CHPRC includes the determination of

  2. Remifentanil in a patient with Huntington's chorea - case report ...

    African Journals Online (AJOL)

    Relatively few published case reports related to the anaesthetic management of Huntington's chorea (HC) exist. At the time of surgery no publications were found related to remifentanil's use in patients with HC. This case report describes the management of a confirmed HC patient requiring urgent decompression of a spinal ...

  3. PSYCHIATRIC ASPECTS OF HUNTINGTON DISEASE – CASE REPORTS

    Directory of Open Access Journals (Sweden)

    Mirela Batta

    2004-04-01

    Full Text Available Background. Huntington disease occurrs rarely, it can be encountered not only by neurologists and psychiatrists but also by other medical practitioners. Its characteristic features are involuntary movements, cognitive disorders and gradual development of dementia. Diagnosis is given on the basis of these clinical features, positive familial anamnesis, with the laboratory exclusion of other neuropsychiatric diseases and with the help of neuroimaging methods (in particular NMR. The disease can be only confirmed by means of genetic analysis.Patients and methods. In this article, four cases of patients with Huntington disease and diverse psychiatric disorders that were hospitalised at the psychiatric department of the Maribor General Hospital between October 2002 and March 2003 are described. All the patients fulfilled the valid criteria for the diagnosis of Huntington disease. However, they differed according to their accompanying psychiatric psychopathology, age and social problems.Conclusions. The purpose of this article is to draw attention to different psychiatric symptoms and clinical manifestations of Huntington disease that are often misleading in the diagnostic process. In addition, exigency of early diagnostics, guidelines for referrals to genetic testing and psychiatric monitoring of these patients are emphasised.

  4. Ethical issues and Huntington's disease | Kromberg | South African ...

    African Journals Online (AJOL)

    The practice of genetic counselling gives rise to many ethical dilemmas, and counsellors need to be familiar with the principles of biomedical ethics. The primary principles include respect for autonomy, beneficence, non-maleficence and justice. A case of identical twins at 50% risk for Huntington's disease, in which only one ...

  5. The emotional experiences of family carers in Huntington disease.

    Science.gov (United States)

    Williams, Janet K; Skirton, Heather; Paulsen, Jane S; Tripp-Reimer, Toni; Jarmon, Lori; McGonigal Kenney, Meghan; Birrer, Emily; Hennig, Bonnie L; Honeyford, Joann

    2009-04-01

    This paper is a report of a study conducted to examine the emotional experience of caregiving by family carers of people with Huntington disease and to describe strategies they used to deal with that experience. Huntington disease, commonly diagnosed in young to middle adulthood, is an inherited single gene disorder involving loss of cognitive, motor and neuropsychiatric function. Many family members become caregivers as well as continuing as parents and wage earners. The emotional aspects of caregiving contribute to mental health risks for family members. Focus groups were conducted with 42 adult carers of people with Huntington disease in four United States and two Canadian Huntington disease centers between 2001 and 2005. Data were analyzed through descriptive coding and thematic analysis. All participants reported multiple aspects of emotional distress. Being a carer was described as experiencing disintegration of one's life. Carers attempted to cope by seeking comfort from selected family members, anticipating the time when the care recipient had died and/or using prescription medications. Spousal carers were distressed by the loss of their relationship with their spouse and dealt with this by no longer regarding the person as an intimate partner. Carers were concerned about the disease risk for children in their families and hoped for a cure. Emotional distress can compromise the well-being of family carers, who attempt to maintain multiple roles. Nurses should monitor carer mental health, identify sources of emotional distress and support effective strategies used by carers to mediate distress.

  6. Major Superficial White Matter Abnormalities in Huntington's Disease

    Science.gov (United States)

    Phillips, Owen R.; Joshi, Shantanu H.; Squitieri, Ferdinando; Sanchez-Castaneda, Cristina; Narr, Katherine; Shattuck, David W.; Caltagirone, Carlo; Sabatini, Umberto; Di Paola, Margherita

    2016-01-01

    Background: The late myelinating superficial white matter at the juncture of the cortical gray and white matter comprising the intracortical myelin and short-range association fibers has not received attention in Huntington's disease. It is an area of the brain that is late myelinating and is sensitive to both normal aging and neurodegenerative disease effects. Therefore, it may be sensitive to Huntington's disease processes. Methods: Structural MRI data from 25 Pre-symptomatic subjects, 24 Huntington's disease patients and 49 healthy controls was run through a cortical pattern-matching program. The surface corresponding to the white matter directly below the cortical gray matter was then extracted. Individual subject's Diffusion Tensor Imaging (DTI) data was aligned to their structural MRI data. Diffusivity values along the white matter surface were then sampled at each vertex point. DTI measures with high spatial resolution across the superficial white matter surface were then analyzed with the General Linear Model to test for the effects of disease. Results: There was an overall increase in the axial and radial diffusivity across much of the superficial white matter (p < 0.001) in Pre-symptomatic subjects compared to controls. In Huntington's disease patients increased diffusivity covered essentially the whole brain (p < 0.001). Changes are correlated with genotype (CAG repeat number) and disease burden (p < 0.001). Conclusions: This study showed broad abnormalities in superficial white matter even before symptoms are present in Huntington's disease. Since, the superficial white matter has a unique microstructure and function these abnormalities suggest it plays an important role in the disease. PMID:27242403

  7. Effects of Building‒roof Cooling on Flow and Distribution of Reactive Pollutants in street canyons

    Science.gov (United States)

    Park, S. J.; Choi, W.; Kim, J.; Jeong, J. H.

    2016-12-01

    The effects of building‒roof cooling on flow and dispersion of reactive pollutants were investigated in the framework of flow dynamics and chemistry using a coupled CFD‒chemistry model. For this, flow characteristics were analyzed first in street canyons in the presence of building‒roof cooling. A portal vortex was generated in street canyon, producing dominant reverse and outward flows near the ground in all the cases. The building‒roof cooling increased horizontal wind speeds at the building roof and strengthened the downward motion near the downwind building in the street canyon, resultantly intensifying street canyon vortex strength. The flow affected the distribution of primary and secondary pollutants. Concentrations of primary pollutants such as NOx, VOC and CO was high near the upwind building because the reverse flows were dominant at street level, making this area the downwind region of emission sources. Concentration of secondary pollutant such as O3 was lower than the background near the ground, where NOX concentrations were high. Building‒roof cooling decreased the concentration of primary pollutants in contrasted to those under non‒cooling conditions. In contrast, building‒roof cooling increased O3 by reducing NO concentrations in urban street canyon compared to concentrations under non‒cooling conditions.

  8. Contemporary sediment-transport processes in submarine canyons.

    Science.gov (United States)

    Puig, Pere; Palanques, Albert; Martín, Jacobo

    2014-01-01

    Submarine canyons are morphological incisions into continental margins that act as major conduits of sediment from shallow- to deep-sea regions. However, the exact mechanisms involved in sediment transfer within submarine canyons are still a subject of investigation. Several studies have provided direct information about contemporary sedimentary processes in submarine canyons that suggests different modes of transport and various triggering mechanisms. Storm-induced turbidity currents and enhanced off-shelf advection, hyperpycnal flows and failures of recently deposited fluvial sediments, dense shelf-water cascading, canyon-flank failures, and trawling-induced resuspension largely dominate present-day sediment transfer through canyons. Additionally, internal waves periodically resuspend ephemeral deposits within canyons and contribute to dispersing particles or retaining and accumulating them in specific regions. These transport processes commonly deposit sediments in the upper- and middle-canyon reaches for decades or centuries before being completely or partially flushed farther down-canyon by large sediment failures.

  9. Red Rock Canyon National Conservation Area Transportation Feasibility Study

    Science.gov (United States)

    2012-07-31

    Red Rock Canyon National Conservation Area is a popular Bureau of Land Management natural area located near Las Vegas, Nevada. Red Rock Canyon experiences heavy congestion on its Scenic Drive and associated parking areas, due to high volumes of visit...

  10. Factor analysis of the hospital anxiety and depression scale among a Huntington's disease population

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Martucci, Rossana

    2015-01-01

    INTRODUCTION: Depression and anxiety are common in Huntington's disease, a genetic neurodegenerative disorder. There is a need for measurement tools of mood to be validated within a Huntington's disease population. The current study aimed to analyze the factor structure of the Hospital Anxiety...... and Depression Scale in Huntington's disease. METHODS: Data from the European Huntington's Disease Network study REGISTRY 3 were used to undertake a factor analysis of the scale among a sample of 492 Huntington's disease mutation carriers. The sample was randomly divided into two equal subsamples...... support for an eight-item version of the scale to be used as a measure of general distress within Huntington's disease populations. © 2015 International Parkinson and Movement Disorder Society....

  11. Disease stage, but not sex, predicts depression and psychological distress in Huntington's disease

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Shimozaki, Steve

    2016-01-01

    OBJECTIVE: Depression and anxiety significantly affect morbidity in Huntington's disease. Mice. models of Huntington's disease have identified sex differences in mood-like behaviours that vary across disease lifespan, but this interaction has not previously been explored in humans with Huntington......'s disease. However, among certain medical populations, evidence of sex differences in mood across various disease stages has been found, reflecting trends among the general population that women tend to experience anxiety and depression 1.5 to 2 times more than men. The current study examined whether...... disease stage and sex, either separately or as an interaction term, predicted anxiety and depression in Huntington's disease. METHODS: A cross-sectional study of REGISTRY data involving 453 Huntington's disease participants from 12 European countries was undertaken using the Hospital Anxiety...

  12. 77 FR 22801 - Glen Canyon Dam Adaptive Management Work Group

    Science.gov (United States)

    2012-04-17

    ... DEPARTMENT OF THE INTERIOR Bureau of Reclamation Glen Canyon Dam Adaptive Management Work Group...). SUMMARY: The Glen Canyon Dam Adaptive Management Work Group (AMWG) makes recommendations to the Secretary..., the AMWG, a technical work group, a Grand Canyon Monitoring and Research Center, and independent...

  13. Crossing fitness canyons by a finite population

    Science.gov (United States)

    Saakian, David B.; Bratus, Alexander S.; Hu, Chin-Kun

    2017-06-01

    We consider the Wright-Fisher model of the finite population evolution on a fitness landscape defined in the sequence space by a path of nearly neutral mutations. We study a specific structure of the fitness landscape: One of the intermediate mutations on the mutation path results in either a large fitness value (climbing up a fitness hill) or a low fitness value (crossing a fitness canyon), the rest of the mutations besides the last one are neutral, and the last sequence has much higher fitness than any intermediate sequence. We derive analytical formulas for the first arrival time of the mutant with two point mutations. For the first arrival problem for the further mutants in the case of canyon crossing, we analytically deduce how the mean first arrival time scales with the population size and fitness difference. The location of the canyon on the path of sequences has a crucial role. If the canyon is at the beginning of the path, then it significantly prolongs the first arrival time; otherwise it just slightly changes it. Furthermore, the fitness hill at the beginning of the path strongly prolongs the arrival time period; however, the hill located near the end of the path shortens it. We optimize the first arrival time by applying a nonzero selection to the intermediate sequences. We extend our results and provide a scaling for the valley crossing time via the depth of the canyon and population size in the case of a fitness canyon at the first position. Our approach is useful for understanding some complex evolution systems, e.g., the evolution of cancer.

  14. Hydrogeology of Middle Canyon, Oquirrh Mountains, Tooele County, Utah

    Science.gov (United States)

    Gates, Joseph Spencer

    1963-01-01

    Geology and climate are the principal influences affecting the hydrology of Middle Canyon, Tooele County, Utah. Reconnaissance in the canyon indicated that the geologic influences on the hydrology may be localized; water may be leaking through fault and fracture zones or joints in sandstone and through solution openings in limestone of the Oquirrh formation of Pennsylvanian and Permian age. Surficial deposits of Quaternary age serve as the main storage material for ground water in the canyon and transmit water from the upper canyon to springs and drains at the canyon mouth. The upper canyon is a more important storage area than the lower canyon because the surficial deposits are thicker, and any zones of leakage in the underlying bedrock of the upper canyon probably would result in greater leakage than would similar outlets in the lower canyon.The total annual discharge from Middle Canyon, per unit of precipitation, decreased between 1910 and 1939. Similar decreases occurred in Parleys Canyon in the nearby Wasatch Range and in other drainage basins in Utah, and it is likely that most of the decrease in discharge from Middle Canyon and other canyons in Utah is due to a change in climate.Chemical analyses of water showed that the high content of sulfate and other constituents in the water from the Utah Metals tunnel, which drains into Middle Canyon, does not have a significant effect on water quality at the canyon mouth. This suggests that much of the tunnel water is lost from the channel by leakage, probably in the upper canyon, during the dry part of the year.Comparison of the 150 acre-feet of water per square mile of drainage area discharged by Middle Canyon in 1947 with the 623 and 543 acre-feet per square mile discharged in 1948 by City Creek and Mill Creek Canyons, two comparable drainage basins in the nearby Wasatch Range, also suggests that there is leakage in Middle Canyon.A hydrologic budget of the drainage basin results in an estimate that about 3,000 acre

  15. Computer-aided performance monitoring program at Diablo Canyon

    International Nuclear Information System (INIS)

    Nelson, T.; Glynn, R. III; Kessler, T.C.

    1992-01-01

    This paper describes the thermal performance monitoring program at Pacific Gas ampersand Electric Company's (PG ampersand E's) Diablo Canyon Nuclear Power Plant. The plant performance monitoring program at Diablo Canyon uses the THERMAC performance monitoring and analysis computer software provided by Expert-EASE Systems. THERMAC is used to collect performance data from the plant process computers, condition that data to adjust for measurement errors and missing data points, evaluate cycle and component-level performance, archive the data for trend analysis and generate performance reports. The current status of the program is that, after a fair amount of open-quotes tuningclose quotes of the basic open-quotes thermal kitclose quotes models provided with the initial THERMAC installation, we have successfully baselined both units to cycle isolation test data from previous reload cycles. Over the course of the past few months, we have accumulated enough data to generate meaningful performance trends and, as a result, have been able to use THERMAC to track a condenser fouling problem that was costing enough megawatts to attract corporate-level attention. Trends from THERMAC clearly related the megawatt loss to a steadily degrading condenser cleanliness factor and verified the subsequent gain in megawatts after the condenser was cleaned. In the future, we expect to rebaseline THERMAC to a beginning of cycle (BOC) data set and to use the program to help track feedwater nozzle fouling

  16. Huntington disease: a case study of early onset presenting as depression.

    Science.gov (United States)

    Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael

    2004-10-01

    Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and rigidity, is reported. Meanwhile, the father developed the adult variant of Huntington disease. The boy's diagnosis was confirmed by molecular genetic analysis and magnetic resonance imaging. It is important to be aware of hereditary conditions such as Huntington disease and to provide family counseling before genetic testing and after the diagnosis is confirmed.

  17. The Huntington disease locus is most likely within 325 kilobases of the chromosome 4p telomere

    International Nuclear Information System (INIS)

    Doggett, N.A.; Cheng, J.F.; Smith, C.L.; Cantor, C.R.

    1989-01-01

    The genetic defect responsible for Huntington disease was originally localized near the tip of the short arm of chromosome 4 by genetic linkage to the locus D4S10. Several markers closer to Huntington disease have since been isolated, but these all appear to be proximal to the defect. A physical map that extends from the most distal of these loci, D4S90, to the telomere of chromosome 4 was constructed. This map identifies at least two CpG islands as markers for Huntington disease candidate genes and places the most likely location of the Huntington disease defect remarkably close (within 325 kilobases) to the telomere

  18. Geo-hazard by sediment mass movements in submarine canyons

    Science.gov (United States)

    Ghaith, Afif; Fakhri, Milad; Ivaldi, Roberta; Ciavola, Paolo

    2017-04-01

    Submarine mass movements and their consequences are of major concern for coastal communities and infrastructures but also for the exploitation and the development of seafloor resources. Elevated awareness of the need for better understanding of the underwater mass movement is coupled with great advances in underwater mapping technologies over the past two decades. The seafloor in the Nahr Ibrahim and Saida regions (Lebanon) is characterized by deep canyons, reaching one thousand meters depths in proximity of the coast. Signs of submarine mass movement instability related to these canyons create a connection between shallow and deep water. The presence of these canyons in a tectonically active area generates a particular drained mechanism to the sediment in form of mass movement and slumping. Identification of potential areas where slope movements could be triggered requires data with high spatial resolution. Since this area is poorly explored, in the framework of an international project between Lebanese Navy, Lebanese National Center for Marine Sciences, University of Ferrara and Italian Hydrographic Institute, we analyse the morpho-bathymetric and sedimentological characters of the coastal and shelf sectors. Multibeam echosounder and sub-bottom profiler acoustic systems calibrated with ground truths (sediment grab and core samples) allow us to characterize the nature of seafloor and sub-seafloor with particular detail to the geotechnical properties of sediments and high resolution seismic stratigraphy of the shallow layers. The detection of particular undersea features provides detail maps which are in support to littoral morpho-dynamics, coastal transport and sediment budget. Multilayer hydro-oceanographic map, referring to the seafloor dynamics in connection with deep water environment and drainage system, in accordance to the International Hydrographic Standards and nautical supports, are produced. This high resolution multibeam bathymetry dataset, integrated

  19. Let's Bet on Sediments! Hudson Canyon Cruise--Grades 9-12. Focus: Sediments of Hudson Canyon.

    Science.gov (United States)

    National Oceanic and Atmospheric Administration (DOC), Rockville, MD.

    These activities are designed to teach about the sediments of Hudson Canyon. Students investigate and analyze the patterns of sedimentation in the Hudson Canyon, observe how heavier particles sink faster than finer particles, and learn that submarine landslides are avalanches of sediment in deep ocean canyons. The activity provides learning…

  20. Psychodynamic theory and counseling in predictive testing for Huntington's disease.

    Science.gov (United States)

    Tassicker, Roslyn J

    2005-04-01

    This paper revisits psychodynamic theory, which can be applied in predictive testing counseling for Huntington's Disease (HD). Psychodynamic theory has developed from the work of Freud and places importance on early parent-child experiences. The nature of these relationships, or attachments are reflected in adult expectations and relationships. Two significant concepts, identification and fear of abandonment, have been developed and expounded by the psychodynamic theorist, Melanie Klein. The processes of identification and fear of abandonment can become evident in predictive testing counseling and are colored by the client's experience of growing up with a parent affected by Huntington's Disease. In reflecting on family-of-origin experiences, clients can also express implied expectations of the future, and future relationships. Case examples are given to illustrate the dynamic processes of identification and fear of abandonment which may present in the clinical setting. Counselor recognition of these processes can illuminate and inform counseling practice.

  1. Family caregivers' views on coordination of care in Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C

    2015-01-01

    BACKGROUND: Collaboration between family caregivers and health professionals in specialised hospitals or community-based primary healthcare systems can be challenging. During the course of severe chronic disease, several health professionals might be involved at a given time, and the patient......'s illness may be unpredictable or not well understood by some of those involved in the treatment and care. AIM: The aim of this study was to explore the experiences and expectations of family caregivers for persons with Huntington's disease concerning collaboration with healthcare professionals. METHODS......: To shed light on collaboration from the perspectives of family caregivers, we conducted an explorative, qualitative interview study with 15 adult participants experienced from caring for family members in all stages of Huntington's disease. Data were analysed with systematic text condensation, a cross...

  2. Caregiver roles in families affected by Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C

    2013-01-01

    AIM: The objective of this study was to explore family caregivers' experiences with the impact of Huntington's disease (HD) on the family structure and roles in the family. METHODOLOGY: We interviewed 15 family caregivers in families affected by HD, based on a semi-structured interview guide...... for impairments by taking on adult responsibilities, and in some families, a child had the role as main caregiver. The increasing need for care could cause conflicts between the role as family member and family caregiver. The burden of care within the family could fragment and isolate the family. CONCLUSIONS......: Huntington's disease has a major impact on family systems. Caregiver roles are shaped by impairments in the affected family member and corresponding dynamic adoption and change in roles within the family. Making assessments of the family structure and roles, professionals may understand more about how...

  3. Geohydrology of White Rock Canyon of the Rio Grande from Otowi to Frijoles Canyon

    International Nuclear Information System (INIS)

    Purtymun, W.D.; Peters, R.J.; Owens, J.W.

    1980-12-01

    Twenty-seven springs discharge from the Totavi Lentil and Tesuque Formation in White Rock Canyon. Water generally acquires its chemical characteristics from rock units that comprise the spring aquifer. Twenty-two of the springs are separated into three groups of similar aquifer-related chemical quality. The five remaining springs make up a fourth group with a chemical quality that differs due to localized conditions in the aquifer. Localized conditions may be related to recharge or discharge in or near basalt intrusion or through faults. Streams from Pajarito, Ancho, and Frijoles Canyons discharge into the Rio Grande in White Rock Canyon. The base flow in the streams is from springs. Sanitary effluent in Mortandad Canyon from the treatment plant at White Rock also reaches the Rio Grande

  4. Striatal grafts in a rat model of Huntington's disease

    DEFF Research Database (Denmark)

    Guzman, R; Meyer, M; Lövblad, K O

    1999-01-01

    Survival and integration into the host brain of grafted tissue are crucial factors in neurotransplantation approaches. The present study explored the feasibility of using a clinical MR scanner to study striatal graft development in a rat model of Huntington's disease. Rat fetal lateral ganglionic...... time-points graft location could not be further verified. Measures for graft size and ventricle size obtained from MR images highly correlated with measures obtained from histologically processed sections (R = 0.8, P fetal rat lateral ganglionic...

  5. Challenges of Huntington's disease and quest for therapeutic biomarkers

    Czech Academy of Sciences Publication Activity Database

    Kotrčová, Eva; Jarkovská, Karla; Valeková, Ivona; Žižková, Martina; Motlík, Jan; Gadher, S. J.; Kovářová, Hana

    2015-01-01

    Roč. 9, 1-2 (2015), s. 147-158 ISSN 1862-8346 R&D Projects: GA MŠk ED2.1.00/03.0124; GA TA ČR(CZ) TA01011466 Institutional support: RVO:67985904 Keywords : HD biomarkers * Huntington´s disease * Huntingtin neurotoxicity * Huntingtin pathogenesis Subject RIV: FH - Neurology Impact factor: 2.959, year: 2015

  6. Pollutant Dilution and Diffusion in Urban Street Canyon Neighboring Streets

    Science.gov (United States)

    Sun, Z.; Fu, Zh. M.

    2011-09-01

    In the present study we investigated the airflow patterns and air quality of a series of typical street canyon combinations, developed a mass balance model to determine the local pollutant dilution rate, and discuss the impact of upstream canyon on the air quality of downstream canyon. The results indicated that the geometrical size of upstream and downstream buildings have significant impacts on the ambient airflow patterns. The pollution distribution within the canyons varies with different building combinations and flow patterns. Within the upstream canyon, pollution always accumulates to the low building side for non-symmetrical canyon, and for symmetrical canyon high level of pollution occurs at the leeward side. The height of the middle and downstream buildings can evidently change the pollutant dispersion direction during the transport process. Within the polluted canyon, the pollutant dilution rate (PDR) also varies with different street canyon combinations. The highest PDR is observed when the upstream buildings are both low buildings no matter the height of downstream building. However, the two cases are likely to contribution pollution to the downstream canyon. The H-L-H combination is mostly against local pollution remove, while the L-H-L case is considered the best optimistic building combination with both the ability of diluting local pollution and not remarkably decreasing air quality of downstream canyon. The current work is expected instructive for city designers to optimize traffic patterns under typical existing geometry or in the development of urban geometry modification for air quality control.

  7. Recreational impacts on Colorado River beaches in Glen Canyon, Arizona

    Science.gov (United States)

    Carothers, Steven W.; Johnson, Robert A.; Dolan, Robert

    1984-07-01

    Recreational impact was measured on eight beaches in Glen Canyon National Recreation Area and 15 beaches in Grand Canyon National Park using permanently located transects and plots. Recreational impact indices included densities of human trash and charcoal and a measure of sand discoloration due to charcoal. Significant increases in the indices occurred on several Glen Canyon beaches over a seven-month period. Sand discoloration became significantly higher over all Glen Canyon beaches during the same time period. All indices were significantly higher in Glen Canyon than on similar Grand Canyon beaches. These differences are probably due to differences in: (a) level of impacts tolerated by the respective management regimes and, (b) in the number of user days among the two National Park Service administrative units. Management alternatives are presented for reversing the present trends of recreational impact on Glen Canyon beaches.

  8. Submarine canyons off the Coromandel coast

    Digital Repository Service at National Institute of Oceanography (India)

    Varadachari, V.V.R.; Nair, R.R.; Murty, P.S.N.

    During the 26th Cruise of I.N.S. `KISTNA', a bathymetric survey was carried out in some detail off the Pondicherry coast. This survey has revealed the existence of three sets of distinctly separate canyons between Cuddalore and Palar River...

  9. Assessment of changes at Glen Canyon Dam

    International Nuclear Information System (INIS)

    Cherry, D.; McCoy, J.; Crandall, S.

    1991-01-01

    This paper describes the complexity associated with the assessment of financial impacts of proposed and actual short-term restrictions at Glen Canyon Dam. The reasons for these restrictions are discussed as well as the methods used to measure their financial impact to Western Area Power Administration

  10. ACUMEN 2012: Atlantic Canyons Undersea Mapping Expeditions

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Between February and August 2012, a team of NOAA and external partners will conduct a mapping ‘blitz’ focused on deepwater canyons off the northeastern...

  11. Turbulent ventilation of a street canyon

    DEFF Research Database (Denmark)

    Nielsen, Morten

    2000-01-01

    A selection of turbulence data corresponding to 185 days of field measurements has een analysed. The non-ideal building geometry influenced the circulation patterns in the street canyon and the largest average vertical velocities were observed in the wake of an unbroken line of buildings. The sta...

  12. Anatomy of La Jolla submarine canyon system; offshore southern California

    Science.gov (United States)

    Paull, C.K.; Caress, D.W.; Lundsten, E.; Gwiazda, R.; Anderson, K.; McGann, M.; Conrad, J.; Edwards, B.; Sumner, E.J.

    2013-01-01

    An autonomous underwater vehicle (AUV) carrying a multibeam sonar and a chirp profiler was used to map sections of the seafloor within the La Jolla Canyon, offshore southern California, at sub-meter scales. Close-up observations and sampling were conducted during remotely operated vehicle (ROV) dives. Minisparker seismic-reflection profiles from a surface ship help to define the overall geometry of the La Jolla Canyon especially with respect to the pre-canyon host sediments. The floor of the axial channel is covered with unconsolidated sand similar to the sand on the shelf near the canyon head, lacks outcrops of the pre-canyon host strata, has an almost constant slope of 1.0° and is covered with trains of crescent shaped bedforms. The presence of modern plant material entombed within these sands confirms that the axial channel is presently active. The sand on the canyon floor liquefied during vibracore collection and flowed downslope, illustrating that the sediment filling the channel can easily fail even on this gentle slope. Data from the canyon walls help constrain the age of the canyon and extent of incision. Horizontal beds of moderately cohesive fine-grained sediments exposed on the steep canyon walls are consistently less than 1.232 million years old. The lateral continuity of seismic reflectors in minisparker profiles indicate that pre-canyon host strata extend uninterrupted from outside the canyon underneath some terraces within the canyon. Evidence of abandoned channels and point bar-like deposits are noticeably absent on the inside bend of channel meanders and in the subsurface of the terraces. While vibracores from the surface of terraces contain thin (< 10 cm) turbidites, they are inferred to be part of a veneer of recent sediment covering pre-canyon host sediments that underpin the terraces. The combined use of state of the art seafloor mapping and exploration tools provides a uniquely detailed view of the morphology within an active submarine canyon.

  13. aliving with Huntington´s disease in the Czech Republic

    Czech Academy of Sciences Publication Activity Database

    Baxa, Monika

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 6-6 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] Institutional support: RVO:67985904 Keywords : Czech Huntington Association * life with Huntington ´ disease Subject RIV: FH - Neurology

  14. 77 FR 71636 - Huntington Foam LLC, Fort Smith, AR; Notice of Revised Determination on Reconsideration

    Science.gov (United States)

    2012-12-03

    ... Smith, AR; Notice of Revised Determination on Reconsideration On August 8, 2012, the Department of Labor... workers and former workers of Huntington Foam LLC, Fort Smith, Arkansas (subject firm). The workers are... reconsideration investigation, I determine that workers of Huntington Foam LLC, Fort Smith, Arkansas, who were...

  15. Anatomy of La Jolla submarine canyon system; offshore southern California

    Science.gov (United States)

    Paull, C.K.; Caress, D.W.; Lundsten, E.; Gwiazda, R.; Anderson, K.; McGann, M.; Conrad, J.; Edwards, B.; Sumner, E.J.

    2013-01-01

    An autonomous underwater vehicle (AUV) carrying a multibeam sonar and a chirp profiler was used to map sections of the seafloor within the La Jolla Canyon, offshore southern California, at sub-meter scales. Close-up observations and sampling were conducted during remotely operated vehicle (ROV) dives. Minisparker seismic-reflection profiles from a surface ship help to define the overall geometry of the La Jolla Canyon especially with respect to the pre-canyon host sediments. The floor of the axial channel is covered with unconsolidated sand similar to the sand on the shelf near the canyon head, lacks outcrops of the pre-canyon host strata, has an almost constant slope of 1.0° and is covered with trains of crescent shaped bedforms. The presence of modern plant material entombed within these sands confirms that the axial channel is presently active. The sand on the canyon floor liquefied during vibracore collection and flowed downslope, illustrating that the sediment filling the channel can easily fail even on this gentle slope. Data from the canyon walls help constrain the age of the canyon and extent of incision. Horizontal beds of moderately cohesive fine-grained sediments exposed on the steep canyon walls are consistently less than 1.232 million years old. The lateral continuity of seismic reflectors in minisparker profiles indicate that pre-canyon host strata extend uninterrupted from outside the canyon underneath some terraces within the canyon. Evidence of abandoned channels and point bar-like deposits are noticeably absent on the inside bend of channel meanders and in the subsurface of the terraces. While vibracores from the surface of terraces contain thin (art seafloor mapping and exploration tools provides a uniquely detailed view of the morphology within an active submarine canyon.

  16. Did the "Woman in the Attic" in Jane Eyre Have Huntington Disease?

    Science.gov (United States)

    Coon, Elizabeth A; Hassan, Anhar

    2015-01-01

    References to neurologic disorders are frequently found in fictional literature and may precede description in the medical literature. Our aim was to compare Charlotte Brontë's depiction of Bertha Mason in Jane Eyre to the tenets set forth in George Huntington's original essay "On chorea" with the hypothesis that Mason was displaying features of Huntington disease. Charlotte Brontë's 1847 Victorian novel Jane Eyre features the character Bertha Mason, who is portrayed with a progressive psychiatric illness, violent movements, and possible cognitive decline. Similar to Huntington's tenets, Mason has a disorder with a strong family history suggestive of autosomal dominant inheritance with onset in adulthood, and culminating in suicide. Brontë's character had features of Huntington disease as originally described by Huntington. Brontë's keen characterization may have increased awareness of treatment of neuropsychiatric patients in the Victorian era.

  17. 3D View of Grand Canyon, Arizona

    Science.gov (United States)

    2000-01-01

    The Grand Canyon is one of North America's most spectacular geologic features. Carved primarily by the Colorado River over the past six million years, the canyon sports vertical drops of 5,000 feet and spans a 445-kilometer-long stretch of Arizona desert. The strata along the steep walls of the canyon form a record of geologic time from the Paleozoic Era (250 million years ago) to the Precambrian (1.7 billion years ago).The above view was acquired by the Advanced Spaceborne Thermal Emission and Reflection Radiometer (ASTER) instrument aboard the Terra spacecraft. Visible and near infrared data were combined to form an image that simulates the natural colors of water and vegetation. Rock colors, however, are not accurate. The image data were combined with elevation data to produce this perspective view, with no vertical exaggeration, looking from above the South Rim up Bright Angel Canyon towards the North Rim. The light lines on the plateau at lower right are the roads around the Canyon View Information Plaza. The Bright Angel Trail, which reaches the Colorado in 11.3 kilometers, can be seen dropping into the canyon over Plateau Point at bottom center. The blue and black areas on the North Rim indicate a forest fire that was smoldering as the data were acquired on May 12, 2000.Advanced Spaceborne Thermal Emission and Reflection Radiometer (ASTER) is one of five Earth-observing instruments launched December 18, 1999, on NASA's Terra satellite. The instrument was built by Japan's Ministry of International Trade and Industry. A joint U.S./Japan science team is responsible for validation and calibration of the instrument and the data products. Dr. Anne Kahle at NASA's Jet Propulsion Laboratory, Pasadena, Calif., is the U.S. Science team leader; Moshe Pniel of JPL is the project manager. ASTER is the only high resolution imaging sensor on Terra. The primary goal of the ASTER mission is to obtain high-resolution image data in 14 channels over the entire land surface, as

  18. Topographic change detection at select archeological sites in Grand Canyon National Park, Arizona, 2007–2010

    Science.gov (United States)

    Collins, Brian D.; Corbett, Skye C.; Fairley, Helen C.; Minasian, Diane L.; Kayen, Robert; Dealy, Timothy P.; Bedford, David R.

    2012-01-01

    collected two new datasets in April and September 2010 and processed and improved upon existing methods to generate high-accuracy (3 to 5 cm vertical change threshold) topographic change-detection maps for 10 survey areas encompassing 9 archeological sites along the Colorado River corridor. We also used terrestrial lidar techniques to investigate several other metrics for studying archeological site stability, including monitoring cultural structures and artifacts and remotely measuring cryptobiotic soil crust areas. Our topographic change results indicate that 9 of 10 survey areas showed signs of either erosion, deposition, or both during the 2007–2010 time interval and that these changes can be linked to a variety of geomorphic processes, primarily overland flow gullying and aeolian sand transport. In several cases, large (>50 cm) vertical change occurred, and in one case, more than 100 m3 of sediment was eroded. Further, for all sites monitored throughout the river corridor during this time period, the overall signal was related to erosion rather than deposition. These results highlight the potential for rapid archeological site change in Grand Canyon. Whereas the topographic change results presented herein provide the highest level of change detection yet performed on entire archeological sites in Grand Canyon, additional work in combining these results with site-specific weather, hydrology, and geomorphology data is needed to provide a more thorough understanding of the causes of the documented topographic changes. Linking lidar-derived measurements of topographic changes with these other data sources should provide land managers with a scientific basis for making management decisions regarding archeological resources in Grand Canyon National Park and assist in answering open questions regarding the influence that sediment-depleted flows from Glen Canyon Dam have on archeological site stability.

  19. R6/2 Huntington's disease mice develop early and progressive abnormal brain metabolism and seizures.

    Science.gov (United States)

    Cepeda-Prado, Efrain; Popp, Susanna; Khan, Usman; Stefanov, Dimitre; Rodríguez, Jorge; Menalled, Liliana B; Dow-Edwards, Diana; Small, Scott A; Moreno, Herman

    2012-05-09

    A hallmark feature of Huntington's disease pathology is the atrophy of brain regions including, but not limited to, the striatum. Though MRI studies have identified structural CNS changes in several Huntington's disease (HD) mouse models, the functional consequences of HD pathology during the progression of the disease have yet to be investigated using in vivo functional MRI (fMRI). To address this issue, we first established the structural and functional MRI phenotype of juvenile HD mouse model R6/2 at early and advanced stages of disease. Significantly higher fMRI signals [relative cerebral blood volumes (rCBVs)] and atrophy were observed in both age groups in specific brain regions. Next, fMRI results were correlated with electrophysiological analysis, which showed abnormal increases in neuronal activity in affected brain regions, thus identifying a mechanism accounting for the abnormal fMRI findings. [(14)C] 2-deoxyglucose maps to investigate patterns of glucose utilization were also generated. An interesting mismatch between increases in rCBV and decreases in glucose uptake was observed. Finally, we evaluated the sensitivity of this mouse line to audiogenic seizures early in the disease course. We found that R6/2 mice had an increased susceptibility to develop seizures. Together, these findings identified seizure activity in R6/2 mice and show that neuroimaging measures sensitive to oxygen metabolism can be used as in vivo biomarkers, preceding the onset of an overt behavioral phenotype. Since fMRI-rCBV can also be obtained in patients, we propose that it may serve as a translational tool to evaluate therapeutic responses in humans and HD mouse models.

  20. Characterization of traffic-related PM concentration distribution and fluctuation patterns in near-highway urban residential street canyons.

    Science.gov (United States)

    Hahn, Intaek; Brixey, Laurie A; Wiener, Russell W; Henkle, Stacy W; Baldauf, Richard

    2009-12-01

    Analyses of outdoor traffic-related particulate matter (PM) concentration distribution and fluctuation patterns in urban street canyons within a microscale distance of less than 500 m from a highway source are presented as part of the results from the Brooklyn Traffic Real-Time Ambient Pollutant Penetration and Environmental Dispersion (B-TRAPPED) study. Various patterns of spatial and temporal changes in the street canyon PM concentrations were investigated using time-series data of real-time PM concentrations measured during multiple monitoring periods. Concurrent time-series data of local street canyon wind conditions and wind data from the John F. Kennedy (JFK) International Airport National Weather Service (NWS) were used to characterize the effects of various wind conditions on the behavior of street canyon PM concentrations.Our results suggest that wind direction may strongly influence time-averaged mean PM concentration distribution patterns in near-highway urban street canyons. The rooftop-level wind speeds were found to be strongly correlated with the PM concentration fluctuation intensities in the middle sections of the street blocks. The ambient turbulence generated by shifting local wind directions (angles) showed a good correlation with the PM concentration fluctuation intensities along the entire distance of the first and second street blocks only when the wind angle standard deviations were larger than 30 degrees. Within-canyon turbulent shearing, caused by fluctuating local street canyon wind speeds, showed no correlation with PM concentration fluctuation intensities. The time-averaged mean PM concentration distribution along the longitudinal distances of the street blocks when wind direction was mostly constantly parallel to the street was found to be similar to the distribution pattern for the entire monitoring period when wind direction fluctuated wildly. Finally, we showed that two different PM concentration metrics-time-averaged mean

  1. Sediment transport processes at the head of Halibut Canyon, Eastern Canada margin: An interplay between internal tides and dense shelf water cascading.

    Science.gov (United States)

    Puig, Pere; Greenan, Blair J. W.; Li, Michael Z.; Prescott, Robert H.; Piper, David J. W.

    2013-04-01

    To investigate the processes by which sediment is transported through a submarine canyon incised in a glaciated margin, the bottom boundary layer quadrapod RALPH was deployed at 276-m depth in the West Halibut Canyon (off Newfoundland) during winter 2008-2009. Two main sediment transport processes were identified throughout the deployment. Firstly, periodic increases of near-bottom suspended-sediment concentrations (SSC) were recorded associated with the up-canyon propagation of the semidiurnal internal tidal bore along the canyon axis, carrying fine sediment particles resuspended from deeper canyon regions. The recorded SSC peaks, lasting less than one hour, were observed sporadically and were linked to bottom intensified up-canyon flows concomitant with sharp drops in temperature. Secondly, sediment transport was also observed during events of intensified down-canyon current velocities that occurred during periods of sustained heat loss from surface waters, but were not associated with large storms. High-resolution velocity profiles throughout the water column during these events revealed that the highest current speeds (~1 m s-1) were centered several meters above the sea floor and corresponded to the region of maximum velocities of a gravity flow. Such flows had associated low SSC and cold water temperatures and have been interpreted as dense shelf water cascading events channelized along the canyon axis. Sediment transport during these events was largely restricted to bedload and saltation, producing winnowing of sands and fine sediments around larger gravel particles. Analysis of historical hydrographic data suggests that the origin of such gravity flows is not related to the formation of coastal dense waters advected towards the canyon head. Rather, the dense shelf waters appear to be generated around the outer shelf, where convection during winter is able to reach the sea floor and generate a pool of near-bottom dense water that cascades into the canyon

  2. LES of flow in the street canyon

    Science.gov (United States)

    Fuka, Vladimír; Brechler, Josef

    2012-04-01

    Results of computer simulation of flow over a series of street canyons are presented in this paper. The setup is adapted from an experimental study by [4] with two different shapes of buildings. The problem is simulated by an LES model CLMM (Charles University Large Eddy Microscale Model) and results are analysed using proper orthogonal decomposition and spectral analysis. The results in the channel (layout from the experiment) are compared with results with a free top boundary.

  3. New supply for canyon fire foam system

    International Nuclear Information System (INIS)

    Gainey, T.

    1995-01-01

    The raw water supply for the B-Plant Canyon fire foam system is being replaced. The 4 inche water supply line to the foam system is being rerouted from the 6 inches raw water line in the Pipe Gallery to the 10 inches raw water main in the Operating Gallery. This document states the acceptance criteria for the flushing and testing to be performed by the contractor

  4. Radiosensitivity in Huntington's disease: implications for pathogenesis and presymptomatic diagnosis

    International Nuclear Information System (INIS)

    Moshell, A.N.; Tarone, R.E.; Barrett, S.F.; Robbins, J.H.

    1980-01-01

    Huntington's disease (HD) is a dominantly inherited fatal disorder characterised by premature death of nerve cells. Cultured lymphocyte lines from four patients with HD were abnormally sensitive to the lethal effects of X rays, as were lines from two of five subjects at risk for HD. The hypersensitivity is specific for ionising radiation, since HD lines had normal survival after exposure to ultraviolet radiation. The hypersensitivity, which may reflect an inherited defect in DNA repair, provides the basis for a presymptomatic diagnostic test for the disease. (author)

  5. Ventilation Processes in a Three-Dimensional Street Canyon

    Science.gov (United States)

    Nosek, Štěpán; Kukačka, Libor; Kellnerová, Radka; Jurčáková, Klára; Jaňour, Zbyněk

    2016-05-01

    The ventilation processes in three different street canyons of variable roof geometry were investigated in a wind tunnel using a ground-level line source. All three street canyons were part of an urban-type array formed by courtyard-type buildings with pitched roofs. A constant roof height was used in the first case, while a variable roof height along the leeward or windward walls was simulated in the two other cases. All street-canyon models were exposed to a neutrally stratified flow with two approaching wind directions, perpendicular and oblique. The complexity of the flow and dispersion within the canyons of variable roof height was demonstrated for both wind directions. The relative pollutant removals and spatially-averaged concentrations within the canyons revealed that the model with constant roof height has higher re-emissions than models with variable roof heights. The nomenclature for the ventilation processes according to quadrant analysis of the pollutant flux was introduced. The venting of polluted air (positive fluctuations of both concentration and velocity) from the canyon increased when the wind direction changed from perpendicular to oblique, irrespective of the studied canyon model. Strong correlations (>0.5) between coherent structures and ventilation processes were found at roof level, irrespective of the canyon model and wind direction. This supports the idea that sweep and ejection events of momentum bring clean air in and detrain the polluted air from the street canyon, respectively.

  6. Strenner Canyon project owes much to Lester Pelton. [Use of Pelton turbine in hydroelectric project

    Energy Technology Data Exchange (ETDEWEB)

    Townsend, C.

    1985-09-01

    The development of the Stenner Canyon Hydroelectric Project involving 14 miles of pipeline in the hills of California and using the Pelton turbine originally designed in 1883 is described. The city of San Luis Obispo generates dollar 300,000 in revenues by selling the power produced. Four million kWh per annum is fed into the local grid which is sufficient to service about 750 householders for the entire year. (U.K.).

  7. Capability to Recover Plutonium-238 in H-Canyon/HB-Line - 13248

    Energy Technology Data Exchange (ETDEWEB)

    Fuller, Kenneth S. Jr.; Smith, Robert H. Jr.; Goergen, Charles R. [Savannah River Nuclear Solutions, LLC, Savannah River Site, Aiken, SC 29802 (United States)

    2013-07-01

    Plutonium-238 is used in Radioisotope Thermoelectric Generators (RTGs) to generate electrical power and in Radioisotope Heater Units (RHUs) to produce heat for electronics and environmental control for deep space missions. The domestic supply of Pu-238 consists of scrap material from previous mission production or material purchased from Russia. Currently, the United States has no significant production scale operational capability to produce and separate new Pu-238 from irradiated neptunium-237 targets. The Department of Energy - Nuclear Energy is currently evaluating and developing plans to reconstitute the United States capability to produce Pu-238 from irradiated Np-237 targets. The Savannah River Site had previously produced and/or processed all the Pu-238 utilized in Radioisotope Thermoelectric Generators (RTGs) for deep space missions up to and including the majority of the plutonium for the Cassini Mission. The previous full production cycle capabilities included: Np- 237 target fabrication, target irradiation, target dissolution and Np-237 and Pu-238 separation and purification, conversion of Np-237 and Pu-238 to oxide, scrap recovery, and Pu-238 encapsulation. The capability and equipment still exist and could be revitalized or put back into service to recover and purify Pu-238/Np-237 or broken General Purpose Heat Source (GPHS) pellets utilizing existing process equipment in HB-Line Scrap Recovery, and H-Canyon Frame Waste Recovery processes. The conversion of Np-237 and Pu-238 to oxide can be performed in the existing HB-Line Phase-2 and Phase- 3 Processes. Dissolution of irradiated Np-237 target material, and separation and purification of Np-237 and Pu-238 product streams would be possible at production rates of ∼2 kg/month of Pu-238 if the existing H-Canyon Frames Process spare equipment were re-installed. Previously, the primary H-Canyon Frames equipment was removed to be replaced: however, the replacement project was stopped. The spare equipment

  8. Motor outcome measures in Huntington disease clinical trials.

    Science.gov (United States)

    Reilmann, Ralf; Schubert, Robin

    2017-01-01

    Deficits in motor function are a hallmark of Huntington disease (HD). The Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS) is a categoric clinical rating scale assessing multiple domains of motor disability in HD. The UHDRS-TMS or subsets of its items have served as primary or secondary endpoints in numerous clinical trials. In spite of a well-established video-based annual online certification system, intra- and interrater variability, subjective error, and rater-induced placebo effects remain a concern. In addition, the UHDRS-TMS was designed to primarily assess motor symptoms in manifest HD. Recently, advancement of technology resulted in the introduction of the objective Q-Motor (i.e., Quantitative-Motor) assessments in biomarker studies and clinical trials in HD. Q-Motor measures detected motor signs in blinded cross-sectional and longitudinal analyses of manifest, prodromal, and premanifest HD cohorts up to two decades before clinical diagnosis. In a multicenter clinical trial in HD, Q-Motor measures were more sensitive than the UHDRS-TMS and exhibited no placebo effects. Thus, Q-Motor measures are currently explored in several multicenter trials targeting both symptomatic and disease-modifying mechanisms. They may supplement the UHDRS-TMS, increase the sensitivity and reliability in proof-of-concept studies, and open the door for phenotype assessments in clinical trials in prodromal and premanifest HD. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. Huntington's Disease in a Patient Misdiagnosed as Conversion Disorder.

    Science.gov (United States)

    Nogueira, João Machado; Franco, Ana Margarida; Mendes, Susana; Valadas, Anabela; Semedo, Cristina; Jesus, Gustavo

    2018-01-01

    Huntington's disease (HD) is an inherited, progressive, and neurodegenerative neuropsychiatric disorder caused by the expansion of cytosine-adenine-guanine (CAG) trinucleotide in Interested Transcript (IT) 15 gene on chromosome 4. This pathology typically presents in individuals aged between 30 and 50 years and the age of onset is inversely correlated with the length of the CAG repeat expansion. It is characterized by chorea, cognitive deficits, and psychiatric symptoms. Usually the psychiatric disorders precede motor and cognitive impairment, Major Depressive Disorder and anxiety disorders being the most common presentations. We present a clinical case of a 65-year-old woman admitted to our Psychiatric Acute Unit. During the 6 years preceding the admission, the patient had clinical assessments made several times by different specialties that focused only on isolated symptoms, disregarding the syndrome as a whole. In the course of her last admission, the patient was referred to our Neuropsychiatric Team, which made the provisional diagnosis of late-onset Huntington's disease, later confirmed by genetic testing. This clinical vignette highlights the importance of a multidisciplinary approach to atypical clinical presentations and raises awareness for the relevance of investigating carefully motor symptoms in psychiatric patients.

  10. Apolipoprotein E and presenilin-1 genotypes in Huntington's disease.

    Science.gov (United States)

    Panas, M; Avramopoulos, D; Karadima, G; Petersen, M B; Vassilopoulos, D

    1999-07-01

    Huntington's disease (HD) is an autosomal dominant degenerative disease of the central nervous system manifested by involuntary movements (chorea), psychiatric manifestations, and cognitive impairment with a variable age at onset. This variability is mainly attributed to genetic factors. The so-called aging genes [e.g., those for apolipoprotein E (APOE) and presenilin-1 (PS-1) have been implicated in determining the age at onset of Alzheimer's disease, a disease sharing common clinical features with HD. In 60 unrelated patients suffering from HD (mean age at onset 40.1 years, range 20-65) we determined number of CAG repeats and the distribution of the APOE alleles (epsilon2, epsilon3, epsilon4) and PS-1 alleles. The results showed that: (a) The age at onset was higher in the group of patients with the epsilon4 allele (51.6 vs. 38.0 P<0.002), (b) The correlation between the age at onset and the number of CAG repeats was strong in patients with the epsilon3/epsilon3 genotype while it was not detected in patients with epsilon3/epsilon4 genotype. (c) No correlation was found between age at onset and PS-1 alleles. In conclusion, APOE seems to be a significant factor influencing the age at onset of Huntington's disease.

  11. Xeroderma pigmentosum is a definite cause of Huntington's disease-like syndrome.

    Science.gov (United States)

    Garcia-Moreno, Hector; Fassihi, Hiva; Sarkany, Robert P E; Phukan, Julie; Warner, Thomas; Lehmann, Alan R; Giunti, Paola

    2018-01-01

    Xeroderma pigmentosum is characterized by cutaneous, ophthalmological, and neurological features. Although it is typical of childhood, late presentations can mimic different neurodegenerative conditions. We report two families presenting as Huntington's disease-like syndromes. The first case (group G) presented with neuropsychiatric features, cognitive decline and chorea. Typical lentigines were only noticed after the neurological disease started. The second case (group B) presented adult-onset chorea and neuropsychiatric symptoms after an aggressive ocular melanoma. Xeroderma pigmentosum can manifest as a Huntington's Disease-like syndrome. Classic dermatological and oncological features have to be investigated in choreic patients with negative genetic tests for Huntington's disease-like phenotypes.

  12. 78 FR 7810 - Glen Canyon Dam Adaptive Management Work Group

    Science.gov (United States)

    2013-02-04

    ... DEPARTMENT OF THE INTERIOR Bureau of Reclamation Glen Canyon Dam Adaptive Management Work Group... Adaptive Management Work Group (AMWG) makes recommendations to the Secretary of the Interior concerning..., the AMWG, a technical work group (TWG), a Grand Canyon Monitoring and Research Center, and independent...

  13. 78 FR 21415 - Glen Canyon Dam Adaptive Management Work Group

    Science.gov (United States)

    2013-04-10

    ... DEPARTMENT OF THE INTERIOR Bureau of Reclamation Glen Canyon Dam Adaptive Management Work Group... Adaptive Management Work Group (AMWG) makes recommendations to the Secretary of the Interior concerning... Federal advisory committee, the AMWG, a technical work group, a Grand Canyon Monitoring and Research...

  14. 75 FR 34476 - Glen Canyon Dam Adaptive Management Work Group

    Science.gov (United States)

    2010-06-17

    ... DEPARTMENT OF THE INTERIOR Bureau of Reclamation Glen Canyon Dam Adaptive Management Work Group... Management Work Group. The purpose of the Adaptive Management Work Group is to advise and to provide... of the Glen Canyon Dam Adaptive Management Work Group is in the public interest in connection with...

  15. 76 FR 24516 - Glen Canyon Dam Adaptive Management Work Group

    Science.gov (United States)

    2011-05-02

    ... DEPARTMENT OF THE INTERIOR Bureau of Reclamation Glen Canyon Dam Adaptive Management Work Group... Adaptive Management Work Group (AMWG) makes recommendations to the Secretary of the Interior concerning..., the AMWG, a technical work group (TWG), a Grand Canyon Monitoring and Research Center, and independent...

  16. Modelling the air flow in symmetric and asymmetric street canyons

    Energy Technology Data Exchange (ETDEWEB)

    Santiago, J.L.; Martin, F. [Research Center for Energy, Environment and Technology (CIEMAT), Madrid (Spain). Fossil Fuels Dept., Numerical Simulation and Modelling Program

    2004-07-01

    In recent years a large amount of research has been conducted on urban scale and street canyon. Control of air quality inside cities is important for human health. To achieve this objective, street canyon modelling plays a significant role. Pollutant dispersion inside canyons are determined by wind flow around this complex geometry. Experimental investigations have been made by means of field measurements such as Vachon, G. et al. or wind tunnel experiences as Meroney, R.N. et al. or Kastner-Klein, P. and E.J. Plate. In many of these researches, they have used CFD models in several configurations, for instance Assimakopoulos, V.D. et al. or Sini, J.-F. et al. These models are based on a numerical resolution of Navier-Stokes equations with a turbulence closure. In this study, the aim is contribute to the understanding of air circulation inside street canyons. In order to achieve this purpose, several configurations of canyons are investigated. Two-dimensional sequences of real-scale street canyons (order to obstacles height is meters) with different features (symmetric canyons and asymmetric canyons forming step-up and step-down notch configurations) are simulated. These general configurations are modified to investigate some parameters such as aspect ratio, W/H, where W is the width of street and H is the height of buildings. Flows with high Reynolds numbers are modelling. FLUENT CFD software is used. (orig.)

  17. Implications of tree planting on pollutant dispersion in street canyons

    NARCIS (Netherlands)

    Gromke, C.B.; Ruck, B.

    2009-01-01

    Traffic pollutant dispersion processes inside urban street canyons with avenue-like tree planting have been studied in wind tunnel experiments. Tree planting of different crown porosities and their effects on the pollutant concentrations at the canyon walls have been investigated for wind

  18. Geology and geomorphology of the Lower Deschutes River Canyon, Oregon.

    Science.gov (United States)

    Robin A. Beebee; Jim E. O' Connor; Gordon E. Grant

    2002-01-01

    This field guide is designed for geologists floating the approximately 80 kilometers (50 miles) of the Deschutes River from the Pelton-Round Butte Dam Complex west of Madras to Maupin, Oregon. The first section of the guide is a geologic timeline tracing the formation of the units that compose the canyon walls and the incision of the present canyon. The second section...

  19. Modeling the Effect of Wider Canyons on Urban Heating

    Directory of Open Access Journals (Sweden)

    Rizwan Ahmed Memon

    2011-04-01

    Full Text Available The k-? turbulence model is adopted in this study to simulate the impact of street canyon AR (Aspect Ratios on heating within street canyon. The two-dimensional model was validated for RANS (Reynolds Averaged Navier Stokes and energy transport equations. The validation process confirms that the results of the model for airtemperature and wind speed could be trusted. The application of the said model is carried out to ideal street canyons of ARs (ratio of building-height-to-street-width from 0.4 to 2 with the same boundary conditions. Notably, street canyon aspect ratio was calculated by varying the street width while keeping the building height constant. Results show that the weighted-average-air-temperature within AR 0.4 was around 0.8% (i.e. 2.4K higher than that within AR 2.0. Conversely, there was strong correlation (i.e., R2>0.9 between air temperature within the street canyon and street canyon AR. Results demonstrate stronger influence of vertical velocity on heating within street canyon. Evidently, increased vertical velocity decreased the temperatures. Conversely, temperatures were higher along the leeward side of the canyon in lower ARs.

  20. Perspective view over the Grand Canyon, Arizona

    Science.gov (United States)

    2001-01-01

    This simulated true color perspective view over the Grand Canyon was created from Advanced Spaceborne Thermal Emission and Reflection Radiometer (ASTER) data acquired on May 12, 2000. The Grand Canyon Village is in the lower foreground; the Bright Angel Trail crosses the Tonto Platform, before dropping down to the Colorado Village and then to the Phantom Ranch (green area across the river). Bright Angel Canyon and the North Rim dominate the view. At the top center of the image the dark blue area with light blue haze is an active forest fire. ASTER is one of five Earth-observing instruments launched December 18, 1999, on NASA's Terra satellite. The instrument was built by Japan's Ministry of Economy, Trade and Industry. A joint U.S./Japan science team is responsible for validation and calibration of the instrument and the data products. The broad spectral coverage and high spectral resolution of ASTER provides scientists in numerous disciplines with critical information for surface mapping, and monitoring of dynamic conditions and temporal change. Example applications are: monitoring glacial advances and retreats; monitoring potentially active volcanoes; identifying crop stress; determining cloud morphology and physical properties; wetlands evaluation; thermal pollution monitoring; coral reef degradation; surface temperature mapping of soils and geology; and measuring surface heat balance. The U.S. science team is located at NASA's Jet Propulsion Laboratory, Pasadena, Calif. The Terra mission is part of NASA's Science Mission Directorate. Size: 5 km in foreground to 40 km Location: 36.3 degrees north latitude, 112 degrees west longitude Orientation: North-northeast at top Original Data Resolution: ASTER 15 meters Dates Acquired: May 12, 2000

  1. The Black Canyon of the Gunnison: Today and Yesterday

    Science.gov (United States)

    Hansen, Wallace R.

    1965-01-01

    Since the early visit of Captain John William Gunnison in the middle of the last century, the Black Canyon of the Gunnison has stirred mixed apprehension and wonder in the hearts of its viewers. It ranks high among the more awesome gorges of North America. Many great western canyons are as well remembered for their brightly colored walls as for their airy depths. Not so the Black Canyon. Though it is assuredly not black, the dark-gray tones of its walls and the hazy shadows of its gloomy depths join together to make its name well deserved. Its name conveys an impression, not a picture. After the first emotional impact of the canyon, the same questions come to the minds of most reflective viewers and in about the following order: How deep is the Black Canyon, how wide, how does it compare with other canyons, what are the rocks, how did it form, and how long did it take? Several western canyons exceed the Black Canyon in overall size. Some are longer; some are deeper; some are narrower; and a few have walls as steep. But no other canyon in North American combines the depth, narrowness, sheerness, and somber countenance of the Black Canyon. In many places the Black Canyon is as deep as it is wide. Between The Narrows and Chasm View in the Black Canyon of the Gunnison National Monument (fig. 15) it is much deeper than wide. Average depth in the monument is about 2,000 feet, ranging from a maximum of about 2,700 feet, north of Warner Point (which also is the greatest depth anywhere in the canyon), to a minimum of about 1,750 feet at The Narrows. The stretch of canyon between Pulpit Rock and Chasm View, including The Narrows, though the shallowest in the monument, is also the narrowest, has some of the steepest walls, and is, therefore, among the most impressive segments of the canyon (fig. 3). Profiles of several well-known western canyons are shown in figure 1. Deepest of these by far is Hells Canyon of the Snake, on the Idaho-Oregon border. Clearly, it dwarfs the

  2. Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles, Vermont

    Data.gov (United States)

    Vermont Center for Geographic Information — Digital Data from VG95-9A Thompson, PJ�and Thompson, TB, 1995, Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles,...

  3. The role of tau in the pathological process and clinical expression of Huntington's disease

    DEFF Research Database (Denmark)

    Vuono, Romina; Winder-Rhodes, Sophie; de Silva, Rohan

    2015-01-01

    and progression of Huntington's disease, the exact molecular mechanisms driving its pathogenic cascade and clinical features, especially the dementia, are not fully understood. Recently the microtubule associated protein tau, MAPT, which is associated with several neurodegenerative disorders, has been implicated......-mortem brain samples from patients with Huntington's disease (n = 16) compared to cases with a known tauopathy and healthy controls. Next, we undertook a genotype-phenotype analysis of a large cohort of patients with Huntington's disease (n = 960) with a particular focus on cognitive decline. We report...... not only on the tau pathology in the Huntington's disease brain but also the association between genetic variation in tau gene and the clinical expression and progression of the disease. We found extensive pathological inclusions containing abnormally phosphorylated tau protein that co-localized in some...

  4. Huntington's disease does not appear to increase the risk of diabetes mellitus

    DEFF Research Database (Denmark)

    Boesgaard, T W; Nielsen, Troels Tolstrup; Josefsen, Knud Elnegaard

    2009-01-01

    Huntington's disease (HD) is an autosomal, dominantly inherited, neurodegenerative disorder characterised by neurological, cognitive and psychiatric symptoms. HD has been associated with diabetes mellitus, which is, to some extent, supported by studies in transgenic HD mice. In transgenic mice...

  5. Transgenic miniature pig as a model for the study of Huntington´s Disease

    Czech Academy of Sciences Publication Activity Database

    Baxa, Monika

    2012-01-01

    Roč. 22, č. 2 (2012), s. 23-25 ISSN 1210-1737 Institutional support: RVO:67985904 Keywords : transgenic pig * Huntington ´s disease * large animal model * neurodegenerative disease Subject RIV: EB - Genetics ; Molecular Biology

  6. Huntington Revisited: Is Conservative Realism Still Essential for the Military Ethic

    National Research Council Canada - National Science Library

    Mahoney-Norris, Kathleen A

    2001-01-01

    ...). Furthermore, Huntington has developed what appears to be a powerful argument as to why conservative realism should be considered a fundamental component of the professional ethic of the military officer...

  7. Remedial action at the Acid/Pueblo Canyon site, Los Alamos, New Mexico. Final report

    International Nuclear Information System (INIS)

    1984-10-01

    The Acid/Pueblo Canyon site (TA-45) was designated in 1976 for remedial action under the Formerly Utilized Sites Remedial Action Program (FUSRAP). During the period 1943 to 1964 untreated and treated liquid wastes generated by nuclear weapons research activities at the Los Alamos Scientific Laboratory (LASL) were discharged into the two canyons. A survey of the site conducted by LASL in 1976 to 1977 identified two areas where radiological contamination exceeded criteria levels. The selected remedial action was based on extensive radiological characterization and comprehensive engineering assessments and comprised the excavation and disposal of 390 yd 3 of contaminated soil and rock. This document describes the background to the remedial action, the parties involved in administering and executing it, the chronology of the work, verification of the adequacy of the remedial action, and the cost incurred. 14 references, 5 figures, 5 tables

  8. Dispersion and photochemical evolution of reactive pollutants in street canyons

    Science.gov (United States)

    Kwak, Kyung-Hwan; Baik, Jong-Jin; Lee, Kwang-Yeon

    2013-05-01

    Dispersion and photochemical evolution of reactive pollutants in street canyons with canyon aspect ratios of 1 and 2 are investigated using a computational fluid dynamics (CFD) model coupled with the carbon bond mechanism IV (CBM-IV). Photochemical ages of NOx and VOC are expressed as a function of the NO2-to-NOx and toluene-to-xylene ratios, respectively. These are found to be useful for analyzing the O3 and OH oxidation processes in the street canyons. The OH oxidation process (O3 oxidation process) is more pronounced in the upper (lower) region of the street canyon with a canyon aspect ratio of 2, which is characterized by more (less) aged air. In the upper region of the street canyon, O3 is chemically produced as well as transported downward across the roof level, whereas O3 is chemically reduced in the lower region of the street canyon. The O3 chemical production is generally favorable when the normalized photochemical ages of NOx and VOC are larger than 0.55 and 0.28, respectively. The sensitivities of O3 chemical characteristics to NOx and VOC emission rates, photolysis rate, and ambient wind speed are examined for the lower and upper regions of the street canyon with a canyon aspect ratio of 2. The O3 concentration and the O3 chemical production rate divided by the O3 concentration increase as the NOx emission rate decreases and the VOC emission rate and photolysis rate increase. The O3 concentration is less sensitive to the ambient wind speed than to other factors considered. The relative importance of the OH oxidation process compared to the O3 oxidation process increases with increasing NOx emission rate and photolysis rate and decreasing VOC emission rate. In this study, both O3 and OH oxidation processes are found to be important in street-canyon scale chemistry. The methodology of estimating the photochemical ages can potentially be adopted to neighborhood scale chemistry.

  9. Warm Season Storms, Floods, and Tributary Sand Inputs below Glen Canyon Dam: Investigating Salience to Adaptive Management in the Context of a 10-Year Long Controlled Flooding Experiment in Grand Canyon National Park, AZ, USA

    Science.gov (United States)

    Jain, S.; Melis, T. S.; Topping, D. J.; Pulwarty, R. S.; Eischeid, J.

    2013-12-01

    The planning and decision processes in the Glen Canyon Dam Adaptive Management Program (GCDAMP) strive to balance numerous, often competing, objectives, such as, water supply, hydropower generation, low flow maintenance, maximizing conservation of downstream tributary sand supply, endangered native fish, and other sociocultural resources of Glen Canyon National Recreation Area and Grand Canyon National Park. In this context, use of monitored and predictive information on the warm season floods (at point-to-regional scales) has been identified as lead-information for a new 10-year long controlled flooding experiment (termed the High-Flow Experiment Protocol) intended to determine management options for rebuilding and maintaining sandbars in Grand Canyon; an adaptive strategy that can potentially facilitate improved planning and dam operations. In this work, we focus on a key concern identified by the GCDAMP, related to the timing and volume of tributary sand input from the Paria and Little Colorado Rivers (located 26 and 124 km below the dam, respectively) into the Colorado River in Grand Canyon National Park. Episodic and intraseasonal variations (with links to equatorial and sub-tropical Pacific sea surface temperature variability) in the southwest hydroclimatology are investigated to understand the magnitude, timing and spatial scales of warm season floods from this relatively small, but prolific sand producing drainage of the semi-arid Colorado Plateau. The coupled variations of the flood-driven sediment input (magnitude and timing) from these two drainages into the Colorado River are also investigated. The physical processes, including diagnosis of storms and moisture sources, are mapped alongside the planning and decision processes for the ongoing experimental flood releases from the Glen Canyon Dam which are aimed at achieving restoration and maintenance of sandbars and instream ecology. The GCDAMP represents one of the most visible and widely recognized

  10. Transgenic animal models for study of the pathogenesis of Huntington's disease and therapy.

    Science.gov (United States)

    Chang, Renbao; Liu, Xudong; Li, Shihua; Li, Xiao-Jiang

    2015-01-01

    Huntington's disease (HD) is caused by a genetic mutation that results in polyglutamine expansion in the N-terminal regions of huntingtin. As a result, this polyQ expansion leads to the misfolding and aggregation of mutant huntingtin as well as age-dependent neurodegeneration. The genetic mutation in HD allows for generating a variety of animal models that express different forms of mutant huntingtin and show differential pathology. Studies of these animal models have provided an important insight into the pathogenesis of HD. Mouse models of HD include transgenic mice, which express N-terminal or full-length mutant huntingtin ubiquitously or selectively in different cell types, and knock-in mice that express full-length mutant Htt at the endogenous level. Large animals, such as pig, sheep, and monkeys, have also been used to generate animal HD models. This review focuses on the different features of commonly used transgenic HD mouse models as well as transgenic large animal models of HD, and also discusses how to use them to identify potential therapeutics. Since HD shares many pathological features with other neurodegenerative diseases, identification of therapies for HD would also help to develop effective treatment for different neurodegenerative diseases that are also caused by protein misfolding and occur in an age-dependent manner.

  11. Reduction in mitochondrial DNA copy number in peripheral leukocytes after onset of Huntington's disease

    DEFF Research Database (Denmark)

    Petersen, Maria Hvidberg; Budtz-Jørgensen, Esben; Sørensen, Sven Asger

    2014-01-01

    Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by movement disorder, cognitive symptoms and psychiatric symptoms with predominantly adult-onset. The mutant huntingtin protein leads to mitochondrial dysfunction in blood leukocytes. This discovery led to the inve......Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by movement disorder, cognitive symptoms and psychiatric symptoms with predominantly adult-onset. The mutant huntingtin protein leads to mitochondrial dysfunction in blood leukocytes. This discovery led...

  12. [The life as a caregiver of a person affected by Chorea Huntington: multiple case study].

    Science.gov (United States)

    Winkler, Evi; Ausserhofer, Dietmar; Mantovan, Franco

    2012-10-01

    Chorea Huntington is an autosomal dominantly inherited, neurodegenerative brain disorder that leads to involuntary hyperkinesia, psychotic symptoms and dementia. The illness not only changes the life of the person itself but also the world of the caregivers. The challenges in the care of a person which is affected by Chorea Huntington have an effect on the daily living as an assemblage of natural and social conditions. a multiple case study was conducted. It included semi-structured interviews with three caregivers of people with Chorea Huntington in South Tyrol. The qualitative data was analyzed using the qualitative structured analysis of Mayring (2007). The objective of this study was to describe the phenomenon of change of life from family members that care people affected by Chorea Huntington in a specific cultural setting (South Tyrol, Italy). The caregivers reported that the diagnosis of Chorea Huntington leads to negative changes in "relationship and family". Particularly, frustration, aggression, impatience and apathy were perceived as stressful. At the same time they highlight the positive changes through home care. They report that the relationship became more intimate and integral and it was characterized by more cohesion. Family caregivers get valuable support from the home care service, however, they complain that there is no facility in South Tyrol, which is specialized to care people with Chorea Huntington. Therefore, the caregivers have to "give up a lot" and don't have any personal desires, dreams and expectations for the future. The caregivers have learned independently to deal with their changed life step by step, and to see also the positive effects of the caring role. The life of family caregivers of a person which is affected by Chorea Huntington is characterized by abandonment. A continuous and professional care would be important for the affected and his caregiver. A continuous and professional care is important for both, addressing the

  13. Isotopic evidence for the influence of typhoons and submarine canyons on the sourcing and transport behavior of biospheric organic carbon to the deep sea

    Science.gov (United States)

    Zheng, Li-Wei; Ding, Xiaodong; Liu, James T.; Li, Dawei; Lee, Tsung-Yu; Zheng, Xufeng; Zheng, Zhenzhen; Xu, Min Nina; Dai, Minhan; Kao, Shuh-Ji

    2017-05-01

    Export of biospheric organic carbon from land masses to the ocean plays an important role in regulating the global carbon cycle. High-relief islands in the western Pacific are hotspots for such land-to-ocean carbon transport due to frequent floods and active tectonics. Submarine canyon systems serve as a major conduit to convey terrestrial organics into the deep sea, particularly during episodic floods, though the nature of ephemeral sediment transportation through such canyons remains unclear. In this study, we deployed a sediment trap in southwestern Taiwan's Gaoping submarine canyon during summer 2008, during which Typhoon Kalmaegi impacted the study area. We investigated sources of particulate organic carbon and quantified the content of fossil organic carbon (OCf) and biospheric non-fossil carbon (OCnf) during typhoon and non-typhoon periods, based on relations between total organic carbon (TOC), isotopic composition (δ13 C, 14C), and nitrogen to carbon ratios (N/C) of newly and previously reported source materials. During typhoons, flooding connected terrestrial rivers to the submarine canyon. Fresh plant debris was not found in the trap except in the hyperpycnal layer, suggesting that only hyperpycnal flow is capable of entraining plant debris, while segregation had occurred during non-hyperpycnal periods. The OCnf components in typhoon flood and trapped samples were likely sourced from aged organics buried in ancient landslides. During non-typhoon periods, the canyon is more connected to the shelf, where waves and tides cause reworking, thus allowing abiotic and biotic processes to generate isotopically uniform and similarly aged OCnf for transport into the canyon. Therefore, extreme events coupled with the submarine canyon system created an efficient method for deep-sea burial of freshly produced organic-rich material. Our results shed light on the ephemeral transport of organics within a submarine canyon system on an active tectonic margin.

  14. Sodium phenylbutyrate in Huntington's disease: a dose-finding study.

    Science.gov (United States)

    Hogarth, Penelope; Lovrecic, Luca; Krainc, Dimitri

    2007-10-15

    Transcriptional dysregulation in Huntington's disease (HD) is mediated in part by aberrant patterns of histone acetylation. We performed a dose-finding study in human HD of sodium phenylbutyrate (SPB), a histone deacetylase inhibitor that ameliorates the HD phenotype in animal models. We used a dose-escalation/de-escalation design, using prespecified toxicity criteria and standard clinical and laboratory safety measures. The maximum tolerated dose was 15 g/day. At higher doses, toxicity included vomiting, lightheadedness, confusion, and gait instability. We saw no significant laboratory or electrocardiographic abnormalities. Gene expression changes in blood suggested an inverse dose-response. In conclusion, SPB at 12 to 15 g/day appears to be safe and well-tolerated in human HD. 2007 Movement Disorder Society

  15. Making a measurable difference in advanced Huntington disease care.

    Science.gov (United States)

    Moskowitz, Carol Brown; Rao, Ashwini K

    2017-01-01

    Neurologists' role in the care of people with advanced Huntington disease (HD) (total functional capacity speech and language pathology), behavioral and psychiatric professionals for problem-solving strategies, which must be reviewed with direct care staff before implementation; (3) encourage and support qualitative and quantitative interdisciplinary research studies, and randomized controlled studies of nonpharmacologic interventions; and (4) assist in the development of meaningful measures to further document what works to provide a good quality of life for the patient and family and a comfortable thoughtful approach to a good death. Collaborative models of care depend on: (1) clear communication; (2) ongoing education and support programs; with (3) pharmacologic and rehabilitation interventions, always in the context of respect for the person with HD, a preservation of the individuals' dignity, autonomy, and individual preferences. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Swallowing endoscopy findings in Huntington's disease: a case report.

    Science.gov (United States)

    Alves, Thaís Coelho; Cola, Paula Cristina; Santos, Rarissa Rúbia Dallaqua Dos; Motonaga, Suely Mayumi; Silva, Roberta Gonçalves da

    2016-01-01

    Huntington's disease (HD) is a degenerative genetic disorder with autosomal-dominant transmission. The triad of symptoms of this disease consists of psychiatric disorders, jerky movements, and dementia. Oropharyngeal dysphagia, which is more evident with disease progression, is also present. Few studies have addressed the swallowing characteristics using objective analysis in this population. The purpose of this research was to describe the swallowing endoscopic findings of the pharyngeal phase in HD. This is a cross-sectional study addressing a clinical case which included two individuals of the same family, male, 32 and 63 years old, designated as individual A and individual B, with progression of the disease for five and 13 years, respectively. Consistent liquid, nectar, and puree were offered during the evaluation. There was presence of posterior oral spillage in liquid and nectar, small amount of pharyngeal residues, and no laryngeal penetration or aspiration in the individuals with HD in this study.

  17. Structural imaging in premanifest and manifest Huntington disease.

    Science.gov (United States)

    Scahill, Rachael I; Andre, Ralph; Tabrizi, Sarah J; Aylward, Elizabeth H

    2017-01-01

    Huntington disease (HD) neuropathology has a devastating effect on brain structure and consequently brain function; neuroimaging provides a means to assess these effects in gene carriers. In this chapter we first outline the unique utility of structural imaging in understanding HD and discuss some of the acquisition and analysis techniques currently available. We review the existing literature to summarize what we know so far about structural brain changes across the spectrum of disease from premanifest through to manifest disease. We then consider how these neuroimaging findings relate to patient function and nonimaging biomarkers, and can be used to predict disease onset. Finally we review the utility of imaging measures for assessment of treatment efficacy in clinical trials. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. [Speed of ocular saccades in Huntington disease. Prospective study].

    Science.gov (United States)

    García Ruiz, P J; Cenjor, C; Ulmer, E; Hernández, J; Cantarero, S; Fanjul, S; García de Yébenes, J

    2001-02-01

    Oculomotor abnormalities, especially slow saccades, have long been recognized in Huntington's disease (HD). To study prospectively horizontal saccade velocity by videonystagmography in 21 patients with genetically confirmed HD. The study included a baseline analysis and a second evaluation after 18.8 +/- 7.1 months. We included a control group of 15 subjects. HD group exhibited decreased saccade velocity when compared with that from a control group (for predictive and unpredictive target). HD patients showed decreased saccade velocity with the passage of time (for predictive target, p < 0.01). Finally we found statistical significant correlation between saccade velocity and triplet length. The measurement of saccade velocity might be an objective method to study the natural evolution of HD, and thus evaluate the effectiveness of future therapies.

  19. Brain atrophy in Huntington's disease: A CT-scan study

    International Nuclear Information System (INIS)

    Starkstein, S.E.; Folstein, S.E.; Brandt, J.; McDonnell, A.; Folstein, M.

    1989-01-01

    CT-scan measurements of cortical and subcortical atrophy were carried out in 34 patients with Huntington's disease (HD). While a significant correlation was observed between parameters of subcortical atrophy (bicaudate ratio, bifrontal ratio and third ventricular ratio) and duration of the disease, there was no significant correlation between these parameters and age. On the other hand, measurements of cortical atrophy (frontal fissure ratio and cortical sulci ratio) correlated significantly with age but not with duration of the disease. When a group of 24 HD patients were compared on CT-scan measurements with a group of 24 age-matched normal controls, significant differences were obtained for all the variables examined, but the bicaudate ratio showed the highest sensitivity and specificity. Even mildly affected patients, with duration of motor symptoms less than 3 years had higher bicaudate ratios than age-matched controls. (orig.)

  20. Nucleic Acid-Based Therapy Approaches for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Tatyana Vagner

    2012-01-01

    Full Text Available Huntington's disease (HD is caused by a dominant mutation that results in an unstable expansion of a CAG repeat in the huntingtin gene leading to a toxic gain of function in huntingtin protein which causes massive neurodegeneration mainly in the striatum and clinical symptoms associated with the disease. Since the mutation has multiple effects in the cell and the precise mechanism of the disease remains to be elucidated, gene therapy approaches have been developed that intervene in different aspects of the condition. These approaches include increasing expression of growth factors, decreasing levels of mutant huntingtin, and restoring cell metabolism and transcriptional balance. The aim of this paper is to outline the nucleic acid-based therapeutic strategies that have been tested to date.

  1. Reduced gluconeogenesis and lactate clearance in Huntington's disease

    DEFF Research Database (Denmark)

    Josefsen, Knud; Nielsen, Signe M B; Campos, André

    2010-01-01

    We studied systemic and brain glucose and lactate metabolism in Huntington's disease (HD) patients in response to ergometer cycling. Following termination of exercise, blood glucose increased abruptly in control subjects, but no peak was seen in any of the HD patients (2.0 ± 0.5 vs. 0.0 ± 0.2mM, P...... HD mouse model R6/2 following a lactate challenge, combined with reduced phosphoenolpyruvate carboxykinase and increased pyruvate kinase activity in the mouse liver suggest a reduced capacity...... for gluconeogenesis in HD, possibly contributing to the clinical symptoms of HD. We propose that blood glucose concentration in the recovery from exercise can be applied as a liver function test in HD patients....

  2. Exploring Genetic Factors Involved in Huntington Disease Age of Onset

    DEFF Research Database (Denmark)

    Valcárcel-Ocete, Leire; Alkorta-Aranburu, Gorka; Iriondo, Mikel

    2015-01-01

    age (motor AO or mAO). Multiple linear regression analyses were performed between genetic variation within 20 candidate genes and eAO or mAO, using DNA and clinical information of 253 HD patients from REGISTRY project. Gene expression analyses were carried out by RT-qPCR with an independent sample......Age of onset (AO) of Huntington disease (HD) is mainly determined by the length of the CAG repeat expansion (CAGexp) in exon 1 of the HTT gene. Additional genetic variation has been suggested to contribute to AO, although the mechanism by which it could affect AO is presently unknown. The aim...... of this study is to explore the contribution of candidate genetic factors to HD AO in order to gain insight into the pathogenic mechanisms underlying this disorder. For that purpose, two AO definitions were used: the earliest age with unequivocal signs of HD (earliest AO or eAO), and the first motor symptoms...

  3. Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey.

    Science.gov (United States)

    Bombard, Yvonne; Veenstra, Gerry; Friedman, Jan M; Creighton, Susan; Currie, Lauren; Paulsen, Jane S; Bottorff, Joan L; Hayden, Michael R

    2009-06-09

    To assess the nature and prevalence of genetic discrimination experienced by people at risk for Huntington's disease who had undergone genetic testing or remained untested. Cross sectional, self reported survey. Seven genetics and movement disorders clinics servicing rural and urban communities in Canada. 233 genetically tested and untested asymptomatic people at risk for Huntington's disease (response rate 80%): 167 underwent testing (83 had the Huntington's disease mutation, 84 did not) and 66 chose not to be tested. Self reported experiences of genetic discrimination and related psychological distress based on family history or genetic test results. Discrimination was reported by 93 respondents (39.9%). Reported experiences occurred most often in insurance (29.2%), family (15.5%), and social (12.4%) settings. There were few reports of discrimination in employment (6.9%), health care (8.6%), or public sector settings (3.9%). Although respondents who were aware that they carried the Huntington's disease mutation reported the highest levels of discrimination, participation in genetic testing was not associated with increased levels of genetic discrimination. Family history of Huntington's disease, rather than the result of genetic testing, was the main reason given for experiences of genetic discrimination. Psychological distress was associated with genetic discrimination (PGenetic discrimination was commonly reported by people at risk for Huntington's disease and was a source of psychological distress. Family history, and not genetic testing, was the major reason for genetic discrimination.

  4. The Addenbrooke's Cognitive Examination-Revised accurately detects cognitive decline in Huntington's disease.

    Science.gov (United States)

    Begeti, Faye; Tan, Adrian Y K; Cummins, Gemma A; Collins, Lucy M; Guzman, Natalie Valle; Mason, Sarah L; Barker, Roger A

    2013-11-01

    Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's disease varied according to disease severity with extensive cognitive decline observed in early-stage Huntington's disease patients, indicating that this would be an optimal stage for interventions designed to halt cognitive decline, and lesser changes in the advanced cases. We next examined cognitive performance in patients prescribed antidopaminergic drugs as these drugs are known to decrease cognition when administered to healthy volunteers. We paradoxically found that these drugs may be beneficial, as early-stage Huntington's disease participants in receipt of them had improved attention and Mini-Mental State Examination scores. In conclusion, this is the first study to test the usefulness of the ACE-R in a Huntington's disease population and demonstrates that this is a brief, inexpensive and practical way to measure global cognitive performance in clinical practice with potential use in clinical trials.

  5. Orphan drugs in development for Huntington's disease: challenges and progress

    Directory of Open Access Journals (Sweden)

    Burgunder JM

    2015-02-01

    advanced strategies to develop novel treatments in Huntington's disease are examined. Keywords: Huntington's disease, symptomatic treatment, disease-modifying therapy

  6. Use of flux and morphologic sediment budgets for sandbar monitoring on the Colorado River in Marble Canyon, Arizona

    Science.gov (United States)

    Grams, Paul E.; Buscombe, Daniel D.; Topping, David J.; Hazel, Joseph E.; Kaplinski, Matt

    2015-01-01

    The magnitude and pfattern of streamflow and sediment supply of the Colorado River in Grand Canyon (Figure 1) has been affected by the existence and operations of Glen Canyon Dam since filling of Lake Powell Reservoir began in March 1963. In the subsequent 30 years, fine sediment was scoured from the downstream channel (Topping et al., 2000; Grams et al., 2007), resulting in a decline in the number and size of sandbars in the eastern half of Grand Canyon National Park (Wright et al., 2005; Schmidt et al., 2004). The Glen Canyon Dam Adaptive Management Program (GCDAMP) administered by the U.S. Department of Interior oversees efforts to manage the Colorado River ecosystem downstream from Glen Canyon Dam. One of the goals of the GCDAMP is to maintain and increase the number and size of sandbars in this context of a limited sand supply. Management actions to benefit sandbars have included curtailment of daily streamflow fluctuations, which occur for hydropower generation, and implementation of controlled floods, also called high-flow experiments.Studies of controlled floods, defined as intentional releases that exceed the maximum discharge capacity of the Glen Canyon Dam powerplant, implemented between 1996 and 2008, have demonstrated that these events cause increases in sandbar size throughout Marble and Grand Canyons (Hazel et al., 2010; Schmidt and Grams, 2011; Mueller et al., 2014), although the magnitude of response is spatially variable (Hazel et al., 1999; 2010). Controlled floods may build some sandbars at the expense of erosion of sand from other, upstream, sandbars (Schmidt, 1999). To increase the frequency and effectiveness of sandbar building, the U.S. Department of Interior adopted a “high-flow experimental protocol” to implement controlled floods regularly under conditions of enriched sand supply (U.S. Department of Interior, 2012). Because the supply of sand available to build sandbars has been substantially reduced by Glen Canyon Dam (Topping et al

  7. Morphodynamic Model of Submarine Canyon Incision by Sandblasting

    Science.gov (United States)

    Zhang, L.; Parker, G.; Izumi, N.; Cartigny, M.; Li, T.; Wang, G.

    2017-12-01

    Submarine canyons are carved by turbidity currents under the deep sea. As opposed to subaerial canyons, the relevant processes are not easy to observe directly. Turbidity currents are bottom-hugging sediment gravity flows of that can incise or deposit on the seafloor to create submarine canyons or fans. The triggers of turbidity currents can be storms, edge waves, internal waves, canyon wall sapping, delta failure, breaching and hyperpycnal flows. The formation and evolution mechanisms of submarine canyons are similar to those of subaerial canyons, but have substantial differences. For example, sandblasting, rather than wear due to colliding gravel clasts is more likely to be the mechanism of bedrock incision. Submarine canyons incise downward, and often develop meander bends and levees within the canyon, so defining "fairways". Here we propose a simple model for canyon incision. The starting point of our model is the Macro Roughness Saltation Abrasion Alluviation model of Zhang et al. [2015], designed for bedrock incision by gravel clasts in mixed bedrock-alluvial rivers. We adapt this formulation to consider sandblasting as a means of wear. We use a layer-averaged model for turbidity current dynamics. The current contains a mixture of mud, which helps drive the flow but which does not cause incision, and sand, which is the agent of incision. We show that the model can successfully model channel downcutting, and indeed illustrate the early formation of net incisional cyclic steps, i.e. upstream-migrating undulations on the bed associated with transcritical (in the Froude sense) flow. These steps can be expected to abet the process of incision.

  8. Ensonifying Change: Repeat Ultra-High-Resolution Surveys in Monterey Canyon before and after Passage of a Turbidity Current

    Science.gov (United States)

    Wolfson-Schwehr, M.; Paull, C. K.; Caress, D. W.; Carvajal, C.; Thomas, H. J.; Maier, K. L.; Parsons, D. R.; Simmons, S.

    2017-12-01

    evolution of scours between events. While the scours may represent a small component of sediment transport within the canyon, their multi-generational structure indicates a complex interaction of scour processes along the canyon bed. These data provide a new means to understanding the detailed changes in canyon floor morphology and sedimentology at the event scale.

  9. Impacts of shape and height of upstream roof on airflow and pollutant dispersion inside an urban street canyon.

    Science.gov (United States)

    Huang, Yuan-Dong; He, Wen-Rong; Kim, Chang-Nyung

    2015-02-01

    A two-dimensional numerical model for simulating flow and pollutant dispersion in an urban street canyon is firstly developed using the FLUENT code and then validated against the wind tunnel results. After this, the flow field and pollutant dispersion inside an urban street canyon with aspect ratio W/H = 1 are examined numerically considering five different shapes (vaulted, trapezoidal, slanted, upward wedged, and downward wedged roofs) as well as three different roof height to building height ratios (Z H /H = 1/6, 1/3, and 1/2) for the upstream building roof. The results obtained reveal that the shape and height of an upstream roof have significant influences on flow pattern and pollutant distribution in an urban canyon. A large single clockwise vortex is generated in the canyon for the vaulted upstream roof at Z H /H = 1/6, 1/3, and 1/2, the trapezoidal and downward wedged roofs at Z H /H = 1/6 and 1/3, and the slanted and upward wedged roofs at Z H /H = 1/6, while a main clockwise vortex and a secondary counterclockwise vortex are established for the trapezoidal and downward wedged roofs at Z H /H = 1/2 and the slanted and upward wedged roofs at Z H /H = 1/3 and 1/2. In the one-vortex flow regime, the clockwise vortex moves upward and grows in size with increasing upstream roof height for the vaulted, trapezoidal, and downward wedged roofs. In the two-vortex flow regime, the size and rotational velocity of both upper clockwise and lower counterclockwise vortices increase with the upstream roof height for the slanted and upward wedged roofs. At Z H /H = 1/6, the pollution levels in the canyon are close among all the upstream roof shapes studied. At Z H /H = 1/3, the pollution levels in the canyon for the upward wedged roof and slanted roof are much higher than those for the vaulted, trapezoidal, and downward wedged roofs. At Z H /H = 1/2, the lowest pollution level appears in the canyon for the vaulted upstream roof, while

  10. AUV Mapping and ROV Exploration of Los Frailes Submarine Canyon, Cabo Pulmo National Marine Park, Baja California Sur, Mexico

    Science.gov (United States)

    Troni, G.; Caress, D. W.; Graves, D.; Thomas, H. J.; Thompson, D.; Barry, J. P.; Aburto-Oropeza, O.; Johnson, A. F.; Lundsten, L.

    2015-12-01

    Los Frailes submarine canyon is located at the south boundary of the Cabo Pulmo National Marine Park on the southeast tip of the Baja California Peninsula. During the Monterey Bay Aquarium Research Institute (MBARI) 2015 Gulf of California expedition we used an autonomous underwater vehicle (AUV) to map this canyon from 50 m to 450 m depths, and then explored the canyon with a small remotely operated vehicle (ROV). This three day R/V Rachel Carson cruise was a collaboration with the Center for Marine Biodiversity and Conservation at Scripps Institution of Oceanography and the Centro para la Biodiversidad Marina y la Conservación in La Paz. The MBARI AUV D. Allan B. collected high resolution bathymetry, sidescan, and subbottom profiles of Los Frailes submarine canyon and part of the north Cabo Pulmo deep reef. In order to safely generate a 1-m lateral resolution multibeam bathymetry map in the nearshore high relief terrain, the mapping operations consisted of an initial short survey following the 100-m isobath followed by a series of short, incremental AUV missions located on the deep edge of the new AUV bathymetry. The MBARI Mini-ROV was used to explore the submarine canyon within the detailed map created by the MBARI AUV. The Mini-ROV is a 1.2-m-long, 350 kg, 1,500-m-depth-rated ROV designed and constructed by MBARI. It is controlled by six 600-watt thrusters and is equipped with a high-definition video camera and navigation sensors. This small ROV carries less accurate, lower cost navigation sensors than larger vehicles. We implemented new algorithms to localize combining Doppler velocity log sensor data and low-cost MEMS-based inertial sensor data with sporadic ultra-short baseline position measurements to provide a high accuracy position estimation. The navigation performance allowed us to colocate the ROV video imagery with the 1-m resolution bathymetric map of the submarine canyon. Upper Los Frailes Canyon is rugged and, aside from small sand pockets along

  11. Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease.

    Science.gov (United States)

    Novak, Marianne J U; Warren, Jason D; Henley, Susie M D; Draganski, Bogdan; Frackowiak, Richard S; Tabrizi, Sarah J

    2012-04-01

    Huntington's disease is an inherited neurodegenerative disease that causes motor, cognitive and psychiatric impairment, including an early decline in ability to recognize emotional states in others. The pathophysiology underlying the earliest manifestations of the disease is not fully understood; the objective of our study was to clarify this. We used functional magnetic resonance imaging to investigate changes in brain mechanisms of emotion recognition in pre-manifest carriers of the abnormal Huntington's disease gene (subjects with pre-manifest Huntington's disease): 16 subjects with pre-manifest Huntington's disease and 14 control subjects underwent 1.5 tesla magnetic resonance scanning while viewing pictures of facial expressions from the Ekman and Friesen series. Disgust, anger and happiness were chosen as emotions of interest. Disgust is the emotion in which recognition deficits have most commonly been detected in Huntington's disease; anger is the emotion in which impaired recognition was detected in the largest behavioural study of emotion recognition in pre-manifest Huntington's disease to date; and happiness is a positive emotion to contrast with disgust and anger. Ekman facial expressions were also used to quantify emotion recognition accuracy outside the scanner and structural magnetic resonance imaging with voxel-based morphometry was used to assess the relationship between emotion recognition accuracy and regional grey matter volume. Emotion processing in pre-manifest Huntington's disease was associated with reduced neural activity for all three emotions in partially separable functional networks. Furthermore, the Huntington's disease-associated modulation of disgust and happiness processing was negatively correlated with genetic markers of pre-manifest disease progression in distributed, largely extrastriatal networks. The modulated disgust network included insulae, cingulate cortices, pre- and postcentral gyri, precunei, cunei, bilateral putamena

  12. Loss of residual heat removal system: Diablo Canyon, Unit 2, April 10, 1987

    International Nuclear Information System (INIS)

    1987-06-01

    This report presents the findings of an NRC Augmented Inspection Team (AIT) investigation into the circumstances associated with the loss of residual heat removal (RHR) system capability for a period of approximately one and one-half hours at the Diablo Canyon, Unit 2 reactor facility on April 10, 1987. This event occurred while the Diablo Canyon, Unit 2, a pressurized water reactor, was shutdown with the reactor coolant system (RCS) water level drained to approximately mid-level of the hot leg piping. The reactor containment building equipment hatch was removed at the time of the event, and plant personnel were in the process of removing the primary side manways to gain access into the steam generator channel head areas. Thus, two fission product barriers were breached throughout the event. The RCS temperature increased from approximately 87 0 F to bulk boiling conditions without RCS temperature indication available to the plant operators. The RCS was subsequently pressurized to approximately 7 to 10 psig. The NRC AIT members concluded that the Diablo Canyon, Unit 2 plant was, at the time of the event, in a condition not previously analyzed by the NRC staff. The AIT findings from this event appear significant and generic to other pressurized water reactor facilities licensed by the NRC

  13. Haze in the Grand Canyon: An evaluation of the Winter Haze Intensive Tracer Experiment

    Energy Technology Data Exchange (ETDEWEB)

    1990-10-01

    The Grand Canyon is one of the most spectacular natural sights on earth. Approximately 4 million visitors travel to Grand Canyon National Park (GCNP) each year to enjoy its majestic geological formations and intensely colored views. However, visibility in GCNP can be impaired by small increases in concentrations of fine suspended particles that scatter and absorb light; the resulting visibility degradation is perceived as haze. Sulfate particles are a major factor in visibility impairment at Grand Canyon in summer and winter. Many wintertime hazes at GCNP are believed to result from the accumulation of emissions from local sources during conditions of air stagnation, which occur more frequently in winter than in summer. In January and February 1987, the National Park Service (NPS) carried out a large-scale experiment known as the Winter Haze Intensive Tracer Experiment (WHITEX) to investigate the causes of wintertime haze in the region of GCNP and Canyonlands National Park. The overall objective of WHITEX was to assess the feasibility of attributing visibility impairment in specific geographic regions to emissions from a single point source. The experiment called for the injection of a tracer, deuterated methane (CD{sub 4}), into one of the stacks of the Navajo Generating Station (NGS), a major coal-fired power plant located 25 km from the GCNP boundary and 110 km northeast of Grand Canyon Village. A network of field stations was established in the vicinity -- mostly to the northeast of GCNP and NGS -- to measure CD{sub 4} concentrations, atmospheric aerosol and optical properties, and other chemical and physical attributes. 19 refs., 3 figs.

  14. Annotated bibliography for the humpback chub (Gila cypha) with emphasis on the Grand Canyon population.

    Energy Technology Data Exchange (ETDEWEB)

    Goulet, C. T.; LaGory, K. E.; Environmental Science Division

    2009-10-05

    Glen Canyon Dam is a hydroelectric facility located on the Colorado River in Arizona that is operated by the U.S. Bureau of Reclamation (Reclamation) for multiple purposes including water storage, flood control, power generation, recreation, and enhancement of fish and wildlife. Glen Canyon Dam operations have been managed for the last several years to improve conditions for the humpback chub (Gila cypha) and other ecosystem components. An extensive amount of literature has been produced on the humpback chub. We developed this annotated bibliography to assist managers and researchers in the Grand Canyon as they perform assessments, refine management strategies, and develop new studies to examine the factors affecting humpback chub. The U.S. Geological Survey recently created a multispecies bibliography (including references on the humpback chub) entitled Bibliography of Native Colorado River Big Fishes (available at www.fort.usgs.gov/Products/data/COFishBib). That bibliography, while quite extensive and broader in scope than ours, is not annotated, and, therefore, does not provide any of the information in the original literature. In developing this annotated bibliography, we have attempted to assemble abstracts from relevant published literature. We present here abstracts taken unmodified from individual reports and articles except where noted. The bibliography spans references from 1976 to 2009 and is organized in five broad topical areas, including: (1) biology, (2) ecology, (3) impacts of dam operations, (4) other impacts, and (5) conservation and management, and includes twenty subcategories. Within each subcategory, we present abstracts alphabetically by author and chronologically by year. We present relevant articles not specific to either the humpback chub or Glen Canyon Dam, but cited in other included reports, under the Supporting Articles subcategory. We provide all citations in alphabetical order in Section 7.

  15. Modeling In-Stream Hydro-Geomorphic Processes After 2012 Waldo Canyon Fire, Colorado

    Science.gov (United States)

    Nourbakhshbeidokhti, S.; Kinoshita, A. M.; Chin, A.

    2016-12-01

    Wildfires can have significant impacts on hydrologic and geomorphic processes. Post-fire sediment transport and runoff generation vary by burn severity, precipitation, and vegetation. A need exists to understand these variable relationships and improve parameterization of post-fire hydro-geomorphic models. This research aims to model pre-fire geomorphic and hydrologic processes in Williams Canyon, a watershed burned by the 2012 Waldo Canyon Fire in Colorado. We develop the KINematic Runoff and EROSion (KINEROS) model with Geographical Information System (GIS)-based information, including a Digital Elevation Model, land cover, soil classification, precipitation, and soil burn severity for a local reference watershed that is unburned. We transfer these parameters to a channel reach in Williams Canyon (Williams Downstream) and adjust them toward post-fire conditions. We model runoff and sediment yield for several storms following the fire. Three post-fire terrestrial Light Detection and Ranging (LiDAR) images (21 April 2013, 14 September 2013, and 16 September 2014) are used to estimate total erosion and deposition at the reach scale. We use the LiDAR-based information to calibrate the post-fire model. Preliminary modeling results indicate 3870-125 kg/ha of sediment in the Williams Downstream reach. The uncalibrated model overestimated (410% in the first year) and underestimated (87.2% in the second year) the erosion. Model calibration reduced the Root Mean Square Error (RMSE) of sediment to 0.016% for the first year and 0.09% for the second year. The parameters calibrated for the Williams Downstream channel reach will be used to develop models for seven other channel reaches within the area burned by the Waldo Canyon Fire, where the performance can be evaluated with LiDAR estimates. Results of this research will enhance our understanding of wildfire disturbance on coupled hydrologic and geomorphic processes. Findings will also improve model parameterization that can

  16. Test-Retest Reliability of Diffusion Tensor Imaging in Huntington's Disease.

    Science.gov (United States)

    Cole, James H; Farmer, Ruth E; Rees, Elin M; Johnson, Hans J; Frost, Chris; Scahill, Rachael I; Hobbs, Nicola Z

    2014-03-21

    Diffusion tensor imaging (DTI) has shown microstructural abnormalities in patients with Huntington's Disease (HD) and work is underway to characterise how these abnormalities change with disease progression. Using methods that will be applied in longitudinal research, we sought to establish the reliability of DTI in early HD patients and controls. Test-retest reliability, quantified using the intraclass correlation coefficient (ICC), was assessed using region-of-interest (ROI)-based white matter atlas and voxelwise approaches on repeat scan data from 22 participants (10 early HD, 12 controls). T1 data was used to generate further ROIs for analysis in a reduced sample of 18 participants. The results suggest that fractional anisotropy (FA) and other diffusivity metrics are generally highly reliable, with ICCs indicating considerably lower within-subject compared to between-subject variability in both HD patients and controls. Where ICC was low, particularly for the diffusivity measures in the caudate and putamen, this was partly influenced by outliers. The analysis suggests that the specific DTI methods used here are appropriate for cross-sectional research in HD, and give confidence that they can also be applied longitudinally, although this requires further investigation. An important caveat for DTI studies is that test-retest reliability may not be evenly distributed throughout the brain whereby highly anisotropic white matter regions tended to show lower relative within-subject variability than other white or grey matter regions.

  17. Nanopublications for exposing experimental data in the life-sciences: a Huntington's Disease case study.

    Science.gov (United States)

    Mina, Eleni; Thompson, Mark; Kaliyaperumal, Rajaram; Zhao, Jun; der Horst, van Eelke; Tatum, Zuotian; Hettne, Kristina M; Schultes, Erik A; Mons, Barend; Roos, Marco

    2015-01-01

    Data from high throughput experiments often produce far more results than can ever appear in the main text or tables of a single research article. In these cases, the majority of new associations are often archived either as supplemental information in an arbitrary format or in publisher-independent databases that can be difficult to find. These data are not only lost from scientific discourse, but are also elusive to automated search, retrieval and processing. Here, we use the nanopublication model to make scientific assertions that were concluded from a workflow analysis of Huntington's Disease data machine-readable, interoperable, and citable. We followed the nanopublication guidelines to semantically model our assertions as well as their provenance metadata and authorship. We demonstrate interoperability by linking nanopublication provenance to the Research Object model. These results indicate that nanopublications can provide an incentive for researchers to expose data that is interoperable and machine-readable for future use and preservation for which they can get credits for their effort. Nanopublications can have a leading role into hypotheses generation offering opportunities to produce large-scale data integration.

  18. Huntington's disease accelerates epigenetic aging of human brain and disrupts DNA methylation levels.

    Science.gov (United States)

    Horvath, Steve; Langfelder, Peter; Kwak, Seung; Aaronson, Jeff; Rosinski, Jim; Vogt, Thomas F; Eszes, Marika; Faull, Richard L M; Curtis, Maurice A; Waldvogel, Henry J; Choi, Oi-Wa; Tung, Spencer; Vinters, Harry V; Coppola, Giovanni; Yang, X William

    2016-07-01

    Age of Huntington's disease (HD) motoric onset is strongly related to the number of CAG trinucleotide repeats in the huntingtin gene, suggesting that biological tissue age plays an important role in disease etiology. Recently, a DNA methylation based biomarker of tissue age has been advanced as an epigenetic aging clock. We sought to inquire if HD is associated with an accelerated epigenetic age. DNA methylation data was generated for 475 brain samples from various brain regions of 26 HD cases and 39 controls. Overall, brain regions from HD cases exhibit a significant epigenetic age acceleration effect (p=0.0012). A multivariate model analysis suggests that HD status increases biological age by 3.2 years. Accelerated epigenetic age can be observed in specific brain regions (frontal lobe, parietal lobe, and cingulate gyrus). After excluding controls, we observe a negative correlation (r=-0.41, p=5.5×10-8) between HD gene CAG repeat length and the epigenetic age of HD brain samples. Using correlation network analysis, we identify 11 co-methylation modules with a significant association with HD status across 3 broad cortical regions. In conclusion, HD is associated with an accelerated epigenetic age of specific brain regions and more broadly with substantial changes in brain methylation levels.

  19. Environmental factors as modulators of neurodegeneration: insights from gene-environment interactions in Huntington's disease.

    Science.gov (United States)

    Mo, Christina; Hannan, Anthony J; Renoir, Thibault

    2015-05-01

    Unlike many other neurodegenerative diseases with established gene-environment interactions, Huntington's disease (HD) is viewed as a disorder governed by genetics. The cause of the disease is a highly penetrant tandem repeat expansion encoding an extended polyglutamine tract in the huntingtin protein. In the year 2000, a pioneering study showed that the disease could be delayed in transgenic mice by enriched housing conditions. This review describes subsequent human and preclinical studies identifying environmental modulation of motor, cognitive, affective and other symptoms found in HD. Alongside the behavioral observations we also discuss potential mechanisms and the relevance to other neurodegenerative disorders, including Alzheimer's and Parkinson's disease. In mouse models of HD, increased sensorimotor and cognitive stimulation can delay or ameliorate various endophenotypes. Potential mechanisms include increased trophic support, synaptic plasticity, adult neurogenesis, and other forms of experience-dependent cellular plasticity. Subsequent clinical investigations support a role for lifetime activity levels in modulating the onset and progression of HD. Stress can accelerate memory and olfactory deficits and exacerbate cellular dysfunctions in HD mice. In the absence of effective treatments to slow the course of HD, environmental interventions offer feasible approaches to delay the disease, however further preclinical and human studies are needed in order to generate clinical recommendations. Environmental interventions could be combined with future pharmacological therapies and stimulate the identification of enviromimetics, drugs which mimic or enhance the beneficial effects of cognitive stimulation and physical activity. Copyright © 2015. Published by Elsevier Ltd.

  20. Radionuclides at the Hudson Canyon disposal site

    International Nuclear Information System (INIS)

    Schell, W.R.; Nevissi, A.E.

    1983-01-01

    A sampling and analytical program was initiated in June 1978 to measure radionuclides in water, sediments, and biota collected at the deepwater (4000 m) radioactive waste disposal site at the mouth of the Hudson Canyon 350km off New York Harbor in the western Atlantic Ocean. Plutonium, americium, cesium, strontium, and uranium series isotopes were measured in selected samples; the /sup 210/Pb data were used to give sedimentation and mixing rates in the upper sediment layers. The results showed that /sup 137/Cs, /sup 239,240/Pu, and /sup 238/Pu were found at low concentrations in the skin, viscera, and stomach contents for some of the fish collected. Significant concentrations of /sup 241/Am were found in tissues of the common rattail Coryphaenoides (Macrouridae) collected at the disposal site, suggesting a local source for this radionuclide and biological accumulation. The edible muscle of this fish contained less than 2.6 x 10/sup -5/ Bq g/sup -1/ (dry wt) of /sup 239,240/Pu. Radionuclides measured in sediment-core profiles showed that mixing occurred to depths of 16 cm and that variable sedimentation or mixing rates, or both, exist at 4000 m deep. Radionuclide deposition near the canisters was not found to be significantly higher than the expected fallout levels at 4000 m deep. At the mouth of the Hudson Canyon variable sedimentation and mixing rates were found using the natural unsupported /sup 210/Pb tracer values; these variable rates were attributed to sediment transport by the currents and to bioturbation

  1. Final Technical Report - Modernization of the Boulder Canyon Hydroelectric Project

    Energy Technology Data Exchange (ETDEWEB)

    Taddeucci, Joe [Dept. of Public Works, Boulder, CO (United States). Utilities Division

    2013-03-29

    The Boulder Canyon Hydroelectric Project (BCH) was purchased by the City of Boulder, CO (the city) in 2001. Project facilities were originally constructed in 1910 and upgraded in the 1930s and 1940s. By 2009, the two 10 MW turbine/generators had reached or were nearing the end of their useful lives. One generator had grounded out and was beyond repair, reducing plant capacity to 10 MW. The remaining 10 MW unit was expected to fail at any time. When the BCH power plant was originally constructed, a sizeable water supply was available for the sole purpose of hydroelectric power generation. Between 1950 and 2001, that water supply had gradually been converted to municipal water supply by the city. By 2001, the water available for hydroelectric power generation at BCH could not support even one 10 MW unit. Boulder lacked the financial resources to modernize the facilities, and Boulder anticipated that when the single, operational historical unit failed, the project would cease operation. In 2009, the City of Boulder applied for and received a U.S. Department of Energy (DOE) grant for $1.18 million toward a total estimated project cost of $5.155 million to modernize BCH. The federal funding allowed Boulder to move forward with plant modifications that would ensure BCH would continue operation. Federal funding was made available through the American Recovery and Reinvestment Act (ARRA) of 2009. Boulder determined that a single 5 MW turbine/generator would be the most appropriate capacity, given the reduced water supply to the plant. Average annual BCH generation with the old 10 MW unit had been about 8,500 MW-hr, whereas annual generation with a new, efficient turbine could average 11,000 to 12,000 MW-hr. The incremental change in annual generation represents a 30% increase in generation over pre-project conditions. The old turbine/generator was a single nozzle Pelton turbine with a 5-to-1 flow turndown and a maximum turbine/generator efficiency of 82%. The new unit is a

  2. Distributions and habitat associations of deep-water corals in Norfolk and Baltimore Canyons, Mid-Atlantic Bight, USA

    NARCIS (Netherlands)

    Brooke, S.D.; Watts, M.W.; Heil, A.D.; Rhode, M.; Mienis, F.; Duineveld, G.C.A.; Davies, A.J.; Ross, S.W.

    2017-01-01

    A multi-disciplinary study of two major submarine canyons, Baltimore Canyon and Norfolk Canyon, off the US mid-Atlantic coast focused on the ecology and biology of canyon habitats, particularly those supporting deep-sea corals. Historical data on deep-sea corals from these canyons were sparse with

  3. Rose Canyon Sustainable Aquaculture Project, San Diego, CA

    Science.gov (United States)

    Documents related to EPA's preparation of an Environmental Assessment (EA) to analyze the potential impacts related to the issuance of a National Pollutant Discharge Elimination System (NPDES) permit for the Rose Canyon Sustainable Aquaculture Project.

  4. Habitat Mapping Cruise - Hudson Canyon (HB0904, EK60)

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Objectives are to: 1) perform multibeam mapping of transitional and deepwater habitats in Hudson Canyon (off New Jersey) with the National Institute of Undersea...

  5. Safety Evaluation for Packaging (onsite) T Plant Canyon Items

    International Nuclear Information System (INIS)

    OBRIEN, J.H.

    2000-01-01

    This safety evaluation for packaging (SEP) evaluates and documents the ability to safely ship mostly unique inventories of miscellaneous T Plant canyon waste items (T-P Items) encountered during the canyon deck clean off campaign. In addition, this SEP addresses contaminated items and material that may be shipped in a strong tight package (STP). The shipments meet the criteria for onsite shipments as specified by Fluor Hanford in HNF-PRO-154, Responsibilities and Procedures for all Hazardous Material Shipments

  6. Diablo Canyon plant information management system and integrated communication system

    International Nuclear Information System (INIS)

    Stanley, J.W.; Groff, C.

    1990-01-01

    The implementation of a comprehensive maintenance system called the plant information management system (PIMS) at the Diablo Canyon plant, together with its associated integrated communication system (ICS), is widely regarded as the most comprehensive undertaking of its kind in the nuclear industry. This paper provides an overview of the program at Diablo Canyon, an evaluation of system benefits, and highlights the future course of PIMS

  7. Diablo Canyon plant information management system and integrated communication system

    Energy Technology Data Exchange (ETDEWEB)

    Stanley, J.W.; Groff, C.

    1990-06-01

    The implementation of a comprehensive maintenance system called the plant information management system (PIMS) at the Diablo Canyon plant, together with its associated integrated communication system (ICS), is widely regarded as the most comprehensive undertaking of its kind in the nuclear industry. This paper provides an overview of the program at Diablo Canyon, an evaluation of system benefits, and highlights the future course of PIMS.

  8. Safety Evaluation for Packaging (onsite) T Plant Canyon Items

    Energy Technology Data Exchange (ETDEWEB)

    OBRIEN, J.H.

    2000-07-14

    This safety evaluation for packaging (SEP) evaluates and documents the ability to safely ship mostly unique inventories of miscellaneous T Plant canyon waste items (T-P Items) encountered during the canyon deck clean off campaign. In addition, this SEP addresses contaminated items and material that may be shipped in a strong tight package (STP). The shipments meet the criteria for onsite shipments as specified by Fluor Hanford in HNF-PRO-154, Responsibilities and Procedures for all Hazardous Material Shipments.

  9. B-Plant Canyon Ventilation Control System Description; FINAL

    International Nuclear Information System (INIS)

    MCDANIEL, K.S.

    1999-01-01

    Project W-059 installed a new B Plant Canyon Ventilation System. Monitoring and control of the system is implemented by the Canyon Ventilation Control System (CVCS). This document describes the CVCS system components which include a Programmable Logic Controller (PLC) coupled with an Operator Interface Unit (OIU) and application software. This document also includes an Alarm Index specifying the setpoints and technical basis for system analog and digital alarms

  10. 6 Ma age of carving Westernmost Grand Canyon: Reconciling geologic data with combined AFT, (U-Th)/He, and 4He/3He thermochronologic data

    Science.gov (United States)

    Winn, Carmen; Karlstrom, Karl E.; Shuster, David L.; Kelley, Shari; Fox, Matthew

    2017-09-01

    Conflicting hypotheses about the timing of carving of the Grand Canyon involve either a 70 Ma (;old;) or conflict with these lines of evidence, but are reconciled in this paper via the integration of three methods of analyses on the same sample: apatite (U-Th)/He ages (AHe), 4He/3He thermochronometry (4He/3He), and apatite fission-track ages and lengths (AFT). HeFTy software was used to generate time-temperature (t-T) paths that predict all new and published 4He/3He, AHe, and AFT data to within assumed uncertainties. These t-T paths show cooling from ∼100 °C to 40-60 °C in the Laramide (70-50 Ma), long-term residence at 40-60 °C in the mid-Tertiary (50-10 Ma), and cooling to near-surface temperatures after 10 Ma, and thus support young incision of the westernmost Grand Canyon. A subset of AHe data, when interpreted alone (i.e. without 4He/3He or AFT data), are better predicted by t-T paths that cool to surface temperatures during the Laramide, consistent with an ;old; Grand Canyon. However, the combined AFT, AHe, and 4He/3He analysis of a key sample from Separation Canyon can only be reconciled by a ;young; Canyon. Additional new AFT (5 samples) and AHe data (3 samples) in several locations along the canyon corridor also support a ;young; Canyon. This inconsistency, which mimics the overall controversy of the age of the Grand Canyon, is reconciled here by optimizing cooling paths so they are most consistent with multiple thermochronometers from the same rocks. To do this, we adjusted model parameters and uncertainties to account for uncertainty in the rate of radiation damage annealing in these apatites during sedimentary burial and the resulting variations in He retentivity. In westernmost Grand Canyon, peak burial conditions (temperature and duration) during the Laramide were likely insufficient to fully anneal radiation damage that accumulated during prolonged, near-surface residence since the Proterozoic. We conclude that application of multiple

  11. Is Canyon Width a Diagnostic Indicator of the Discharge of Megafloods on Earth and Mars?

    Science.gov (United States)

    Lapotre, M. G.; Lamb, M. P.

    2013-12-01

    On Earth, large floods have carved steep-walled and amphitheater-headed canyons from the Pleistocene (e.g. Box Canyon, ID) through the Holocene (e.g. Asbyrgi Canyon, Iceland), to historic times (e.g. Canyon Lake Gorge, TX). The geologic record on Mars suggests that similar floods have carved canyons by waterfall retreat about 3.5 billion years ago, when the red planet was wetter and possibly warmer. We currently lack robust paleo-hydraulic tools to reconstruct the discharge of ancient floods, especially on Mars where sediment sizes are obscured from observation. To address this issue, we hypothesize that the width of canyon escarpment is controlled by the hydraulics of the canyon-carving flood due to focusing of the flood into the canyon head. We compiled field data from multiple canyons and floods on Earth and Mars and show that there is a correlation between estimated flood discharge and canyon headwall width. To explore what sets this relationship, we identified five important parameters using dimensional analysis: the Froude number, the ratio of backwater length to canyon length, the ratio of backwater length to flood width, the ratio of canyon width to flood width, and the topographic slope upstream of the canyon. We used the hydraulic numerical modeling suite ANUGA to simulate overland flow over different canyon geometries and flood parameters to systematically explore the relative bed shear stresses along the canyon rim as a metric for flow focusing. Results show that canyons that exceed a certain length, scaling with the hydraulic backwater length, have shear stresses at their heads that are significantly higher than near the canyon mouth. Shear stresses along the rim of the canyon sidewalls are limited, in comparison to stresses along the canyon head, when the flood width is of the order of the backwater length. Flow focusing only occurs for subcritical flow. Together, these results suggest that canyons may only grow from a perturbation that is large

  12. [Effect of greenbelt on pollutant dispersion in street canyon].

    Science.gov (United States)

    Xu, Wei-Jia; Xing, Hong; Yu, Zhi

    2012-02-01

    The effect feature of greenbelt on flow field and pollutant dispersion in urban street canyon was researched. The greenbelt was assumed as uniform porous media and its aerodynamics property defined by the pressure loss coefficient. Subsequently, the pollutant dispersion in the street canyon of which there was greenbelt in the middle was simulated with the steady-state standard kappa-epsilon turbulence model and species transport equation. The simulated results agreed well with the wind-tunnel data. Compared with the treeless case, it finds that the street canyon contain a clockwise vortex, the pollutant concentration of the leeward was several times than the windward and the growth rate of pollutant concentration was 46.0%. The further simulation for the impact of tree crown position on the airflow and pollutant dispersion finds that the height of major vortex center in the street canyon increases with the height of tree crown and gradually closes the top of windward building This causes that the average wind speed in the street canyon decreases. Especially when the top of tree crown over the roof and hinder the air flow above the street canyon, the average pollutant concentration increases with the height of tree crown rapidly.

  13. Huntington Disease - principles and practice of nutritional management.

    Science.gov (United States)

    Zukiewicz-Sobczak, Wioletta; Król, Renata; Wróblewska, Paula; Piątek, Jacek; Gibas-Dorna, Magdalena

    2014-01-01

    Huntington disease (HD) is a degenerative brain disease clinically manifested by the characteristic triad: physical symptoms including involuntary movements and poor coordination, cognitive changes with less ability to organize routine tasks, and some emotional and behavioral disturbances. For patients with HD, feeding is one of the problems they have to face. People with HD often have lower than average body weight and struggle with malnutrition. As a part of therapy, good nutrition is an intervention maintaining health and functional ability for maximally prolonged time. In the early stages of HD, small amounts of blenderized foods given orally are recommended. In more advanced stages, enteral nutrition is essential using gastric, or jejunal tubes for short term. Most severe cases require gastrostomy or gastrojejunostomy. Although enteral feeding is well tolerated by most of the patients, a number of complications may occur, including damage to the nose, pharynx, or esophagus, aspiration pneumonia, sinusitis, metabolic imbalances due to improper nutrient and fluid supply, adverse effects affecting gastrointestinal system, and refeeding syndrome. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  14. The story of George Huntington and his disease

    Directory of Open Access Journals (Sweden)

    Kalyan B Bhattacharyya

    2016-01-01

    Full Text Available George Huntington described some families with choreiform movements in 1872 in the United States of America and since then many such families have been described in other parts of the world and works on the genetics of the disease have brought new vistas in the understanding of the disease. In 1958, Americo Negrette, a young Venezuelan physician observed similar subjects in the vicinity of Lake Maracaibo which was presented by his co-worker, Ramon Avilla Giron at New York in 1972 when United States of America had been commemorating the centenary year of Huntington′s disease. Nancy Wexler, a psychoanalyst, whose mother had been suffering from the disease attended the meeting and organized a research team to Venezuela and they systematically studied more than 18,000 individuals in order to work out a common pedigree. They identified the genetic locus of the disease in the short arm of chromosome 4 and observed that it was a trinucleotide repeat disorder.

  15. Bradykinesia in Huntington's disease. A prospective, follow-up study.

    Science.gov (United States)

    García Ruiz, Pedro J; Hernández, Jaime; Cantarero, Susana; Bartolomé, Manuel; Sánchez Bernardos, Vicenta; García de Yébenez, Justo

    2002-04-01

    Bradykinesia is a frequent finding in Huntington's disease (HD), but some aspects are presently unknown; including the natural evolution of bradykinesia over time and the correlation between bradykinesia and functional capacity. We studied the motor performance of 20 genetically confirmed patients with HD (age: 40+/-10.8 years; age at onset 33.6+/-11 years; total functional capacity (TFC): 9.57+/-3; UHDRS total motor scale: 31.4+/-13, triplet length (CAG)n: 46.7+/-4 triplets). These patients were studied in baseline conditions and after 18.7+/-6 months of follow-up. In addition, HD patients were compared with 20 age-matched normal controls. Motor study included the four CAPIT timed tests commonly used for Parkinson's disease: pronation-supination (PS), finger dexterity (FD), movement between two points (MTP) and walking test (WT). HD patients were significantly slower than controls in all motor tasks. A significant deterioration occurred over time in three of the four motor tasks (especially FD and WT). A significant correlation between timed tests and TFC score was found (for MTP, r: -0.845; p < 0,0001). In addition a significant correlation between timed tests and the UHDRDS total motor scale was also found (for MTP, r: 0.864; p < 0.0001). In conclusion, simple timed motor tests can detect a deterioration of motor activity over time in HD. Timed tests might be useful to follow the natural evolution of HD and to assess the efficacy of new therapies.

  16. Medical management of motor manifestations of Huntington disease.

    Science.gov (United States)

    McCusker, Elizabeth A; Loy, Clement T

    2017-01-01

    The motor and movement disorders of Huntington disease (HD) are managed in the context of the other disease features. Chorea and dystonia are the most common HD-associated movement disorders, and they can be assessed on research rating scales. However other motor manifestations have a significant impact. In particular, dysphagia influences choice and tolerance of treatment for the movement disorder, as will comorbidities, patient awareness, and distress related to the motor feature or movement. Treatment for other disease features may aggravate the motor disorder, e.g., increased swallowing difficulty associated with antipsychotic agents. Basic principles in deciding to institute a treatment are outlined as well as treatment of specific motor manifestations and movements. There is a paucity of evidence to support the treatments available for the motor disorder, with only one agent with class 1 evidence, tetrabenazine, for chorea. There are, however, treatments informed by expert opinion which reflect the management of a wider HD phenotype than that represented in clinical trials. Some treatments are based on evidence from use in other conditions. Medical management is usually undertaken later in the disease with concurrent nonmedical interventions after multidisciplinary assessments. Medication review with HD progression is essential. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. Pitfalls in the detection of cholesterol in Huntington's disease models.

    Science.gov (United States)

    Marullo, Manuela; Valenza, Marta; Leoni, Valerio; Caccia, Claudio; Scarlatti, Chiara; De Mario, Agnese; Zuccato, Chiara; Di Donato, Stefano; Carafoli, Ernesto; Cattaneo, Elena

    2012-10-11

    Background Abnormalities in brain cholesterol homeostasis have been reported in Huntington's disease (HD), an adult-onset neurodegenerative disorder caused by an expansion in the number of CAG repeats in the huntingtin (HTT) gene. However, the results have been contradictory with respect to whether cholesterol levels increase or decrease in HD models. Biochemical and mass spectrometry methods show reduced levels of cholesterol precursors and cholesterol in HD cells and in the brains of several HD animal models. Abnormal brain cholesterol homeostasis was also inferred from studies in HD patients. In contrast, colorimetric and enzymatic methods indicate cholesterol accumulation in HD cells and tissues. Here we used several methods to investigate cholesterol levels in cultured cells in the presence or absence of mutant HTT protein. Results Colorimetric and enzymatic methods with low sensitivity gave variable results, whereas results from a sensitive analytical method, gas chromatography-mass spectrometry, were more reliable. Sample preparation, high cell density and cell clonality also influenced the detection of intracellular cholesterol. Conclusions Detection of cholesterol in HD samples by colorimetric and enzymatic assays should be supplemented by detection using more sensitive analytical methods. Care must be taken to prepare the sample appropriately. By evaluating lathosterol levels using isotopic dilution mass spectrometry, we confirmed reduced cholesterol biosynthesis in knock-in cells expressing the polyQ mutation in a constitutive or inducible manner. *Correspondence should be addressed to Elena Cattaneo: elena.cattaneo@unimi.it.

  18. Mitochondrial DNA levels in Huntington disease leukocytes and dermal fibroblasts.

    Science.gov (United States)

    Jędrak, Paulina; Krygier, Magdalena; Tońska, Katarzyna; Drozd, Małgorzata; Kaliszewska, Magdalena; Bartnik, Ewa; Sołtan, Witold; Sitek, Emilia J; Stanisławska-Sachadyn, Anna; Limon, Janusz; Sławek, Jarosław; Węgrzyn, Grzegorz; Barańska, Sylwia

    2017-08-01

    Huntington disease (HD) is an inherited neurodegenerative disorder caused by mutations in the huntingtin gene. Involvement of mitochondrial dysfunctions in, and especially influence of the level of mitochondrial DNA (mtDNA) on, development of this disease is unclear. Here, samples of blood from 84 HD patients and 79 controls, and dermal fibroblasts from 10 HD patients and 9 controls were analysed for mtDNA levels. Although the type of mitochondrial haplogroup had no influence on the mtDNA level, and there was no correlation between mtDNA level in leukocytes in HD patients and various parameters of HD severity, some considerable differences between HD patients and controls were identified. The average mtDNA/nDNA relative copy number was significantly higher in leukocytes, but lower in fibroblasts, of symptomatic HD patients relative to the control group. Moreover, HD women displayed higher mtDNA levels in leukocytes than HD men. Because this is the largest population analysed to date, these results might contribute to explanation of discrepancies between previously published studies concerning levels of mtDNA in cells of HD patients. We suggest that the size of the investigated population and type of cells from which DNA is isolated could significantly affect results of mtDNA copy number estimation in HD. Hence, these parameters should be taken into consideration in studies on mtDNA in HD, and perhaps also in other diseases where mitochondrial dysfunction occurs.

  19. Cognitive and behavioral changes in Huntington disease before diagnosis.

    Science.gov (United States)

    Paulsen, Jane S; Miller, Amanda C; Hayes, Terry; Shaw, Emily

    2017-01-01

    Phenotypic manifestations of Huntington disease (HD) can be detected at least 15 years prior to the time when a motor diagnosis is given. Advances in clinical care and future research will require consistent use of HD definitions and HD premanifest (prodromal) stages being used across clinics, sites, and countries. Cognitive and behavioral (psychiatric) changes in HD are summarized and implications for ongoing advancement in our knowledge of prodromal HD are suggested. The earliest detected cognitive changes are observed in the Symbol Digit Modalities Test, Stroop Interference, Stroop Color and Word Test-interference condition, and Trail Making Test. Cognitive changes in the middle and near motor diagnostic stages of prodromal HD involve nearly every cognitive test administered and the greatest changes over time (i.e., slopes) are found in those prodromal HD participants who are nearest to motor diagnosis. Psychiatric changes demonstrate significant worsening over time and remain elevated compared with healthy controls throughout the prodromal disease course. Psychiatric and behavior changes in prodromal HD are much lower than that obtained using cognitive assessment, although the psychiatric and behavioral changes represent symptoms most debilitating to independent capacity and wellness. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Controlled clinical trial of cannabidiol in Huntington's disease.

    Science.gov (United States)

    Consroe, P; Laguna, J; Allender, J; Snider, S; Stern, L; Sandyk, R; Kennedy, K; Schram, K

    1991-11-01

    Based on encouraging preliminary findings, cannabidiol (CBD), a major nonpsychotropic constituent of Cannabis, was evaluated for symptomatic efficacy and safety in 15 neuroleptic-free patients with Huntington's Disease (HD). The effects of oral CBD (10 mg/kg/day for 6 weeks) and placebo (sesame oil for 6 weeks) were ascertained weekly under a double-blind, randomized cross-over design. A comparison of the effects of CBD and placebo on chorea severity and other therapeutic outcome variables, and on a Cannabis side effect inventory, clinical lab tests and other safety outcome variables, indicated no significant (p greater than 0.05) or clinically important differences. Correspondingly, plasma levels of CBD were assayed by GC/MS, and the weekly levels (mean range of 5.9 to 11.2 ng/ml) did not differ significantly over the 6 weeks of CBD administration. In summary, CBD, at an average daily dose of about 700 mg/day for 6 weeks, was neither symptomatically effective nor toxic, relative to placebo, in neuroleptic-free patients with HD.

  1. Predictive gene testing for Huntington disease and other neurodegenerative disorders.

    Science.gov (United States)

    Wedderburn, S; Panegyres, P K; Andrew, S; Goldblatt, J; Liebeck, T; McGrath, F; Wiltshire, M; Pestell, C; Lee, J; Beilby, J

    2013-12-01

    Controversies exist around predictive testing (PT) programmes in neurodegenerative disorders. This study sets out to answer the following questions relating to Huntington disease (HD) and other neurodegenerative disorders: differences between these patients in their PT journeys, why and when individuals withdraw from PT, and decision-making processes regarding reproductive genetic testing. A case series analysis of patients having PT from the multidisciplinary Western Australian centre for PT over the past 20 years was performed using internationally recognised guidelines for predictive gene testing in neurodegenerative disorders. Of 740 at-risk patients, 518 applied for PT: 466 at risk of HD, 52 at risk of other neurodegenerative disorders - spinocerebellar ataxias, hereditary prion disease and familial Alzheimer disease. Thirteen percent withdrew from PT - 80.32% of withdrawals occurred during counselling stages. Major withdrawal reasons related to timing in the patients' lives or unknown as the patient did not disclose the reason. Thirty-eight HD individuals had reproductive genetic testing: 34 initiated prenatal testing (of which eight withdrew from the process) and four initiated pre-implantation genetic diagnosis. There was no recorded or other evidence of major psychological reactions or suicides during PT. People withdrew from PT in relation to life stages and reasons that are unknown. Our findings emphasise the importance of: (i) adherence to internationally recommended guidelines for PT; (ii) the role of the multidisciplinary team in risk minimisation; and (iii) patient selection. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

  2. Constitutive upregulation of chaperone-mediated autophagy in Huntington's disease.

    Science.gov (United States)

    Koga, Hiroshi; Martinez-Vicente, Marta; Arias, Esperanza; Kaushik, Susmita; Sulzer, David; Cuervo, Ana Maria

    2011-12-14

    Autophagy contributes to the removal of prone-to-aggregate proteins, but in several instances these pathogenic proteins have been shown to interfere with autophagic activity. In the case of Huntington's disease (HD), a congenital neurodegenerative disorder resulting from mutation in the huntingtin protein, we have previously described that the mutant protein interferes with the ability of autophagic vacuoles to recognize cytosolic cargo. Growing evidence supports the existence of cross talk among autophagic pathways, suggesting the possibility of functional compensation when one of them is compromised. In this study, we have identified a compensatory upregulation of chaperone-mediated autophagy (CMA) in different cellular and mouse models of HD. Components of CMA, namely the lysosome-associated membrane protein type 2A (LAMP-2A) and lysosomal-hsc70, are markedly increased in HD models. The increase in LAMP-2A is achieved through both an increase in the stability of this protein at the lysosomal membrane and transcriptional upregulation of this splice variant of the lamp-2 gene. We propose that CMA activity increases in response to macroautophagic dysfunction in the early stages of HD, but that the efficiency of this compensatory mechanism may decrease with age and so contribute to cellular failure and the onset of pathological manifestations.

  3. Huntington's disease in Greece: the experience of 14 years.

    Science.gov (United States)

    Panas, M; Karadima, G; Vassos, E; Kalfakis, N; Kladi, A; Christodoulou, K; Vassilopoulos, D

    2011-12-01

    A large scale genetic and epidemiological study of Huntington's disease (HD) was carried out in Greece from January 1995 to December 2008. Diagnostic testing was carried out in 461 symptomatic individuals, while 256 were tested for presymptomatic purposes. The diagnosis of HD with a CAG expansion ≥ 36 was confirmed in 278 symptomatic individuals. The prevalence of HD in Greece was estimated at approximately 2.5 to 5.4:100,000, while the mean minimum incidence was estimated at 2.2 to 4.4 per million per year. The molecular diagnosis of HD was confirmed in the majority of patients (84.4%) sent for confirmation. The false-positive cases 15.6% were characterized by the absence of a family history of HD and the presence of an atypical clinical picture. The uptake of predictive testing for HD was 8.6%. A prenatal test was requested in six pregnancies. The findings of our study do not differ significantly from those of similar studies from other European countries despite the relative genetic isolation of Greece. Of interest is the identification of clusters of HD in Greece. The presence or absence of a family history of HD should be interpreted cautiously, during the diagnostic process. © 2010 John Wiley & Sons A/S.

  4. Plasma homovanillic acid and prolactin in Huntington's disease.

    Science.gov (United States)

    Markianos, Manolis; Panas, Marios; Kalfakis, Nikos; Vassilopoulos, Dimitrios

    2009-05-01

    Dopaminergic activity is expected to be altered in patients with Huntington's disease (HD) and be related to factors like duration and severity of illness or patients' specific symptomatology like dementia, depression, or psychotic features. We assessed plasma homovanillic acid (pHVA) and plasma prolactin (pPRL), two correlates of dopaminergic activity, in 116 subjects with CAG repeats expansion in the HD gene, 26 presymptomatic (18 females) and 90 with overt symptomatology (43 females). Patients were evaluated using the Unified HD Rating Scale and the Total Functional Capacity Scale. Presence of dementia, depression, and psychotic features were also assessed. The age range of the patients was 22-83 years, duration of illness from 0.5 to 27 years, and CAG repeat number from 34 to 66. A group of 60 age and sex matched healthy subjects served as control group. Plasma PRL in subjects at risk and in neuroleptic-free patients, evaluated separately for males and females, did not differ from controls. Plasma HVA levels did not differ from controls in the group of presymptomatic subjects, but were significantly higher in the patients group. This increase was positively associated mainly with severity of illness and functional capacity of the patients, and not with presence of depression or dementia. Plasma HVA levels may be proven to be a peripheral index of disease progression. Reducing dopaminergic activity may have not only symptomatic, but also neuroprotective effects in HD.

  5. Effect of asymmetrical street canyons on pedestrian thermal comfort in warm-humid climate of Cuba

    Science.gov (United States)

    Rodríguez-Algeciras, José; Tablada, Abel; Matzarakis, Andreas

    2017-07-01

    Walkability and livability in cities can be enhanced by creating comfortable environments in the streets. The profile of an urban street canyon has a substantial impact on outdoor thermal conditions at pedestrian level. This paper deals with the effect of asymmetrical street canyon profiles, common in the historical centre of Camagüey, Cuba, on outdoor thermal comfort. Temporal-spatial analyses are conducted using the Heliodon2 and the RayMan model, which enable the generation of accurate predictions about solar radiation and thermal conditions of urban spaces, respectively. On these models, urban settings are represented by asymmetrical street canyons with five different height-to-width ratios and four street axis orientations (N-S, NE-SW, E-W, SE-NW). Results are evaluated for daytime hours across the street canyon, by means of the physiologically equivalent temperature (PET index) which allows the evaluation of the bioclimatic conditions of outdoor environments. Our findings revealed that high profiles (façades) located on the east-facing side of N-S streets, on the southeast-facing side of NE-SW streets, on the south-facing side of E-W street, and on the southwest-facing side of SE-NW streets, are recommended to reduce the total number of hours under thermal stress. E-W street canyons are the most thermally stressed ones, with extreme PET values around 36 °C. Deviating from this orientation ameliorates the heat stress with reductions of up to 4 h in summer. For all analysed E-W orientations, only about one fifth of the street can be comfortable, especially for high aspect ratios (H/W > 3). Optimal subzones in the street are next to the north side of the E-W street, northwest side of the NE-SW street, and southwest side of the SE-NW street. Besides, when the highest profile is located on the east side of N-S streets, then the subzone next to the east-facing façade is recommendable for pedestrians. The proposed urban guidelines enable urban planners to create

  6. Identification of elevated urea as a severe, ubiquitous metabolic defect in the brain of patients with Huntington's disease.

    Science.gov (United States)

    Patassini, Stefano; Begley, Paul; Reid, Suzanne J; Xu, Jingshu; Church, Stephanie J; Curtis, Maurice; Dragunow, Mike; Waldvogel, Henry J; Unwin, Richard D; Snell, Russell G; Faull, Richard L M; Cooper, Garth J S

    Huntington's disease (HD) is a neurodegenerative disorder wherein the aetiological defect is a mutation in the Huntington's gene (HTT), which alters the structure of the huntingtin protein through the lengthening of a polyglutamine tract and initiates a cascade that ultimately leads to dementia and premature death. However, neurodegeneration typically manifests in HD only in middle age, and processes linking the causative mutation to brain disease are poorly understood. Here, our objective was to elucidate further the processes that cause neurodegeneration in HD, by measuring levels of metabolites in brain regions known to undergo varying degrees of damage. We applied gas-chromatography/mass spectrometry-based metabolomics in a case-control study of eleven brain regions in short post-mortem-delay human tissue from nine well-characterized HD patients and nine controls. Unexpectedly, a single major abnormality was evident in all eleven brain regions studied across the forebrain, midbrain and hindbrain, namely marked elevation of urea, a metabolite formed in the urea cycle by arginase-mediated cleavage of arginine. Urea cycle activity localizes primarily in the liver, where it functions to incorporate protein-derived amine-nitrogen into urea for recycling or urinary excretion. It also occurs in other cell-types, but systemic over-production of urea is not known in HD. These findings are consistent with impaired local urea regulation in brain, by up-regulation of synthesis and/or defective clearance. We hypothesize that defective brain urea metabolism could play a substantive role in the pathogenesis of neurodegeneration, perhaps via defects in osmoregulation or nitrogen metabolism. Brain urea metabolism is therefore a target for generating novel monitoring/imaging strategies and/or therapeutic interventions aimed at ameliorating the impact of HD in patients. Copyright © 2015 Elsevier Inc. All rights reserved.

  7. Stochastic backscatter modelling for the prediction of pollutant removal from an urban street canyon: A large-eddy simulation

    Science.gov (United States)

    O'Neill, J. J.; Cai, X.-M.; Kinnersley, R.

    2016-10-01

    The large-eddy simulation (LES) approach has recently exhibited its appealing capability of capturing turbulent processes inside street canyons and the urban boundary layer aloft, and its potential for deriving the bulk parameters adopted in low-cost operational urban dispersion models. However, the thin roof-level shear layer may be under-resolved in most LES set-ups and thus sophisticated subgrid-scale (SGS) parameterisations may be required. In this paper, we consider the important case of pollutant removal from an urban street canyon of unit aspect ratio (i.e. building height equal to street width) with the external flow perpendicular to the street. We show that by employing a stochastic SGS model that explicitly accounts for backscatter (energy transfer from unresolved to resolved scales), the pollutant removal process is better simulated compared with the use of a simpler (fully dissipative) but widely-used SGS model. The backscatter induces additional mixing within the shear layer which acts to increase the rate of pollutant removal from the street canyon, giving better agreement with a recent wind-tunnel experiment. The exchange velocity, an important parameter in many operational models that determines the mass transfer between the urban canopy and the external flow, is predicted to be around 15% larger with the backscatter SGS model; consequently, the steady-state mean pollutant concentration within the street canyon is around 15% lower. A database of exchange velocities for various other urban configurations could be generated and used as improved input for operational street canyon models.

  8. A 24-Hour Study of the Hypothalamo-Pituitary Axes in Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Eirini Kalliolia

    Full Text Available Huntington's disease is an inherited neurodegenerative disorder characterised by motor, cognitive and psychiatric disturbances. Patients exhibit other symptoms including sleep and mood disturbances, muscle atrophy and weight loss which may be linked to hypothalamic pathology and dysfunction of hypothalamo-pituitary axes.We studied neuroendocrine profiles of corticotropic, somatotropic and gonadotropic hypothalamo-pituitary axes hormones over a 24-hour period in controlled environment in 15 healthy controls, 14 premanifest and 13 stage II/III Huntington's disease subjects. We also quantified fasting levels of vasopressin, oestradiol, testosterone, dehydroepiandrosterone sulphate, thyroid stimulating hormone, free triiodothyronine, free total thyroxine, prolactin, adrenaline and noradrenaline. Somatotropic axis hormones, growth hormone releasing hormone, insulin-like growth factor-1 and insulin-like factor binding protein-3 were quantified at 06:00 (fasting, 15:00 and 23:00. A battery of clinical tests, including neurological rating and function scales were performed.24-hour concentrations of adrenocorticotropic hormone, cortisol, luteinizing hormone and follicle-stimulating hormone did not differ significantly between the Huntington's disease group and controls. Daytime growth hormone secretion was similar in control and Huntington's disease subjects. Stage II/III Huntington's disease subjects had lower concentration of post-sleep growth hormone pulse and higher insulin-like growth factor-1:growth hormone ratio which did not reach significance. In Huntington's disease subjects, baseline levels of hypothalamo-pituitary axis hormones measured did not significantly differ from those of healthy controls.The relatively small subject group means that the study may not detect subtle perturbations in hormone concentrations. A targeted study of the somatotropic axis in larger cohorts may be warranted. However, the lack of significant results despite many

  9. Music therapy in Huntington's disease: a protocol for a multi-center randomized controlled trial.

    Science.gov (United States)

    van Bruggen-Rufi, Monique; Vink, Annemieke; Achterberg, Wilco; Roos, Raymund

    2016-07-26

    Huntington's disease is a progressive, neurodegenerative disease with autosomal dominant inheritance, characterized by motor disturbances, cognitive decline and behavioral and psychological symptoms. Since there is no cure, all treatment is aimed at improving quality of life. Music therapy is a non-pharmacological intervention, aiming to improve the quality of life, but its use and efficacy in patients with Huntington's disease has hardly been studied. In this article, a protocol is described to study the effects of music therapy in comparison with a control intervention to improve quality of life through stimulating expressive and communicative skills. By targeting these skills we assume that the social-cognitive functioning will improve, leading to a reduction in behavioral problems, resulting in an overall improvement of the quality of life in patients with Huntington's disease. The study is designed as a multi-center single-blind randomised controlled intervention trial. Sixty patients will be randomised using centre-stratified block-permuted randomisation. Patients will be recruited from four long-term care facilities specialized in Huntington's disease-care in The Netherlands. The outcome measure to assess changes in expressive and communication skills is the Behaviour Observation Scale Huntington and changes in behavior will be assessed by the Problem Behaviour Assesment-short version and by the BOSH. Measurements take place at baseline, then 8, 16 (end of intervention) and 12 weeks after the last intervention (follow-up). This randomized controlled study will provide greater insight into the effectiveness of music therapy on activities of daily living, social-cognitive functioning and behavior problems by improving expressive and communication skills, thus leading to a better quality of life for patients with Huntington's disease. Netherlands Trial Register: NTR4904 , registration date Nov. 15, 2014.

  10. Characterisation of aggression in Huntington's disease: rates, types and antecedents in an inpatient rehabilitation setting.

    Science.gov (United States)

    Brown, Anahita; Sewell, Katherine; Fisher, Caroline A

    2017-10-01

    To systematically review aggression in an inpatient Huntington's cohort examining rates, types and antecedents. Although the prevalence of aggression in Huntington's disease is high, research into this problematic behaviour has been limited. Few studies have investigated the nature of aggressive behaviour in Huntington's disease or antecedents that contribute to its occurrence. A systematic, double-coded, electronic medical file audit. The electronic hospital medical records of 10 people with Huntington's disease admitted to a brain disorders unit were audited for a 90-day period using the Overt Aggression Scale-Modified for Neurorehabilitation framework, yielding 900 days of clinical data. Nine of 10 clients exhibited aggression during the audit period. Both verbal (37·1%) aggression and physical aggression were common (33·8%), along with episodes of mixed verbal and physical aggression (15·2%), while aggression to objects/furniture was less prevalent (5·5%). The most common antecedent was physical guidance with personal care, far exceeding any other documented antecedents, and acting as the most common trigger for four of the nine clients who exhibited aggression. For the remaining five clients, there was intraindividual heterogeneity in susceptibility to specific antecedents. In Huntington's sufferers at mid- to late stages following disease onset, particular care should be made with personal care assistance due to the propensity for these procedures to elicit an episode of aggression. However, given the degree of intraindividual heterogeneity in susceptibility to specific antecedents observed in the present study, individualised behaviour support plans and sensory modulation interventions may be the most useful in identifying triggers and managing aggressive episodes. Rates of aggression in Huntington's disease inpatients can be high. Knowledge of potential triggers, such as personal care, is important for nursing and care staff, so that attempts can be

  11. Davis Canyon noise analysis: Revision 2

    International Nuclear Information System (INIS)

    1985-11-01

    A study was performed as part of the Civilian Radioactive Waste Management Program to quantify the level and effect of noise from the various major phases of development of the proposed potentially acceptable nuclear waste repository site at Davis Canyon, Utah. This report contains the results of a predictive noise level study for the site characterization, repository construction, and repository operational phases. Included herein are graphic representations of energy averaged sound levels, and of audibility levels representing impact zones expected during each phase. Sound levels from onsite and offsite activity including traffic on highways and railroad routes are presented in isopleth maps. A description of the Environmental Noise Prediction Model used for the study, the study basis and methodologies, and actual modeling data are provided. Noise and vibration levels from blasting are also predicted and evaluated. Protective noise criteria containing a margin of safety are used in relation to residences, schools, churches, noise-sensitive recreation areas, and noise-sensitive biological resources. Protective ground motion criteria for ruins and delicate rock formation in Canyonlands National Park and for human annoyance are used in the evaluation of blasting. The evaluations provide the basis for assessing the noise impacts from the related activities at the proposed repository. 45 refs., 21 figs., 15 tabs

  12. Geology of the Nine Canyon Map Area

    International Nuclear Information System (INIS)

    Jones, M.G.; Landon, R.D.

    1978-09-01

    The basalt stratigraphy and structure of a 175-square kilometer area (the Nine Canyon Map Area) along the southern margin of the Pasco Basin have been studied to help assess the feasibility of a nuclear waste terminal storage facility. Detailed mapping shows that uplift of the Horse Heaven Hills began prior to extrusion of the Priest Rapids Member of the Wanapum Basalt, Columbia River Basalt Group. Both the Pomoma and the Elephant Mountain members (Saddle Mountains Basalt, Columbia River Basalt Group) are wide-spread throughout the basin, but thin considerably along the Horse Heaven Hills in the vicinity of Wallula Gap. The Ice Harbor Member is present only along the northern margin of the map area and possibly occupies a paleo-channel. The Rattlesnake Hills-Wallula Gap Lineament trends north 60 degrees west and intersects the older Horse Heaven Hills anticline in Wallula Gap. Four faults of short length and small vertical displacement are located along this structure. Within the map area, the intensity of folding increases, and the style of faulting changes from normal to reverse with proximity to the Wallula Gap area. No evidence for Quaternary deformation was found

  13. Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease

    DEFF Research Database (Denmark)

    Aziz, N A; Jurgens, C K; Landwehrmeyer, G B

    2009-01-01

    OBJECTIVE: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in the HD gene (HTT). We aimed to assess whether interaction between CAG repeat sizes in the mutant and normal allele could affect disease severity and progression. METHODS: Using...... with less severe symptoms and pathology. CONCLUSIONS: Increasing CAG repeat size in normal HTT diminishes the association between mutant CAG repeat size and disease severity and progression in Huntington disease. The underlying mechanism may involve interaction of the polyglutamine domains of normal...

  14. Mental Symptoms in Huntington's Disease and a Possible Primary Aminergic Neuron Lesion

    Science.gov (United States)

    Mann, J. John; Stanley, Michael; Gershon, Samuel; Rossor, M.

    1980-12-01

    Monoamine oxidase activity was higher in the cerebral cortex and basal ganglia of patients dying from Huntington's disease than in controls. Enzyme kinetics and multiple substrate studies indicated that the increased activity was due to elevated concentrations of monoamine oxidase type B. Concentrations of homovanillic acid were increased in the cerebral cortex but not in the basal ganglia of brains of patients with Huntington's disease. These changes may represent a primary aminergic lesion that could underlie some of the mental symptoms of this disease.

  15. Yuntaishan Global Geopark VS Grand Canyon World Heritage Site A Contrast of Yuntai/Grand Canyon Physiognomy

    Science.gov (United States)

    Ting, Zhao; Xun, Zhao

    2017-04-01

    Yuntai/Grand Canyon is a result of long-term historical evolution and a rare natural heritage of the world. With its rich heritages of geological physiognomy, systematic geological record, abundant biological fossil combination, long history of structural evolution, they are of contrastive research values worldwide. The Grand Canyon was declared national natural heritage on eleventh January, and in 1979 it was entitled World Natural Heritage Site. Though the two major sites are separated by tremendous seas, they reached agreements in the protection of natural heritages worldwide on account of the shared ideas of society, demonstrating to our children how can we protect the two scenery sites. Keyword:Geopark, Geoheritage, Yuntai Landform, GrandCanyon Mt. Taihang rises from the central part of north China and extends to the west edge of North China Plain. Towering, and with ragged peaks, precarious cliffs, long strips of walls, deep valleys and shaded streams, Mt. Taihang poses impressive sights with its clear water, dense forest and wonderful sceneries. It is indeed the east slope of Qin-Jin Plateau. Indeed things tend to coincide. On the other side of the Pacific Ocean, along the west edge of north America and on the wide and spacious Colorado Plateau, there is a winding and deep valley where there are layers of rocks, extensive sharp cliffs, intercrossing ravines and forests of peaks; it is totally impressive. Both sceneries are known to the world for their beauty. Identical geological conditions and similar history of evolution left two natural sights that resemble each other so much. Geological changes are infinite, and sedimentation works in similar ways on both sights; and the changing ecological environment gives the world two colorful and comparable geological records. Both sights are merely brief periods in the long history of earth development, but they show us how cradles of human proliferation and social civilization had looked. 1,Comparison of two parks

  16. 2013 Pacific Gas and Electric Diablo Canyon Power Plant (DCPP): San Simeon, CA Central Coast

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Diablo Canyon Power Plant (DCPP) LiDAR and Imagery datasets are comprised of three separate LiDAR surveys: Diablo Canyon (2010), Los Osos (2011), and San Simeon...

  17. 2011 Pacific Gas and Electric Diablo Canyon Power Plant (DCPP): Los Osos, CA Central Coast

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Diablo Canyon Power Plant (DCPP) LiDAR and Imagery datasets are comprised of three separate LiDAR surveys: Diablo Canyon (2010), Los Osos (2011), and San Simeon...

  18. Predictors of Workplace Disability in a Premanifest Huntington's Disease Cohort.

    Science.gov (United States)

    Goh, Anita M Y; You, Emily; Perin, Stephanie; Clay, Fiona J; Loi, Samantha; Ellis, Kathryn; Chong, Terence; Ames, David; Lautenschlager, Nicola

    2018-01-01

    Huntington's disease (HD) is an inherited neurodegenerative disease involving motor, cognitive, and psychiatric/behavioral impairments that will eventually affect work role functioning. Few objective data exist regarding predictors of workplace disability in HD. The authors explored the predictors of work impairment and disability in a cross-sectional cohort of 656 employed, premanifest HD (preHD) individuals. In this cohort-the majority of whom were female, urban-dwelling, married/partnered, and working full-time, with minimal cognitive impairment, good function, minimal motor abnormality, and no indication of significant mental health issues-the number of participants who reported that they had missed work due to HD was low (2.4%). However, 12% of the study sample reported experiencing impairment while working due to preHD, 12.2% reported work-related activity impairment due to preHD, and 12.7% reported impairment in their overall work ability. Higher numbers of CAG repeats on the mutant allele and having more motor symptoms were associated with significantly higher odds of experiencing workplace impairment. Importantly, several modifiable factors were also found to predict workplace disability. Specifically, higher levels of anxiety symptoms were associated with significantly higher odds of experiencing workplace impairment. Good mental and physical health served as protective factors, where good physical health was associated with 6% lower odds of experiencing impairment or missing work time and good mental health was associated with of 10%-12% lower. The results provide important new knowledge for the development of future targeted intervention trials to support preHD individuals in maintaining their work roles as long as possible.

  19. Patterns of False Memory in Patients with Huntington's Disease.

    Science.gov (United States)

    Chen, I-Wen; Chen, Chiung-Mei; Wu, Yih-Ru; Hua, Mau-Sun

    2017-06-01

    Increased false memory recognition in patients with Huntington's disease (HD) has been widely reported; however, the underlying memory constructive processes remain unclear. The present study explored gist memory, item-specific memory, and monitoring ability in patients with HD. Twenty-five patients (including 13 patients with mild HD and 12 patients with moderate-to-severe HD) and 30 healthy comparison participants (HC) were recruited. We used the Deese-Roediger-McDermott (DRM) paradigm to investigate participants' false recognition patterns, along with neuropsychological tests to assess general cognitive function. Both mild and moderate-to-severe patients with HD showed significant executive functioning and episodic memory impairment. On the DRM tasks, both HD patient groups showed significantly impaired performance in tasks assessing unrelated false recognition and item-specific memory as compared to the HC group; moderate-to-severe patients performed more poorly than mild patients did. Only moderate-severe patients exhibited significantly poorer related false recognition index scores than HCs in the verbal DRM task; performance of HD patient groups was comparable to the HC group on the pictorial DRM task. It appears that diminished verbatim memory and monitoring ability are early signs of cognitive decline during the HD course. Conversely, gist memory is relatively robust, with only partial decline during advanced-stage HD. Our findings suggest that medial temporal lobe function is relatively preserved compared to that of frontal-related structures in early HD. Thus, gist-based memory rehabilitation programs might be beneficial for patients with HD. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  20. An electrophysiological analysis of altered cognitive functions in Huntington disease.

    Science.gov (United States)

    Münte, T F; Ridao-Alonso, M E; Preinfalk, J; Jung, A; Wieringa, B M; Matzke, M; Dengler, R; Johannes, S

    1997-09-01

    Neuropsychological deficits are a main feature of Huntington disease (HD) with previous data suggesting involvement of memory functions and visual processing. To increase the knowledge about cognitive malfunction in HD in the domains of visual processing and memory by the use of modern electrophysiological techniques (event-related potentials [ERPs]). A case-control design was used. Three ERP paradigms were used; a parallel visual search paradigm allowed for the simultaneous processing of a multi-element visual array in search of a target stimulus, while a serial search paradigm with varied numbers of distractor items necessitated a serial one by one scanning of the arrays. The third experiment was a word-recognition memory task. The measurements were obtained in a neurophysiological laboratory of a university hospital. Nine patients with HD and 9 control subjects matched for age, sex, and education were studied. Components of averaged ERPs were quantified by latency and amplitude measures and subjected to statistical analysis. Behavioral measures (search time, hit rate, and recognition accuracy) were assessed as well. The early visual components showed a significant latency shift (delay of about 50 milliseconds) in HD. In the search paradigms the P3 components differentiating target and standard stimuli were virtually absent in HD as was the ERP effect indexing word recognition. This was accompanied by a marked delay in search times and lower hit rates in the search tasks and a grossly reduced recognition accuracy in the memory task. The results suggest marked impairments of patients with HD in early visual sensory processing (early components). Deficits in visual search might be attributed to an impairment to deploy attentional resources across the visual field and/or an inability to control eye movements. The ERPs in the memory task differed grossly from similar data obtained by others in patients with Alzheimer disease, suggesting a different neural basis for

  1. Identification of extreme motor phenotypes in Huntington's disease.

    Science.gov (United States)

    Braisch, Ulrike; Hay, Birgit; Muche, Rainer; Rothenbacher, Dietrich; Landwehrmeyer, G Bernhard; Long, Jeffrey D; Orth, Michael

    2017-04-01

    The manifestation of motor signs in Huntington's disease (HD) has a well-known inverse relationship with HTT CAG repeat length, but the prediction is far from perfect. The probability of finding disease modifiers is enhanced in individuals with extreme HD phenotypes. We aimed to identify extreme HD motor phenotypes conditional on CAG and age, such as patients with very early or very late onset of motor manifestation. Retrospective data were available from 1,218 healthy controls and 9,743 HD participants with CAG repeats ≥40, and a total of about 30,000 visits. Boundaries (2.5% and 97.5% quantiles) for extreme motor phenotypes (UHDRS total motor score (TMS) and motor age-at-onset) were estimated using quantile regression for longitudinal data. More than 15% of HD participants had an extreme TMS phenotype for at least one visit. In contrast, only about 4% of participants were consistent TMS extremes at two or more visits. Data from healthy controls revealed an upper cut-off of 13 for the TMS representing the extreme of motor ratings for a normal aging population. In HD, boundaries of motor age-at-onset based on diagnostic confidence or derived from the TMS data cut-off in controls were similar. In summary, a UHDRS TMS of more than 13 in an individual carrying the HD mutation indicates a high likelihood of motor manifestations of HD irrespective of CAG repeat length or age. The identification of motor phenotype extremes can be useful in the search for disease modifiers, for example, genetic or environmental such as medication. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  2. Drosophila eye color mutants as therapeutic tools for Huntington disease.

    Science.gov (United States)

    Green, Edward W; Campesan, Susanna; Breda, Carlo; Sathyasaikumar, Korrapati V; Muchowski, Paul J; Schwarcz, Robert; Kyriacou, Charalambos P; Giorgini, Flaviano

    2012-01-01

    Huntington disease (HD) is a fatal inherited neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin protein (htt). A pathological hallmark of the disease is the loss of a specific population of striatal neurons, and considerable attention has been paid to the role of the kynurenine pathway (KP) of tryptophan (TRP) degradation in this process. The KP contains three neuroactive metabolites: 3-hydroxykynurenine (3-HK), quinolinic acid (QUIN), and kynurenic acid (KYNA). 3-HK and QUIN are neurotoxic, and are increased in the brains of early stage HD patients, as well as in yeast and mouse models of HD. Conversely, KYNA is neuroprotective and has been shown to be decreased in HD patient brains. We recently used a Drosophila model of HD to measure the neuroprotective effect of genetic and pharmacological inhibition of kynurenine monoxygenase (KMO)-the enzyme catalyzing the formation of 3-HK at a pivotal branch point in the KP. We found that KMO inhibition in Drosophila robustly attenuated neurodegeneration, and that this neuroprotection was correlated with reduced levels of 3-HK relative to KYNA. Importantly, we showed that KP metabolites are causative in this process, as 3-HK and KYNA feeding experiments modulated neurodegeneration. We also found that genetic inhibition of the upstream KP enzyme tryptophan-2,3-dioxygenase (TDO) was neuroprotective in flies. Here, we extend these results by reporting that genetic impairment of KMO or TDO is protective against the eclosion defect in HD model fruit flies. Our results provide further support for the possibility of therapeutic KP interventions in HD.

  3. Brain imaging and cognitive dysfunctions in Huntington's disease

    Science.gov (United States)

    Montoya, Alonso; Price, Bruce H.; Menear, Matthew; Lepage, Martin

    2006-01-01

    Recent decades have seen tremendous growth in our understanding of the cognitive dysfunctions observed in Huntington's disease (HD). Advances in neuroimaging have contributed greatly to this growth. We reviewed the role that structural and functional neuroimaging techniques have played in elucidating the cerebral bases of the cognitive deficits associated with HD. We conducted a computer-based search using PubMed and PsycINFO databases to retrieve studies of patients with HD published between 1965 and December 2004 that reported measures on cognitive tasks and used neuroimaging techniques. Structural neuroimaging has provided important evidence of morphological brain changes in HD. Striatal and cortical atrophy are the most common findings, and they correlate with cognitive deficits in attention, working memory and executive functions. Functional studies have also demonstrated correlations between striatal dysfunction and cognitive performance. Striatal hypoperfusion and decreased glucose utilization correlate with executive dysfunction. Hypometabolism also occurs throughout the cerebral cortex and correlates with performance on recognition memory, language and perceptual tests. Measures of presynaptic and postsynaptic dopamine biochemistry have also correlated with measurements of episodic memory, speed of processing and executive functioning. Aided by the results of numerous neuroimaging studies, it is becoming increasingly clear that cognitive deficits in HD involve abnormal connectivity between the basal ganglia and cortical areas. In the future, neuroimaging techniques may shed the most light on the pathophysiology of HD by defining neurodegenerative disease phenotypes as a valuable tool for knowing when patients become “symptomatic,” having been in a gene-positive presymptomatic state, and as a biomarker in following the disease, thereby providing a prospect for improved patient care. PMID:16496032

  4. Progressive microstructural changes of the occipital cortex in Huntington's disease.

    Science.gov (United States)

    Odish, Omar F F; Reijntjes, Robert H A M; van den Bogaard, Simon J A; Roos, Raymund A C; Leemans, Alexander

    2018-02-28

    In this study we longitudinally investigated the rate of microstructural alterations in the occipital cortex in different stages of Huntington's disease (HD) by applying an automated atlas-based approach to diffusion MRI data. Twenty-two premanifest (preHD), 10 early manifest HD (early HD) and 24 healthy control subjects completed baseline and two year follow-up scans. The preHD group was stratified based on the predicted years to disease onset into a far (preHD-A) and near (preHD-B) to disease onset group. Clinical and behavioral measures were collected per assessment time point. An automated atlas-based DTI analysis approach was used to obtain the mean, axial and radial diffusivities of the occipital cortex. We found that the longitudinal rate of diffusivity change in the superior occipital gyrus (SOG), middle occipital gyrus (MOG), and inferior occipital gyrus (IOG) was significantly higher in early HD compared to both preHD and controls (all p's ≤ 0.005), which can be interpreted as an increased rate of microstructural degeneration. Furthermore, the change rate in the diffusivity of the MOG could significantly discriminate between preHD-B compared to preHD-A and the other groups (all p's ≤ 0.04). Finally, we found an inverse correlation between the Stroop Word Reading task and diffusivities in the SOG and MOG (all p's ≤ 0.01). These findings suggest that measures obtained from the occipital cortex can serve as sensitive longitudinal biomarkers for disease progression in preHD-B and early HD. These could in turn be used to assess potential effects of proposed disease modifying therapies.

  5. Late-onset Huntington's disease: diagnostic and prognostic considerations.

    Science.gov (United States)

    Koutsis, Georgios; Karadima, Georgia; Kladi, Athina; Panas, Marios

    2014-07-01

    To address diagnostic and prognostic issues in patients with late-onset Huntington's disease (HD). We analyzed a cohort of 41 late-onset (≥60 years) HD patients and compared them to 39 late-onset patients referred for HD testing that were negative for the HD-expansion and to 290 usual-onset (20-59 years) HD patients. Disease severity was assessed by the Total Functional Capacity Scale. Late-onset HD comprised 11.5% of our HD cohort. In total, 70.7% of late-onset HD patients had positive family history compared to 15.4% of late-onset expansion-negative patients (p < 0.001). Clinical features at onset or presentation could not usefully distinguish between late-onset expansion-positive and negative patients, excepting hemichorea, which was absent from the HD group (p = 0.024). Chorea was the first clinical feature in 53.7% and a presenting feature in 90.2% of late-onset HD. The mutation hit rate for late-onset patients was 51.3%, lower than in usual-onset patients (p = 0.04). Frequencies of chorea, cognitive impairment and psychiatric manifestations at onset or presentation were not significantly different between late-onset and usual-onset HD patients. Gait unsteadiness however was more common at presentation in late-onset HD (p = 0.007). Late-onset HD patients reached a severe stage of illness on average 2.8 years earlier than usual-onset HD patients (p = 0.046). A positive family history suggestive of HD, although absent in a third of patients, remains a helpful clue in diagnosing late-onset HD. Prognosis of late-onset HD in terms of Total Functional Capacity appears no better and shows a trend of being somewhat less favorable compared to usual-onset HD. Copyright © 2014 Elsevier Ltd. All rights reserved.

  6. On the escape of pollutants from urban street canyons

    Energy Technology Data Exchange (ETDEWEB)

    Baik, J.J.; Kim, J.J. [Kwangju Inst. of Science and Technology (Korea). Dept. of Environmental Science and Engineering

    2002-07-01

    Pollutant transport from urban street canyons is numerically investigated using a two-dimensional flow and dispersion model. The ambient wind blows perpendicular to the street and passive pollutants are released at the street level. Results from the control experiment with a street aspect ratio of 1 show that at the roof level of the street canyon, the vertical turbulent flux of pollutants is upward everywhere and the vertical flux of pollutants by mean flow is upward or downward. The horizontally integrated vertical flux of pollutants by mean flow at the roof level of the street canyon is downward and its magnitude is much smaller than that by turbulent process. These results indicate that pollutants escape from the street canyon mainly by turbulent process and that the net effect of mean flow is to make some escaped pollutants reenter the street canyon. Further experiments with different inflow turbulence intensities, inflow wind speeds, and street aspect ratio confirm the findings from the control experiment. In the case of two isolated buildings, the horizontally integrated vertical flux of pollutants by mean flow is upward due to flow separation but the other main results are the same as those from the control experiment. (author)

  7. Cold-water coral ecosystems in Cassidaigne Canyon: An assessment of their environmental living conditions

    OpenAIRE

    Fabri, Marie-claire; Bargain, Annaelle; Pairaud, Ivane; Pedel, Laura; Taupier-letage, I.

    2017-01-01

    The Cassidaigne canyon is one of the two canyons (together with Lacaze-Duthiers) of the French Mediterranean coast in which cold-water corals have settled and formed large colonies, providing a structural habitat for other species. Nevertheless, the communities settled in the Cassidaigne canyon are physically impacted by discharges of bauxite residues. New information on the distribution of the species Madrepora oculata and the associated species diversity in Cassidaigne canyon was provid...

  8. CODASC : a database for the validation of street canyon dispersion models

    OpenAIRE

    Gromke, C.B.

    2013-01-01

    CODASC stands for Concentration Data of Street Canyons (CODASC 2008, www.codasc.de). It is a database which provides traffic pollutant concentrations in urban street canyons obtained from wind-tunnel dispersion experiments. CODASC comprises concentration data of street canyons with different aspect ratios subjected to various wind directions and also for street canyons with tree-avenues. The database includes concentration data of tree-avenue configurations of different tree arrangement, tree...

  9. JUNCTOPHILIN 3 (JPH3) EXPANSION MUTATIONS CAUSING HUNTINGTON DISEASE LIKE 2 (HDL2) ARE COMMON IN SOUTH AFRICAN PATIENTS WITH AFRICAN ANCESTRY AND A HUNTINGTON DISEASE PHENOTYPE

    Science.gov (United States)

    Krause, A; Mitchell, CL; Essop, F; Tager, S; Temlett, J; Stevanin, G; Ross, CA; Rudnicki, DD; Margolis, RL

    2015-01-01

    Huntington disease (HD) is a progressive autosomal dominant neurodegenerative disorder, characterized by abnormal movements, cognitive decline and psychiatric symptoms, caused by a CAG repeat expansion in the huntingtin (HTT) gene on chromosome 4p. A CAG/CTG repeat expansion in the junctophilin-3 (JPH3) gene on chromosome 16q24.2 causes a Huntington disease-like phenotype (HDL2). All patients to date with HDL2 have some African ancestry. The present study aimed to characterize the genetic basis of the Huntington disease phenotype in South Africans and to investigate the possible origin of the JPH3 mutation. In a sample of unrelated South African individuals referred for diagnostic HD testing, 62% (106/171) of white patients compared to only 36% (47/130) of black patients had an expansion in HTT. However, 15% (20/130) of black South African patients and no white patients (0/171) had an expansion in JPH3, confirming the diagnosis of Huntington disease like 2 (HDL2). Individuals with HDL2 share many clinical features with individuals with HD and are clinically indistinguishable in many cases, although the average age of onset and diagnosis in HDL2 is 5 years later than HD and individual clinical features may be more prominent. HDL2 mutations contribute significantly to the HD phenotype in South Africans with African ancestry. JPH3 haplotype studies in 31 families, mainly from South Africa and North America, provide evidence for a founder mutation and support a common African origin for all HDL2 patients. Molecular testing in individuals with an HD phenotype and African ancestry should include testing routinely for JPH3 mutations. PMID:26079385

  10. Influence of the Nazaré Canyon, central Portuguese margin, on late winter coccolithophore assemblages

    NARCIS (Netherlands)

    Guerreiro, C.; Sá, C.; de Stigter, H.; Oliveira, A.; Cachão, M.; Cros, L.; Borges, C.; Quaresma, L.; Santos, A.I.; Fortuño, J.-M.; Rodrigez, A.

    2014-01-01

    This paper presents a first attempt to characterize coccolithophore assemblages occurring in the context of an active submarine canyon. Coccolithophores from the upper-middle sections of the Nazaré Canyon (central Portuguese margin) – one of the largest canyons of the European continental margin –

  11. The urban canyon and building energy use: Urban density versus daylight and passive solar gains

    DEFF Research Database (Denmark)

    Strømann-Andersen, Jakob Bjørn; Sattrup, Peter Andreas

    2011-01-01

    .It was found that the geometry of urban canyons has an impact on total energy consumption in the range of up to +30% for offices and +19% for housing, which shows that the geometry of urban canyons is a key factor in energy use in buildings. It was demonstrated how the reflectivity of urban canyons plays...

  12. 77 FR 8895 - Jimbilnan, Pinto Valley, Black Canyon, Eldorado, Ireteba Peaks, Nellis Wash, Spirit Mountain, and...

    Science.gov (United States)

    2012-02-15

    ..., Pinto Valley, Black Canyon, Eldorado, Ireteba Peaks, Nellis Wash, Spirit Mountain, and Bridge Canyon..., Eldorado, Ireteba Peaks, Nellis Wash, Spirit Mountain, and Bridge Canyon Wilderness Areas, Lake Mead... wilderness character; providing for reasonable use of Spirit Mountain and adjacent areas in a manner meeting...

  13. CODASC : a database for the validation of street canyon dispersion models

    NARCIS (Netherlands)

    Gromke, C.B.

    2013-01-01

    CODASC stands for Concentration Data of Street Canyons (CODASC 2008, www.codasc.de). It is a database which provides traffic pollutant concentrations in urban street canyons obtained from wind-tunnel dispersion experiments. CODASC comprises concentration data of street canyons with different aspect

  14. Trees in urban street canyons and their impact on the dispersion of automobile exhausts

    NARCIS (Netherlands)

    Gromke, C.B.; Ruck, B.

    2007-01-01

    The aim of the present study is to clarify the influence of trees on the dispersion of automobile exhausts in urban street canyons. For this purpose, measurements have been performed with a small scale wind tunnel model of an idealized, isolated street canyon with model trees placed along the canyon

  15. 33 CFR 165.1171 - Copper Canyon, Lake Havasu, Colorado River-Regulated Navigation Area.

    Science.gov (United States)

    2010-07-01

    ... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Copper Canyon, Lake Havasu... Guard District § 165.1171 Copper Canyon, Lake Havasu, Colorado River—Regulated Navigation Area. (a) Location. The following is a regulated navigation area: (1) In the water area of Copper Canyon, Lake Havasu...

  16. Standardized methods for Grand Canyon fisheries research 2015

    Science.gov (United States)

    Persons, William R.; Ward, David L.; Avery, Luke A.

    2013-01-01

    This document presents protocols and guidelines to persons sampling fishes in the Grand Canyon, to help ensure consistency in fish handling, fish tagging, and data collection among different projects and organizations. Most such research and monitoring projects are conducted under the general umbrella of the Glen Canyon Dam Adaptive Management Program and include studies by the U.S. Geological Survey (USGS), U.S. Fish and Wildlife Service (FWS), National Park Service (NPS), the Arizona Game and Fish Department (AGFD), various universities, and private contractors. This document is intended to provide guidance to fieldworkers regarding protocols that may vary from year to year depending on specific projects and objectives. We also provide herein documentation of standard methods used in the Grand Canyon that can be cited in scientific publications, as well as a summary of changes in protocols since the document was first created in 2002.

  17. Behavioral testing of minipigs transgenic for the Huntington gene-A three-year observational study

    Czech Academy of Sciences Publication Activity Database

    Schuldenzucker, V.; Schubert, R.; Muratori, L. M.; Freisfeld, F.; Rieke, L.; Matheis, T.; Schramke, S.; Motlík, Jan; Kemper, N.; Radespiel, U.; Reilmann, R.

    2017-01-01

    Roč. 12, č. 10 (2017), č. článku e0185970. E-ISSN 1932-6203 Institutional support: RVO:67985904 Keywords : Huntington´s disease * minipigs Subject RIV: EG - Zoology OBOR OECD: Behavioral sciences biology Impact factor: 2.806, year: 2016

  18. Motor, emotional and cognitive deficits in adult BACHD mice : A model for Huntington's disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Schreiber, Rudy; Ellenbroek, Bart

    2013-01-01

    Rationale: Huntington's disease (HD) is characterized by progressive motor dysfunction, emotional disturbances and cognitive deficits. It is a genetic disease caused by an elongation of the polyglutamine repeats in the huntingtin gene. Whereas HD is a complex disorder, previous studies in mice

  19. Vertraagde diagnose van de ziekte van Huntington in een psychiatrische setting

    NARCIS (Netherlands)

    Tak, L M; Sizoo, B; de Stegge, B M Aan; Adema, S; van Duijn, E; Kremer, B

    2017-01-01

    Huntington's disease (hd) is characterised by a triad of neuropsychiatric symptoms, motor disturbances and cognitive decline. If initial symptoms are of neuropsychiatric nature they maybe misinterpreted, which can lead to delayed diagnosis. Three examples of delayed hd diagnosis in a psychiatric

  20. CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion

    DEFF Research Database (Denmark)

    Lee, J-M; Ramos, E M; Lee, J-H

    2012-01-01

    Age at onset of diagnostic motor manifestations in Huntington disease (HD) is strongly correlated with an expanded CAG trinucleotide repeat. The length of the normal CAG repeat allele has been reported also to influence age at onset, in interaction with the expanded allele. Due to profound...

  1. Striatal dopamine D2 receptors, metabolism, and volume in preclinical Huntington disease

    NARCIS (Netherlands)

    van Oostrom, JCH; Maguire, RP; Verschuuren-Bemelmans, CC; van der Duin, LV; Pruim, J; Roos, RAC; Leenders, KL

    2005-01-01

    Among 27 preclinical carriers of the Huntington disease mutation (PMC), the authors found normal striatal values for MRI volumetry in 88% and for fluorodesoxyglucose PET metabolic index in 67%. Raclopride PET binding potential (RAC-BP) was decreased in 50% and correlated with increases in the

  2. Hypocretin and melanin-concentrating hormone in patients with Huntington disease.

    NARCIS (Netherlands)

    Aziz, A.; Fronczek, R.; Maat-Schieman, M.L.; Unmehopa, U.A.; Roelandse, F.W.; Overeem, S.; Duinen, S.G. van; Lammers, G.J.; Swaab, D.F.; Roos, R.A.C.

    2008-01-01

    To evaluate whether hypocretin-1 (orexin-A) and melanin-concentrating hormone (MCH) neurotransmission are affected in patients with Huntington disease (HD), we immunohistochemically stained hypocretin and MCH neurons and estimated their total numbers in the lateral hypothalamus of both HD patients

  3. Functional Compensation of Motor Function in Pre-Symptomatic Huntington's Disease

    Science.gov (United States)

    Kloppel, Stefan; Draganski, Bogdan; Siebner, Hartwig R.; Tabrizi, Sarah J.; Weiller, Cornelius; Frackowiak, Richard S. J.

    2009-01-01

    Involuntary choreiform movements are a clinical hallmark of Huntington's disease. Studies in clinically affected patients suggest a shift of motor activations to parietal cortices in response to progressive neurodegeneration. Here, we studied pre-symptomatic gene carriers to examine the compensatory mechanisms that underlie the phenomenon of…

  4. 4p16.3 haplotype modifying age at onset of Huntington disease

    DEFF Research Database (Denmark)

    Nørremølle, A; Budtz-Jørgensen, E; Fenger, K

    2009-01-01

    Huntington disease (HD) is caused by an expanded CAG repeat sequence in the HD gene. Although the age at onset is correlated to the CAG repeat length, this correlation only explains approximately half of the variation in onset age. Less variation between siblings indicates that the variation is, ...

  5. The use of stem cells in regenerative medicine for Parkinson's and Huntington's Diseases.

    Science.gov (United States)

    Lescaudron, L; Naveilhan, P; Neveu, I

    2012-01-01

    Cell transplantation has been proposed as a means of replacing specific cell populations lost through neurodegenerative processes such as that seen in Parkinson's or Huntington's diseases. Improvement of the clinical symptoms has been observed in a number of Parkinson and Huntington's patients transplanted with freshly isolated fetal brain tissue but such restorative approach is greatly hampered by logistic and ethical concerns relative to the use of fetal tissue, in addition to potential side effects that remain to be controlled. In this context, stem cells that are capable of self-renewal and can differentiate into neurons, have received a great deal of interest, as demonstrated by the numerous studies based on the transplantation of neural stem/progenitor cells, embryonic stem cells or mesenchymal stem cells into animal models of Parkinson's or Huntington's diseases. More recently, the induction of pluripotent stem cells from somatic adult cells has raised a new hope for the treatment of neurodegenerative diseases. In the present article, we review the main experimental approaches to assess the efficiency of cell-based therapy for Parkinson's or Huntington's diseases, and discuss the recent advances in using stem cells to replace lost dopaminergic mesencephalic or striatal neurons. Characteristics of the different stem cells are extensively examined with a special attention to their ability of producing neurotrophic or immunosuppressive factors, as these may provide a favourable environment for brain tissue repair and long-term survival of transplanted cells in the central nervous system. Thus, stem cell therapy can be a valuable tool in regenerative medicine.

  6. Selected CSF biomarkers indicate no evidence of early neuroinflammation in Huntington disease

    DEFF Research Database (Denmark)

    Vinther-Jensen, Tua; Börnsen, Lars Svend; Budtz-Jorgensen, Esben

    2016-01-01

    Objective: To investigate CSF biomarkers of neuroinflammation and neurodegeneration in Huntington disease (HD) gene-expansion carriers compared to controls and to investigate these biomarkers in association with clinical HD rating scales and disease burden score. Methods: We collected CSF from 32...

  7. Beautiful Science: The Public and Private History of Astronomy at the Huntington Library

    Science.gov (United States)

    Lewis, Daniel

    2009-05-01

    The history of astronomy has a long tradition within research libraries. The rare collections at the Huntington Library (encompassing American and British history from around 1000 CE to the present, in many different subject areas) are among the most heavily-used in the United States, The history of astronomy holdings are a cornerstone within the library's history of science holdings. This talk will present the two faces of the history of astronomy holdings at the Huntington Library. The first of these is the research end of operations: what the collections consist of, how the scholarly public uses the collections, and what the implications are for modern astronomical practice. The second element concerns the public exhibit face of the history of astronomy holdings at The Huntington. Of the 600,000 people who visit the Huntington each year, the majority visit public displays and rare book and manuscript exhibits. "Beautiful Science: Ideas That Changed the World” is a new permanent history of science exhibit. One quarter of the exhibit relates to the history of astronomy. Public exhibits require a particular kind of planning and bring a specific set of values to the history of astronomy. Public exhibits also have their own concerns, and this talk will cover a number of those issues as well as the research issues.

  8. 77 FR 22616 - Huntington Asset Advisors, Inc., et al.; Notice of Application

    Science.gov (United States)

    2012-04-16

    ...''), Huntington Strategy Shares (``Trust''), and SEI Investments Distribution Co. Summary of Application.... \\14\\ If Shares are listed on The NASDAQ Stock Market LLC (``Nasdaq'') or a similar electronic Listing... SECURITIES AND EXCHANGE COMMISSION [Investment Company Act Release No. 30032; 812-13785...

  9. 77 FR 51064 - Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application...

    Science.gov (United States)

    2012-08-23

    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-81,475] Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application for Reconsideration By application dated May 21, 2012, the State Workforce Office requested administrative reconsideration of the negative...

  10. The use of olanzapine in Huntington disease accompanied by psychotic symptoms

    Directory of Open Access Journals (Sweden)

    Cafer Alhan

    2014-06-01

    Full Text Available Huntington's disease is an autosomal dominant neurodegenerative disease. The disease begins between the ages of 30-50, including motor symptoms, psychiatric symptoms and is characterized by progressive dementia. Common psychiatric disorders of Huntington’s disease include mood and anxiety disorders, behavior and personality changes. Psychosis is relatively rare. Here, a patient is present, who has Huntington’s disease, which is associated with psychotic symptoms. 61-year-old male patient who were followed for Huntington disease for 25 years was admitted for complaints of thinking of poisoning and refuse to eat something. Patient was started on olanzapine at dose of 5 mg/day. In follow up psychotic symptoms disappeared. Emerging psychotic symptoms in Huntington disease is created a need for antipsychotic treatment. Atypical antipsychotic agents should be preferred in the treatment and as in the case olanzapine may be used as a treatment option should be kept in mind to control both involuntary movements and psychotic symptoms in Huntington's disease with psychotic features. J Clin Exp Invest 2014; 5 (2: 326-328

  11. 3-NP-induced neurodegeneration studies in experimental models of Huntington's disease.

    NARCIS (Netherlands)

    Vis, J.C.

    2005-01-01

    This thesis investigates the possible role of apoptosis, or programmed cell death, in Huntington's disease (HD). HD is caused by an expanded CAG repeat in the N-terminal region of the huntingtin protein leading to specific neostriatal neurodegeneration. The sequence of events that leads to this

  12. Evidence for Deficits on Different Components of Theory of Mind in Huntington's Disease

    NARCIS (Netherlands)

    Allain, P.; Havel-Thomassin, V.; Verny, C.; Gohier, B.; Lancelot, C.; Besnard, J.; Fasotti, L.; Le Gall, D.

    2011-01-01

    Objective: The main aim of this study was to investigate the effects of Huntington's disease (HD) on cognitive and affective Theory of Mind (ToM) abilities. The relation of ToM performance and executive functions was also examined. Method: Eighteen HD patients, early in the course of the disease,

  13. A new mutation for Huntington disease following maternal transmission of an intermediate allele

    NARCIS (Netherlands)

    Semaka, Alicia; Kay, Chris; Belfroid, René D. M.; Bijlsma, Emilia K.; Losekoot, Monique; van Langen, Irene M.; van Maarle, Merel C.; Oosterloo, Mayke; Hayden, Michael R.; van Belzen, Martine J.

    2015-01-01

    New mutations for Huntington disease (HD) originate from CAG repeat expansion of intermediate alleles (27-35 CAG). Expansions of such alleles into the pathological range (≥ 36 CAG) have been exclusively observed in paternal transmission. We report the occurrence of a new mutation that defies the

  14. 75 FR 11939 - Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation

    Science.gov (United States)

    2010-03-12

    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-71,749] Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation Pursuant to Section 221 of the Trade Act of 1974, as amended, an investigation was initiated in response to a petition filed on July 21...

  15. Reversal learning and associative memory impairments in a BACHD rat model for Huntington disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Nguyen, Huu Phuc; Ellenbroek, Bart; Schreiber, Rudy

    2013-01-01

    Chorea and psychiatric symptoms are hallmarks of Huntington disease (HD), a neurodegenerative disorder, genetically characterized by the presence of expanded CAG repeats (>35) in the HUNTINGTIN (HTT) gene. HD patients present psychiatric symptoms prior to the onset of motor symptoms and we recently

  16. Motor cortex synchronization influences the rhythm of motor performance in premanifest huntington's disease.

    Science.gov (United States)

    Casula, Elias P; Mayer, Isabella M S; Desikan, Mahalekshmi; Tabrizi, Sarah J; Rothwell, John C; Orth, Michael

    2018-03-01

    In Huntington's disease there is evidence of structural damage in the motor system, but it is still unclear how to link this to the behavioral disorder of movement. One feature of choreic movement is variable timing and coordination between sequences of actions. We postulate this results from desynchronization of neural activity in cortical motor areas. The objective of this study was to explore the ability to synchronize activity in a motor network using transcranial magnetic stimulation and to relate this to timing of motor performance. We examined synchronization in oscillatory activity of cortical motor areas in response to an external input produced by a pulse of transcranial magnetic stimulation. We combined this with EEG to compare the response of 16 presymptomatic Huntington's disease participants with 16 age-matched healthy volunteers to test whether the strength of synchronization relates to the variability of motor performance at the following 2 tasks: a grip force task and a speeded-tapping task. Phase synchronization in response to M1 stimulation was lower in Huntington's disease than healthy volunteers (P synchronization (r = -0.356; P synchronization and desynchronization could be a physiological basis for some key clinical features of Huntington's disease. © 2018 International Parkinson and Movement Disorder Society. © 2018 International Parkinson and Movement Disorder Society.

  17. BLANCO MOUNTAIN AND BLACK CANYON ROADLESS AREAS, CALIFORNIA.

    Science.gov (United States)

    Diggles, Michael F.; Rains, Richard L.

    1984-01-01

    The mineral survey of the Blanco Mountain and Black Canyon Roadless Areas, California indicated that areas of probable and substantiated mineral-resource potential exist only in the Black Canyon Roadless Area. Gold with moderate amounts of lead, silver, zinc, and tungsten, occurs in vein deposits and in tactite. The nature of the geological terrain indicates little likelihood for the occurrence of energy resources in the roadless areas. Detailed geologic mapping might better define the extent of gold mineralization. Detailed stream-sediment sampling and analysis of heavy-mineral concentrations could better define tungsten resource potential.

  18. Are amphitheater headed canyons indicative of a particular formative process?

    Science.gov (United States)

    Ryan, A. J.; Whipple, K. X.; Johnson, J. P.

    2012-12-01

    Tributary canyons with amphitheater-shaped heads have previously been interpreted as evidence for groundwater seepage erosion, particularly in environments where fluvial processes are assumed to be negligible. However, some have questioned whether this canyon morphology is truly diagnostic of a particular formative process. We seek to determine the relative roles of fluvial and groundwater-related processes and the strength of stratigraphic control on the Colorado Plateau through a combination of fieldwork and GIS analysis. Amphitheater valleys may have overhanging or steep-sided headwalls with a semicircular plan-view pattern. It is reasonable to assume that this form is a result of focused erosion at the base of the headwall (i.e. sapping). Two frequently cited agents may lead to undermining: plunge-pool scour at the base of waterfalls and seepage induced weathering and erosion where the groundwater table intersects the land surface. Both processes are enhanced where weaker, less permeable layers underlie stronger cap rock. We conducted preliminary fieldwork in two locations on the Colorado Plateau, where there are many classic examples of amphitheater headed canyons. The Escalante River landscape is highly variable with a range of canyon and valley-head forms, many of which cut through the thick Navajo Sandstone into the underlying shale and sand of the Kayenta Formation. Northeast of Escalante National Monument, at the base of the Henry Mountains, is Tarantula Mesa. The canyons there are also considerably variable, with nearly all containing at least one abrupt amphitheater knickpoint at the valley head or farther downstream. Our observations are presented here with an analysis of the canyon profiles, surrounding topography, and potential structural controls. We have found that nearly all amphitheaters in both locales show signs of groundwater seepage weathering and plausibly seepage erosion. However, many also contain plunge pools and evidence of substantial

  19. Review of the Diablo Canyon probabilistic risk assessment

    International Nuclear Information System (INIS)

    Bozoki, G.E.; Fitzpatrick, R.G.; Bohn, M.P.; Sabek, M.G.; Ravindra, M.K.; Johnson, J.J.

    1994-08-01

    This report details the review of the Diablo Canyon Probabilistic Risk Assessment (DCPRA). The study was performed under contract from the Probabilistic Risk Analysis Branch, Office of Nuclear Reactor Research, USNRC by Brookhaven National Laboratory. The DCPRA is a full scope Level I effort and although the review touched on all aspects of the PRA, the internal events and seismic events received the vast majority of the review effort. The report includes a number of independent systems analyses sensitivity studies, importance analyses as well as conclusions on the adequacy of the DCPRA for use in the Diablo Canyon Long Term Seismic Program

  20. VCE-003.2, a novel cannabigerol derivative, enhances neuronal progenitor cell survival and alleviates symptomatology in murine models of Huntington's disease.

    Science.gov (United States)

    Díaz-Alonso, Javier; Paraíso-Luna, Juan; Navarrete, Carmen; Del Río, Carmen; Cantarero, Irene; Palomares, Belén; Aguareles, José; Fernández-Ruiz, Javier; Bellido, María Luz; Pollastro, Federica; Appendino, Giovanni; Calzado, Marco A; Galve-Roperh, Ismael; Muñoz, Eduardo

    2016-07-19

    Cannabinoids have shown to exert neuroprotective actions in animal models by acting at different targets including canonical cannabinoid receptors and PPARγ. We previously showed that VCE-003, a cannabigerol (CBG) quinone derivative, is a novel neuroprotective and anti-inflammatory cannabinoid acting through PPARγ. We have now generated a non-thiophilic VCE-003 derivative named VCE-003.2 that preserves the ability to activate PPARγ and analyzed its neuroprotective activity. This compound exerted a prosurvival action in progenitor cells during neuronal differentiation, which was prevented by a PPARγ antagonist, without affecting neural progenitor cell proliferation. In addition, VCE-003.2 attenuated quinolinic acid (QA)-induced cell death and caspase-3 activation and also reduced mutant huntingtin aggregates in striatal cells. The neuroprotective profile of VCE-003.2 was analyzed using in vivo models of striatal neurodegeneration induced by QA and 3-nitropropionic acid (3NP) administration. VCE-003.2 prevented medium spiny DARPP32(+) neuronal loss in these Huntington's-like disease mice models improving motor deficits, reactive astrogliosis and microglial activation. In the 3NP model VCE-003.2 inhibited the upregulation of proinflammatory markers and improved antioxidant defenses in the brain. These data lead us to consider VCE-003.2 to have high potential for the treatment of Huntington's disease (HD) and other neurodegenerative diseases with neuroinflammatory traits.

  1. An image-based model of brain volume biomarker changes in Huntington's disease.

    Science.gov (United States)

    Wijeratne, Peter A; Young, Alexandra L; Oxtoby, Neil P; Marinescu, Razvan V; Firth, Nicholas C; Johnson, Eileanoir B; Mohan, Amrita; Sampaio, Cristina; Scahill, Rachael I; Tabrizi, Sarah J; Alexander, Daniel C

    2018-05-01

    Determining the sequence in which Huntington's disease biomarkers become abnormal can provide important insights into the disease progression and a quantitative tool for patient stratification. Here, we construct and present a uniquely fine-grained model of temporal progression of Huntington's disease from premanifest through to manifest stages. We employ a probabilistic event-based model to determine the sequence of appearance of atrophy in brain volumes, learned from structural MRI in the Track-HD study, as well as to estimate the uncertainty in the ordering. We use longitudinal and phenotypic data to demonstrate the utility of the patient staging system that the resulting model provides. The model recovers the following order of detectable changes in brain region volumes: putamen, caudate, pallidum, insula white matter, nonventricular cerebrospinal fluid, amygdala, optic chiasm, third ventricle, posterior insula, and basal forebrain. This ordering is mostly preserved even under cross-validation of the uncertainty in the event sequence. Longitudinal analysis performed using 6 years of follow-up data from baseline confirms efficacy of the model, as subjects consistently move to later stages with time, and significant correlations are observed between the estimated stages and nonimaging phenotypic markers. We used a data-driven method to provide new insight into Huntington's disease progression as well as new power to stage and predict conversion. Our results highlight the potential of disease progression models, such as the event-based model, to provide new insight into Huntington's disease progression and to support fine-grained patient stratification for future precision medicine in Huntington's disease.

  2. Dantrolene is neuroprotective in Huntington's disease transgenic mouse model

    Directory of Open Access Journals (Sweden)

    Chen Xi

    2011-11-01

    Full Text Available Abstract Background Huntington's disease (HD is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs. Our group has previously demonstrated that calcium (Ca2+ signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128. Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2 and spinocerebellar ataxia 3 (SCA3 mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. Results The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Conclusions Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that Ryan

  3. Dantrolene is neuroprotective in Huntington's disease transgenic mouse model.

    Science.gov (United States)

    Chen, Xi; Wu, Jun; Lvovskaya, Svetlana; Herndon, Emily; Supnet, Charlene; Bezprozvanny, Ilya

    2011-11-25

    Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs). Our group has previously demonstrated that calcium (Ca2+) signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128). Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT) MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2) and spinocerebellar ataxia 3 (SCA3) mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg) twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that RyanR inhibitors and Ca2+ signaling stabilizers such as

  4. A Wind Tunnel Investigation of the Influence of Solar-Induced Wall-Heating on the Flow Regime within a Simulated Urban Street Canyon

    International Nuclear Information System (INIS)

    Kovar-Panskus, A.; Moulinneuf, L.; Savory, E.; Abdelqari, A.; Sini, J.-F.; Rosant, J.-M.; Robins, A.; Toy, N.

    2002-01-01

    A wind tunnel study has been undertaken to assess the influence of solar-induced wall heating on the airflow pattern within a street canyon under low-speed wind conditions. This flow is normally dominated by large-scale vortical motion, such that the wind moves downwards at the downstream wall. In the present work the aim has been to examine whether the buoyancy forces generated at this wall by solar-induced heating are of sufficient strength to oppose the downward inertial forces and, thereby, change the canyon flow pattern. Such changes will also influence the dispersion of pollutants within the street. In the experiments the windward-facing wall of a canyon has been uniformly heated to simulate the effect of solar radiation.Four different test cases, representing different degrees of buoyancy (defined by a test Froude number, Fr), have been examined using a simple, 2-D, square-section canyon model in a wind tunnel. For reference purposes, the neutral case (no wall heating), has also been studied. The approach flow boundary layer conditions have been well defined, with the wind normal to the main canyon axis, and measurements have been taken of canyon wall and air temperatures and profiles of mean velocities and turbulence intensities.Analysis of the results shows clear differences in the flow patterns. As Fr decreases from the neutral case there are reductions of up to 50% in the magnitudes of the reverseflow velocities near the ground and in the upward motion near the upstream wall. A marked transition occurs at Fr ∼ 1, where the single dominant vortex, existing at higher Fr values, weakens and moves upwards whilst a lower region of relatively stagnant flow appears. This transition had previously been observed in numerical model predictions but at a Fr at least an order of magnitude higher

  5. Passive air exchanges between building and urban canyon via openings in a single facade

    International Nuclear Information System (INIS)

    Syrios, K.; Hunt, G.R.

    2008-01-01

    The results of an experimental study examining the steady exchange of air and heat between a building and an urban canyon are presented. The focus is on the effect of the canyon aspect ratio on the airflow through openings made exclusively in one side of the building. The interaction of the external wind flow and the internal thermally-driven flow was shown to depend upon the ratio of the building height H b to the canyon width W (distance between buildings forming the canyons). The trends observed as this aspect ratio (H b /W) was varied allow for identification of canyon geometries that yield reduced or enhanced building ventilation airflow rates

  6. Submarine canyons as coral and sponge habitat on the eastern Bering Sea slope

    Directory of Open Access Journals (Sweden)

    Robert J. Miller

    2015-07-01

    Full Text Available Submarine canyons have been shown to positively influence pelagic and benthic biodiversity and ecosystem function. In the eastern Bering Sea, several immense canyons lie under the highly productive “green belt” along the continental slope. Two of these, Pribilof and Zhemchug canyons, are the focus of current conservation interest. We used a maximum entropy modeling approach to evaluate the importance of these two canyons, as well as canyons in general, as habitat for gorgonian (alcyonacean corals, pennatulacean corals, and sponges, in an area comprising most of the eastern Bering Sea slope and outer shelf. These invertebrates create physical structure that is a preferred habitat for many mobile species, including commercially important fish and invertebrates. We show that Pribilof canyon is a hotspot of structure-forming invertebrate habitat, containing over 50% of estimated high-quality gorgonian habitat and 45% of sponge habitat, despite making up only 1.7% of the total study area. The amount of quality habitat for gorgonians and sponges varied in other canyons, but canyons overall contained more high-quality habitat for structure-forming invertebrates compared to other slope areas. Bottom trawling effort was not well correlated with habitat quality for structure-forming invertebrates, and bottom-contact fishing effort in general, including longlining and trawling, was not particularly concentrated in the canyons examined. These results suggest that if conserving gorgonian coral habitat is a management goal, canyons, particularly Pribilof Canyon, may be a prime location to do this without excessive impact on fisheries.

  7. Striatal pre-enkephalin overexpression improves Huntington's disease symptoms in the R6/2 mouse model of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    Stéphanie Bissonnette

    Full Text Available The reduction of pre-enkephalin (pENK mRNA expression might be an early sign of striatal neuronal dysfunction in Huntington's disease (HD, due to mutated huntingtin protein. Indeed, striatopallidal (pENK-containing neurodegeneration occurs at earlier stage of the disease, compare to the loss of striatonigral neurons. However, no data are available about the functional role of striatal pENK in HD. According to the neuroprotective properties of opioids that have been recognized recently, the objective of this study was to investigate whether striatal overexpression of pENK at early stage of HD can improve motor dysfunction, and/or reduce striatal neuronal loss in the R6/2 transgenic mouse model of HD. To achieve this goal recombinant adeno-associated-virus (rAAV2-containing green fluorescence protein (GFP-pENK was injected bilaterally in the striatum of R6/2 mice at 5 weeks old to overexpress opioid peptide pENK. Striatal injection of rAAV2-GFP was used as a control. Different behavioral tests were carried out before and/or after striatal injections of rAAV2. The animals were euthanized at 10 weeks old. Our results demonstrate that striatal overexpression of pENK had beneficial effects on behavioral symptoms of HD in R6/2 by: delaying the onset of decline in muscular force; reduction of clasping; improvement of fast motor activity, short-term memory and recognition; as well as normalization of anxiety-like behavior. The improvement of behavioral dysfunction in R6/2 mice having received rAAV2-GFP-pENK associated with upregulation of striatal pENK mRNA; the increased level of enkephalin peptide in the striatum, globus pallidus and substantia nigra; as well as the slight increase in the number of striatal neurons compared with other groups of R6/2. Accordingly, we suggest that at early stage of HD upregulation of striatal enkephalin might play a key role at attenuating illness symptoms.

  8. Carbonaceous aerosol particles from common vegetation in the Grand Canyon

    International Nuclear Information System (INIS)

    Hallock, K.A.; Mazurek, M.A.; Cass, G.R.

    1992-05-01

    The problem of visibility reduction in the Grand Canyon due to fine organic aerosol particles in the atmosphere has become an area of increased environmental concern. Aerosol particles can be derived from many emission sources. In this report, we focus on identifying organic aerosols derived from common vegetation in the Grand Canyon. These aerosols are expected to be significant contributors to the total atmospheric organic aerosol content. Aerosol samples from living vegetation were collected by resuspension of surface wax and resin components liberated from the leaves of vegetation common to areas of the Grand Canyon. The samples were analyzed using high-resolution gas chromatography/mass spectrometry (GC/MS). Probable identification of compounds was made by comparison of sample spectra with National Institute of Standards and Technology (NIST) mass spectral references and positive identification of compounds was made when possible by comparison with authentic standards as well as NIST references. Using these references, we have been able to positively identify the presence of n-alkane and n-alkanoic acid homolog series in the surface waxes of the vegetation sampled. Several monoterpenes, sesquiterpenes, and diterpenes were identified also as possible biogenic aerosols which may contribute to the total organic aerosol abundance leading to visibility reduction in the Grand Canyon

  9. 77 FR 9265 - Glen Canyon Dam Adaptive Management Work Group

    Science.gov (United States)

    2012-02-16

    ... DEPARTMENT OF THE INTERIOR Bureau of Reclamation Glen Canyon Dam Adaptive Management Work Group... Adaptive Management Work Group (AMWG) makes recommendations to the Secretary of the Interior concerning.... L. 102-575) of 1992. The AMP includes a Federal advisory committee, the AMWG, a technical work group...

  10. Aerodynamic effects of trees on pollutant concentration in street canyons

    NARCIS (Netherlands)

    Buccolieri, R.; Gromke, C.B.; Sabatino, Di S.; Ruck, B.

    2009-01-01

    This paper deals with aerodynamic effects of avenue-like tree planting on flow and traffic-originated pollutant dispersion in urban street canyons by means of wind tunnel experiments and numerical simulations. Several parameters affecting pedestrian level concentration are investigated, namely plant

  11. Sedimentary characteristics of samples collected from some submarine canyons

    NARCIS (Netherlands)

    Bouma, Arnold H.

    Oriented rectangular cores of 20.3 × 30.5 cm and 45.7 cm high have been collected in a number of submarine canyons off southern California (U.S.A.) and off the southern tip of Baja California (Mexico) for a detailed study of their sedimentary structures. By applying several methods, mainly X-ray

  12. Small Mammal Sampling in Mortandad and Los Alamos Canyons, 2005

    International Nuclear Information System (INIS)

    Kathy Bennett; Sherri Sherwood; Rhonda Robinson

    2006-01-01

    As part of an ongoing ecological field investigation at Los Alamos National Laboratory, a study was conducted that compared measured contaminant concentrations in sediment to population parameters for small mammals in the Mortandad Canyon watershed. Mortandad Canyon and its tributary canyons have received contaminants from multiple solid waste management units and areas of concern since establishment of the Laboratory in the 1940s. The study included three reaches within Effluent and Mortandad canyons (E-1W, M-2W, and M-3) that had a spread in the concentrations of metals and radionuclides and included locations where polychlorinated biphenyls and perchlorate had been detected. A reference location, reach LA-BKG in upper Los Alamos Canyon, was also included in the study for comparison purposes. A small mammal study was initiated to assess whether potential adverse effects were evident in Mortandad Canyon due to the presence of contaminants, designated as contaminants of potential ecological concern, in the terrestrial media. Study sites, including the reference site, were sampled in late July/early August. Species diversity and the mean daily capture rate were the highest for E-1W reach and the lowest for the reference site. Species composition among the three reaches in Mortandad was similar with very little overlap with the reference canyon. Differences in species composition and diversity were most likely due to differences in habitat. Sex ratios, body weights, and reproductive status of small mammals were also evaluated. However, small sample sizes of some species within some sites affected the analysis. Ratios of males to females by species of each site (n = 5) were tested using a Chi-square analysis. No differences were detected. Where there was sufficient sample size, body weights of adult small mammals were compared between sites. No differences in body weights were found. Reproductive status of species appears to be similar across sites. However, sample

  13. Small Mammal Sampling in Mortandad and Los Alamos Canyons, 2005

    Energy Technology Data Exchange (ETDEWEB)

    Bennett, Kathy; Sherwood, Sherri; Robinson, Rhonda

    2006-08-15

    As part of an ongoing ecological field investigation at Los Alamos National Laboratory, a study was conducted that compared measured contaminant concentrations in sediment to population parameters for small mammals in the Mortandad Canyon watershed. Mortandad Canyon and its tributary canyons have received contaminants from multiple solid waste management units and areas of concern since establishment of the Laboratory in the 1940s. The study included three reaches within Effluent and Mortandad canyons (E-1W, M-2W, and M-3) that had a spread in the concentrations of metals and radionuclides and included locations where polychlorinated biphenyls and perchlorate had been detected. A reference location, reach LA-BKG in upper Los Alamos Canyon, was also included in the study for comparison purposes. A small mammal study was initiated to assess whether potential adverse effects were evident in Mortandad Canyon due to the presence of contaminants, designated as contaminants of potential ecological concern, in the terrestrial media. Study sites, including the reference site, were sampled in late July/early August. Species diversity and the mean daily capture rate were the highest for E-1W reach and the lowest for the reference site. Species composition among the three reaches in Mortandad was similar with very little overlap with the reference canyon. Differences in species composition and diversity were most likely due to differences in habitat. Sex ratios, body weights, and reproductive status of small mammals were also evaluated. However, small sample sizes of some species within some sites affected the analysis. Ratios of males to females by species of each site (n = 5) were tested using a Chi-square analysis. No differences were detected. Where there was sufficient sample size, body weights of adult small mammals were compared between sites. No differences in body weights were found. Reproductive status of species appears to be similar across sites. However, sample

  14. Phenology of the adult angel lichen moth (Cisthene angelus) in Grand Canyon, USA

    Science.gov (United States)

    Metcalfe, Anya; Kennedy, Theodore A.; Muehlbauer, Jeffrey D.

    2016-01-01

    We investigated the phenology of adult angel lichen moths (Cisthene angelus) along a 364-km long segment of the Colorado River in Grand Canyon, Arizona, USA, using a unique data set of 2,437 light-trap samples collected by citizen scientists. We found that adults of C. angelus were bivoltine from 2012 to 2014. We quantified plasticity in wing lengths and sex ratios among the two generations and across a 545-m elevation gradient. We found that abundance, but not wing length, increased at lower elevations and that the two generations differed in size and sex distributions. Our results shed light on the life history and morphology of a common, but poorly known, species of moth endemic to the southwestern United States and Mexico.

  15. Thermal bioclimate in idealized urban street canyons in Campinas, Brazil

    Science.gov (United States)

    Abreu-Harbich, Loyde V.; Labaki, Lucila C.; Matzarakis, Andreas

    2014-01-01

    Among several urban design parameters, the height-to-width ratio (H/W) and orientation are important parameters strongly affecting thermal conditions in cities. This paper quantifies changes in thermal comfort due to typical urban canyon configurations in Campinas, Brazil, and presents urban guidelines concerning H/W ratios and green spaces to adapt urban climate change. The study focuses on thermal comfort issues of humans in urban areas and performs evaluation in terms of physiologically equivalent temperature (PET), based on long-term data. Meteorological data of air temperature, relative humidity, wind speed and solar radiation over a 7-year period (2003-2010) were used. A 3D street canyon model was designed with RayMan Pro software to simulate the influence of urban configuration on urban thermal climate. The following configurations and setups were used. The model canyon was 500 m in length, with widths 9, 21, and 44 m. Its height varied in steps of 2.5 m, from 5 to 40 m. The canyon could be rotated in steps of 15°. The results show that urban design parameters such as width, height, and orientation modify thermal conditions within street canyons. A northeast-southwest orientation can reduce PET during daytime more than other scenarios. Forestry management and green areas are recommended to promote shade on pedestrian areas and on façades, and to improve bioclimate thermal stress, in particular for H/W ratio less than 0.5. The method and results can be applied by architects and urban planners interested in developing responsive guidelines for urban climate issues.

  16. Savannah River Site: Canyons and associated facilities utilization study

    International Nuclear Information System (INIS)

    Ellison, D.; Dickenson, J.

    1995-01-01

    The Westinghouse Savannah River Company was asked by the U.S. Department of Energy (DOE) to study options for utilization of Savannah River Site (SRS) Canyons and Associated Facilities to support existing and potential future material stabilization and/or disposition missions. This report is WSRC's response to that request. It includes: (1) A compilation of pending DOE material stabilization and/or disposition decisions involving utilization of SRS canyons and associated facilities, including discussion of quantities and expected availability of materials for which SRS handling and/or processing capability is a reasonable alternative under consideration. (2) A description of SRS canyons and associated facilities affected by pending DOE material stabilization and/or disposition decisions, including discussion of material handling and/or processing capabilities and capacities. (3) A comparative evaluation of three proposed scenarios for SRS canyon utilization with respect to startup and operating schedules; annual and life cycle costs; impacts on completion of commitments in the DOE Implementation Plan (IP) for Defense Nuclear Facilities Safety Board (DNFSB) Recommendation 94-1; SRS ability to support alternatives under consideration in pending DOE materials stabilization and/or disposition decisions; and timing for potential transition to deactivation. (4) The sensitivity of the comparative evaluation of the three canyon utilization scenarios to the effect of the selection of other alternatives for individual stabilization missions or individual new missions. Briefings on the scope of this study have been presented to key representatives of several SRS public stakeholder groups. Briefings on the major conclusions from this study have been presented to WSRC Management, DOE-SR, EM-60, EM-1, and the DNFSB

  17. Analysis of dust samples collected from spent nuclear fuel interim storage containers at Hope Creek, Delaware, and Diablo Canyon, California

    Energy Technology Data Exchange (ETDEWEB)

    Bryan, Charles R. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Enos, David George [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2014-07-01

    Potentially corrosive environments may form on the surface of spent nuclear fuel dry storage canisters by deliquescence of deposited dusts. To assess this, samples of dust were collected from in-service dry storage canisters at two near-marine sites, the Hope Creek and Diablo Canyon storage installations, and have been characterized with respect to mineralogy, chemistry, and texture. At both sites, terrestrially-derived silicate minerals, including quartz, feldspars, micas, and clays, comprise the largest fraction of the dust. Also significant at both sites were particles of iron and iron-chromium metal and oxides generated by the manufacturing process. Soluble salt phases were minor component of the Hope Creek dusts, and were compositionally similar to inland salt aerosols, rich in calcium, sulfate, and nitrate. At Diablo Canyon, however, sea-salt aerosols, occurring as aggregates of NaCl and Mg-sulfate, were a major component of the dust samples. The seasalt aerosols commonly occurred as hollow spheres, which may have formed by evaporation of suspended aerosol seawater droplets, possibly while rising through the heated annulus between the canister and the overpack. The differences in salt composition and abundance for the two sites are attributed to differences in proximity to the open ocean and wave action. The Diablo Canyon facility is on the shores of the Pacific Ocean, while the Hope Creek facility is on the shores of the Delaware River, several miles from the open ocean.

  18. Presence of tau pathology within foetal neural allografts in patients with Huntington's and Parkinson's disease.

    Science.gov (United States)

    Cisbani, Giulia; Maxan, Alexander; Kordower, Jeffrey H; Planel, Emmanuel; Freeman, Thomas B; Cicchetti, Francesca

    2017-11-01

    Cell replacement has been explored as a therapeutic strategy to repair the brain in patients with Huntington's and Parkinson's disease. Post-mortem evaluations of healthy grafted tissue in such cases have revealed the development of Huntington- or Parkinson-like pathology including mutant huntingtin aggregates and Lewy bodies. An outstanding question remains if tau pathology can also be seen in patients with Huntington's and Parkinson's disease who had received foetal neural allografts. This was addressed by immunohistochemical/immunofluorescent stainings performed on grafted tissue of two Huntington's disease patients, who came to autopsy 9 and 12 years post-transplantation, and two patients with Parkinson's disease who came to autopsy 18 months and 16 years post-transplantation. We show that grafts also contain tau pathology in both types of transplanted patients. In two patients with Huntington's disease, the grafted tissue showed the presence of hyperphosphorylated tau [both AT8 (phospho-tau Ser202 and Thr205) and CP13 (pSer202) immunohistochemical stainings] pathological inclusions, neurofibrillary tangles and neuropil threads. In patients with Parkinson's disease, the grafted tissue was characterized by hyperphosphorylated tau (AT8; immunofluorescent staining) pathological inclusions, neurofibrillary tangles and neuropil threads but only in the patient who came to autopsy 16 years post-transplantation. Abundant tau-related pathology was observed in the cortex and striatum of all cases studied. While the striatum of the grafted Huntington's disease patient revealed an equal amount of 3-repeat and 4-repeat isoforms of tau, the grafted tissue showed elevated 4-repeat isoforms by western blot. This suggests that transplants may have acquired tau pathology from the host brain, although another possibility is that this was due to acceleration of ageing. This finding not only adds to the recent reports that tau pathology is a feature of these neurodegenerative

  19. Environmental analysis of Lower Pueblo/Lower Los Alamos Canyon, Los Alamos, New Mexico

    International Nuclear Information System (INIS)

    Ferenbaugh, R.W.; Buhl, T.E.; Stoker, A.K.; Becker, N.M.; Rodgers, J.C.; Hansen, W.R.

    1994-12-01

    The radiological survey of the former radioactive waste treatment plant site (TA-45), Acid Canyon, Pueblo Canyon, and Los Alamos Canyon found residual contamination at the site itself and in the channel and banks of Acid, Pueblo, and lower Los Alamos Canyons all the way to the Rio Grande. The largest reservoir of residual radioactivity is in lower Pueblo Canyon, which is on DOE property. However, residual radioactivity does not exceed proposed cleanup criteria in either lower Pueblo or lower Los Alamos Canyons. The three alternatives proposed are (1) to take no action, (2) to construct a sediment trap in lower Pueblo Canyon to prevent further transport of residual radioactivity onto San Ildefonso Indian Pueblo land, and (3) to clean the residual radioactivity from the canyon system. Alternative 2, to cleanup the canyon system, is rejected as a viable alternative. Thousands of truckloads of sediment would have to be removed and disposed of, and this effort is unwarranted by the low levels of contamination present. Residual radioactivity levels, under either present conditions or projected future conditions, will not result in significant radiation doses to persons exposed. Modeling efforts show that future transport activity will not result in any residual radioactivity concentrations higher than those already existing. Thus, although construction of a sediment trap in lower Pueblo Canyon is a viable alternative, this effort also is unwarranted, and the no-action alternative is the preferred alternative

  20. Environmental analysis of Lower Pueblo/Lower Los Alamos Canyon, Los Alamos, New Mexico

    Energy Technology Data Exchange (ETDEWEB)

    Ferenbaugh, R.W.; Buhl, T.E.; Stoker, A.K.; Becker, N.M.; Rodgers, J.C.; Hansen, W.R.

    1994-12-01

    The radiological survey of the former radioactive waste treatment plant site (TA-45), Acid Canyon, Pueblo Canyon, and Los Alamos Canyon found residual contamination at the site itself and in the channel and banks of Acid, Pueblo, and lower Los Alamos Canyons all the way to the Rio Grande. The largest reservoir of residual radioactivity is in lower Pueblo Canyon, which is on DOE property. However, residual radioactivity does not exceed proposed cleanup criteria in either lower Pueblo or lower Los Alamos Canyons. The three alternatives proposed are (1) to take no action, (2) to construct a sediment trap in lower Pueblo Canyon to prevent further transport of residual radioactivity onto San Ildefonso Indian Pueblo land, and (3) to clean the residual radioactivity from the canyon system. Alternative 2, to cleanup the canyon system, is rejected as a viable alternative. Thousands of truckloads of sediment would have to be removed and disposed of, and this effort is unwarranted by the low levels of contamination present. Residual radioactivity levels, under either present conditions or projected future conditions, will not result in significant radiation doses to persons exposed. Modeling efforts show that future transport activity will not result in any residual radioactivity concentrations higher than those already existing. Thus, although construction of a sediment trap in lower Pueblo Canyon is a viable alternative, this effort also is unwarranted, and the no-action alternative is the preferred alternative.

  1. Submarine canyons represent an essential habitat network for krill hotspots in a Large Marine Ecosystem.

    Science.gov (United States)

    Santora, Jarrod A; Zeno, Ramona; Dorman, Jeffrey G; Sydeman, William J

    2018-05-15

    Submarine canyon systems are ubiquitous features of marine ecosystems, known to support high levels of biodiversity. Canyons may be important to benthic-pelagic ecosystem coupling, but their role in concentrating plankton and structuring pelagic communities is not well known. We hypothesize that at the scale of a large marine ecosystem, canyons provide a critical habitat network, which maintain energy flow and trophic interactions. We evaluate canyon characteristics relative to the distribution and abundance of krill, critically important prey in the California Current Ecosystem. Using a geological database, we conducted a census of canyon locations, evaluated their dimensions, and quantified functional relationships with krill hotspots (i.e., sites of persistently elevated abundance) derived from hydro-acoustic surveys. We found that 76% of krill hotspots occurred within and adjacent to canyons. Most krill hotspots were associated with large shelf-incising canyons. Krill hotspots and canyon dimensions displayed similar coherence as a function of latitude and indicate a potential regional habitat network. The latitudinal migration of many fish, seabirds and mammals may be enhanced by using this canyon-krill network to maintain foraging opportunities. Biogeographic assessments and predictions of krill and krill-predator distributions under climate change may be improved by accounting for canyons in habitat models.

  2. Canyon formation constraints on the discharge of catastrophic outburst floods of Earth and Mars

    Science.gov (United States)

    Lapotre, Mathieu G. A.; Lamb, Michael P.; Williams, Rebecca M. E.

    2016-07-01

    Catastrophic outburst floods carved amphitheater-headed canyons on Earth and Mars, and the steep headwalls of these canyons suggest that some formed by upstream headwall propagation through waterfall erosion processes. Because topography evolves in concert with water flow during canyon erosion, we suggest that bedrock canyon morphology preserves hydraulic information about canyon-forming floods. In particular, we propose that for a canyon to form with a roughly uniform width by upstream headwall retreat, erosion must occur around the canyon head, but not along the sidewalls, such that canyon width is related to flood discharge. We develop a new theory for bedrock canyon formation by megafloods based on flow convergence of large outburst floods toward a horseshoe-shaped waterfall. The model is developed for waterfall erosion by rock toppling, a candidate erosion mechanism in well fractured rock, like columnar basalt. We apply the model to 14 terrestrial (Channeled Scablands, Washington; Snake River Plain, Idaho; and Ásbyrgi canyon, Iceland) and nine Martian (near Ares Vallis and Echus Chasma) bedrock canyons and show that predicted flood discharges are nearly 3 orders of magnitude less than previously estimated, and predicted flood durations are longer than previously estimated, from less than a day to a few months. Results also show a positive correlation between flood discharge per unit width and canyon width, which supports our hypothesis that canyon width is set in part by flood discharge. Despite lower discharges than previously estimated, the flood volumes remain large enough for individual outburst floods to have perturbed the global hydrology of Mars.

  3. Radiation sensitivity of fibroblast strains from patients with Usher's syndrome, Duchenne muscular dystrophy, and Huntington's disease

    International Nuclear Information System (INIS)

    Nove, J.; Little, J.B.; Tarone, R.E.; Robbins, J.H.

    1987-01-01

    The colony-forming ability of 10 normal human fibroblast cell strains and of 10 strains representing 3 degenerative diseases of either nerve or muscle cells was determined after exposure of the cells to X-rays or β-particles from tritiated water. Both methods of irradiation yielded similar comparative results. The fibroblast strains from the 5 Usher's syndrome patients and from 1 of the 2 Huntington's disease patients were hypersensitive to radiation, while those from the 3 Duchenne muscular dystrophy patients and the second Huntington's disease patient had normal sensitivity to radiation. These results indicate both disease-specific and strain-specific differences in the survival of fibroblasts after exposure to ionizing radiation. 38 refs.; 2 figs.; 3 tabs

  4. Huntington's disease predictive testing: the case for an assessment approach to requests from adolescents.

    Science.gov (United States)

    Binedell, J; Soldan, J R; Scourfield, J; Harper, P S

    1996-01-01

    Adolescents who are actively requesting Huntington's predictive testing of their own accord pose a dilemma to those providing testing. In the absence of empirical evidence as regards the impact of genetic testing on minors, current policy and guidelines, based on the ethical principles of non-maleficence and respect for individual autonomy and confidentiality, generally exclude the testing of minors. It is argued that adherence to an age based exclusion criterion in Huntington's disease predictive testing protocols is out of step with trends in UK case law concerning minors' consent to medical treatment. Furthermore, contributions from developmental psychology and research into adolescents' decision making competence suggest that adolescents can make informed choices about their health and personal lives. Criteria for developing an assessment approach to such requests are put forward and the implications of a case by case evaluation of competence to consent in terms of clinicians' tolerance for uncertainty are discussed. PMID:8950670

  5. Marine litter in submarine canyons of the Bay of Biscay

    Science.gov (United States)

    van den Beld, Inge M. J.; Guillaumont, Brigitte; Menot, Lénaïck; Bayle, Christophe; Arnaud-Haond, Sophie; Bourillet, Jean-François

    2017-11-01

    Marine litter is a matter of increasing concern worldwide, from shallow seas to the open ocean and from beaches to the deep-seafloor. Indeed, the deep sea may be the ultimate repository of a large proportion of litter in the ocean. We used footage acquired with a Remotely Operated Vehicle (ROV) and a towed camera to investigate the distribution and composition of litter in the submarine canyons of the Bay of Biscay. This bay contains many submarine canyons housing Vulnerable Marine Ecosystems (VMEs) such as scleractinian coral habitats. VMEs are considered to be important for fish and they increase the local biodiversity. The objectives of the study were to investigate and discuss: (i) litter density, (ii) the principal sources of litter, (iii) the influence of environmental factors on the distribution of litter, and (iv) the impact of litter on benthic communities. Litter was found in all 15 canyons and at three sites on the edge of the continental shelf/canyon, in 25 of 29 dives. The Belle-île and Arcachon Canyons contained the largest amounts of litter, up to 12.6 and 9.5 items per 100 images respectively. Plastic items were the most abundant (42%), followed by fishing-related items (16%). The litter had both a maritime and a terrestrial origin. The main sources could be linked to fishing activities, major shipping lanes and river discharges. Litter appeared to accumulate at water depths of 801-1100 m and 1401-1700 m. In the deeper of these two depth ranges, litter accumulated on a geologically structured area, accounting for its high frequency at this depth. A larger number of images taken in areas of coral in the shallower of these two depth ranges may account for the high frequency of litter detection at this depth. A larger number of litter items, including plastic objects in particular, were observed on geological structures and in coral areas than on areas of bare substratum. The distribution of fishing-related items was similar for the various types of

  6. Characterisation of current and future GNSS performance in urban canyons using a high quality 3-D urban model of Melbourne, Australia

    Science.gov (United States)

    Gang-jun, Liu; Kefei, Zhang; Falin, Wu; Liam, Densley; Retscher, Günther

    2009-03-01

    Global Navigation Satellite System (GNSS) is a critical space-borne geospatial infrastructure providing essential positioning supports to a range of location-sensitive applications. GNSS is currently dominated by the US Global Positioning System (GPS) constellation. The next generation GNSS is expected to offer more satellites, better positioning provision, and improved availability and continuity of navigation support. However, GNSS performance in 3-D urban environments is problematic because GNSS signals are either completely blocked or severely degraded by high-rising geographic features like buildings. The aim of this study is to gain an in-depth understanding of the changing spatial patterns of GNSS performance, measured by the number of visible satellites (NVS) and position dilution-of-precision (PDOP), in the urban canyons of Melbourne, Australia. The methodology used includes the following steps: (1) determination of the dynamic orbital positions of current and future GNSS satellites; (2) development of a 3-D urban model of high geometric quality for Melbourne Central Business District (CBD); (3) evaluation of GNSS performance for every specified location in the urban canyons; and (4) visualisation and characterisation of the dynamic spatial patterns of GNSS performances in the urban canyons. As expected, the study shows that the integration of the GPS and Galileo constellations results in higher availability and stronger geometry, leading to significant improvement of GNSS performance in urban canyons of Melbourne CBD. Some conclusions are drawn and further research currently undertaken is also outlined.

  7. Grunting in genetically modified minipig animal model for Huntington ´s disease - a pilot experiment

    Czech Academy of Sciences Publication Activity Database

    Tykalová, T.; Hlavnička, J.; Mačáková, Monika; Baxa, Monika; Cmejla, R.; Motlík, Jan; Klempíř, J.; Rusz, J.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 12-13 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * mitochondria * DNA damage Subject RIV: FH - Neurology

  8. Double strand DNA breaks response in Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Šolc, Petr; Valášek, Jan; Rausová, Petra; Juhásová, Jana; Juhás, Štefan; Motlík, Jan

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 15-15 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * DNA damage * double strand DNA breaks Subject RIV: FH - Neurology

  9. Dynamics of the connectome in Huntington's disease: A longitudinal diffusion MRI study

    OpenAIRE

    Odish, Omar F.F.; Caeyenberghs, Karen; Hosseini, Hadi; van den Bogaard, Simon J.A.; Roos, Raymund A.C.; Leemans, Alexander

    2015-01-01

    Abstract Objectives To longitudinally investigate the connectome in different stages of Huntington's disease (HD) by applying graph theoretical analysis to diffusion MRI data. Experimental design We constructed weighted structural networks and calculated their topological properties. Twenty-two premanifest (preHD), 10 early manifest HD and 24 healthy controls completed baseline and 2 year follow-up scans. We stratified the preHD group based on their predicted years to disease onset into a far...

  10. Minipig Model of Huntington's Disease: H-1 Magnetic Resonance Spectroscopy of the Brain

    Czech Academy of Sciences Publication Activity Database

    Jozefovičová, M.; Herynek, V.; Jírů, F.; Dezortová, M.; Juhásová, Jana; Juhás, Štefan; Motlík, Jan; Hájek, M.

    2016-01-01

    Roč. 65, č. 1 (2016), s. 155-163 ISSN 0862-8408 R&D Projects: GA TA ČR(CZ) TA01011466; GA MŠk(CZ) 7F14308; GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington´s disease * minipigs * magnetic resonance spectroscopy Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 1.461, year: 2016

  11. Human glia can both induce and rescue aspects of disease phenotype in Huntington disease

    DEFF Research Database (Denmark)

    Benraiss, Abdellatif; Wang, Su; Herrlinger, Stephanie

    2016-01-01

    The causal contribution of glial pathology to Huntington disease (HD) has not been heavily explored. To define the contribution of glia to HD, we established human HD glial chimeras by neonatally engrafting immunodeficient mice with mutant huntingtin (mHTT)-expressing human glial progenitor cells...... chimeras are hyperexcitable. Conversely, normal glia can ameliorate disease phenotype in transgenic HD mice, as striatal transplantation of normal glia rescues aspects of electrophysiological and behavioural phenotype, restores interstitial potassium homeostasis, slows disease progression and extends...

  12. The Effect of Music Therapy in Patients with Huntington's Disease: A Randomized Controlled Trial.

    Science.gov (United States)

    van Bruggen-Rufi, Monique C H; Vink, Annemieke C; Wolterbeek, Ron; Achterberg, Wilco P; Roos, Raymund A C

    2017-01-01

    Music therapy may have beneficial effects on improving communication and expressive skills in patients with Huntington's disease (HD). Most studies are, however, small observational studies and methodologically limited. Therefore we conducted a multi-center randomized controlled trial. To determine the efficacy of music therapy in comparison with recreational therapy in improving quality of life of patients with advanced Huntington's disease by means of improving communication. Sixty-three HD-patients with a Total Functional Capacity (TFC) score of ≤7, admitted to four long-term care facilities in The Netherlands, were randomized to receive either group music therapy or group recreational therapy in 16 weekly sessions. They were assessed at baseline, after 8, 16 and 28 weeks using the Behaviour Observation Scale for Huntington (BOSH) and the Problem Behaviour Assessment-short version (PBA-s). A linear mixed model with repeated measures was used to compare the scores between the two groups. Group music therapy offered once weekly for 16 weeks to patients with Huntington's disease had no additional beneficial effect on communication or behavior compared to group recreational therapy. This was the first study to assess the effect of group music therapy on HD patients in the advanced stages of the disease. The beneficial effects of music therapy, recorded in many, mainly qualitative case reports and studies, could not be confirmed with the design (i.e. group therapy vs individual therapy) and outcome measures that have been used in the present study. A comprehensive process-evaluation alongside the present effect evaluation is therefore performed.

  13. EMQN/CMGS best practice guidelines for the molecular genetic testing of Huntington disease

    OpenAIRE

    Losekoot, Monique; van Belzen, Martine J; Seneca, Sara; Bauer, Peter; Stenhouse, Susan A R; Barton, David E

    2012-01-01

    Huntington disease (HD) is caused by the expansion of an unstable polymorphic trinucleotide (CAG)n repeat in exon 1 of the HTT gene, which translates into an extended polyglutamine tract in the protein. Laboratory diagnosis of HD involves estimation of the number of CAG repeats. Molecular genetic testing for HD is offered in a wide range of laboratories both within and outside the European community. In order to measure the quality and raise the standard of molecular genetic testing in these ...

  14. Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease

    OpenAIRE

    Mealer, Robert G.; Subramaniam, Srinivasa; Snyder, Solomon H.

    2013-01-01

    Although the mutated protein causing Huntington's disease (HD) is expressed throughout the body, the major pathology of HD is localized to the striatum of the brain. We previously reported that the striatal-enriched protein Rhes binds the mutated huntingtin protein and enhances its cytotoxicity. We now demonstrate that Rhes-deleted mice are dramatically protected from neurotoxicity and motor dysfunction in a striatal-specific model of HD elicited by 3-nitropropionic acid. This finding suggest...

  15. Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study.

    Science.gov (United States)

    2008-12-01

    To determine whether ethyl-eicosapentaenoic acid (ethyl-EPA), an omega-3 fatty acid, improves the motor features of Huntington disease. Six-month multicenter, randomized, double-blind, placebo-controlled trial followed by a 6-month open-label phase without disclosing initial treatment assignments. Forty-one research sites in the United States and Canada. Three hundred sixteen adults with Huntington disease, enriched for a population with shorter trinucleotide (cytosine-adenine-guanine) repeat length expansions. Random assignment to placebo or ethyl-EPA, 1 g twice a day, followed by open-label treatment with ethyl-EPA. Six-month change in the Total Motor Score 4 component of the Unified Huntington's Disease Rating Scale analyzed for all research participants and those with shorter cytosine-adenine-guanine repeat length expansions (<45). At 6 months, the Total Motor Score 4 point change for patients receiving ethyl-EPA did not differ from that for those receiving placebo. No differences were found in measures of function, cognition, or global impression. Before public disclosure of the 6-month placebo-controlled results, 192 individuals completed the open-label phase. The Total Motor Score 4 change did not worsen for those who received active treatment for 12 continuous months compared with those who received active treatment for only 6 months (2.0-point worsening; P=.02). Ethyl-EPA was not beneficial in patients with Huntington disease during 6 months of placebo-controlled evaluation. Clinical Trial Registry clinicaltrials.gov Identifier: NCT00146211.

  16. Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease

    OpenAIRE

    Keene, C. Dirk; Rodrigues, Cecilia M. P.; Eich, Tacjana; Chhabra, Manik S.; Steer, Clifford J.; Low, Walter C.

    2002-01-01

    Huntington's disease (HD) is an untreatable neurological disorder caused by selective and progressive degeneration of the caudate nucleus and putamen of the basal ganglia. Although the etiology of HD pathology is not fully understood, the observed loss of neuronal cells is thought to occur primarily through apoptosis. Furthermore, there is evidence in HD that cell death is mediated through mitochondrial pathways, and mitochondrial deficits are commonly associated with HD. We have previously r...

  17. Characterizing the sponge grounds of Grays Canyon, Washington, USA

    Science.gov (United States)

    Powell, Abby N.; Clarke, M. Elizabeth; Fruh, Erica; Chaytor, Jason; Reiswig, Henry M.; Whitmire, Curt E.

    2018-01-01

    Deep-sea sponge grounds are relatively understudied ecosystems that may provide key habitats for a large number of fish and invertebrates including commercial species. Glass sponge grounds have been discovered from the tropics to polar regions but there are only a few places with high densities of dictyonine sponges. Dictyonine glass sponges have a fused skeleton, which stays intact when they die and in some areas the accumulation of successive generations of sponges leads to the formation of reefs. In 2010 and 2016, we surveyed an area near Grays Canyon in Washington, USA, where dense aggregations of glass sponges and potential sponge reefs were discovered in 2007. Our primary aims were to make a preliminary assessment of whether the glass sponges form reefs at this location, characterize the sponge assemblage present at this site and examine associations between the sponges and commercially important species. Multibeam mapping and sub-bottom profiling indicate that the glass sponges at this site do not form reefs and are mostly attached to hard substrates. Analysis of photographs collected by an autonomous underwater vehicle and samples collected by a remotely operated vehicle guided by telepresence revealed the presence of two abundant dictyonine sponge species at this site, Heterochone calyx and Aphrocallistes vastus (mean densities = 1.43 ± 0.057 per 10 m2, max = 24 per 10 m2). We also observed a large number of non-reef-building glass sponges and various demosponges including a potentially new species in the genus Acarnus. A diverse fish assemblage was recorded at this site including eight species of rockfish. Rockfish abundance was positively related to sponge abundance. Spot prawns (Pandalus platyceros) were also abundant and were strongly associated with sponges. Despite not finding sponge reefs, this is an ecologically significant area. Further research is necessary to determine the environmental factors that give rise to the abundance of large

  18. What to Do with a Second Chance in Life? Long-Term Experiences of Non-carriers of Huntington's Disease.

    Science.gov (United States)

    Winnberg, Elisabeth; Winnberg, Ulrika; Pohlkamp, Lilian; Hagberg, Anette

    2018-04-07

    Little is known about how people's lives are influenced when going from a 50% risk status of Huntington's disease (HD) to no risk after performing predictive testing. In this study, 20 interviews were conducted to explore the long-term (> 5 years) experiences after receiving predictive test results as a non-carrier of HD. The results showed a broad variety of both positive and negative reactions. The most prominent positive reaction reported was feelings of relief and gratitude, of not carrying the HD mutation for themselves and for their children. Also, the non-carrier status promoted in some individuals' significant life changes such as a wishing to have (more) children, pursuing a career or breaking up from an unhappy relationship. However, negative reactions on their psychological well-being were also described. Some had experienced psychological pressure of needing to do something extraordinary in their lives; others expressed feelings of guilt towards affected or untested siblings, resulting in sadness or clinical depression. The new genetic risk status could generate a need of re-orientation, a process that for some persons took several years to accomplish. The results of the present study show the importance of offering long-term post-result counselling for non-carriers in order to deal with the psychological consequences that may follow predictive testing.

  19. Prevalence of Huntington's disease gene CAG trinucleotide repeat alleles in patients with bipolar disorder.

    Science.gov (United States)

    Ramos, Eliana Marisa; Gillis, Tammy; Mysore, Jayalakshmi S; Lee, Jong-Min; Alonso, Isabel; Gusella, James F; Smoller, Jordan W; Sklar, Pamela; MacDonald, Marcy E; Perlis, Roy H

    2015-06-01

    Huntington's disease is a neurodegenerative disorder characterized by motor, cognitive, and psychiatric symptoms that are caused by huntingtin gene (HTT) CAG trinucleotide repeat alleles of 36 or more units. A greater than expected prevalence of incompletely penetrant HTT CAG repeat alleles observed among individuals diagnosed with major depressive disorder raises the possibility that another mood disorder, bipolar disorder, could likewise be associated with Huntington's disease. We assessed the distribution of HTT CAG repeat alleles in a cohort of individuals with bipolar disorder. HTT CAG allele sizes from 2,229 Caucasian individuals diagnosed with DSM-IV bipolar disorder were compared to allele sizes in 1,828 control individuals from multiple cohorts. We found that HTT CAG repeat alleles > 35 units were observed in only one of 4,458 chromosomes from individuals with bipolar disorder, compared to three of 3,656 chromosomes from control subjects. These findings do not support an association between bipolar disorder and Huntington's disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. Palliative Care in Huntington Disease: Personal Reflections and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Christopher G. Tarolli

    2017-04-01

    Full Text Available Background Huntington disease is a fatal, autosomal dominant, neurodegenerative disorder manifest by the triad of a movement disorder, behavioral disturbances, and dementia. At present, no curative or disease modifying therapies exist for the condition and current treatments are symptomatic. Palliative care is an approach to care that focuses on symptom relief, patient and caregiver support, and end of life care. There is increasing evidence of the benefit of palliative care throughout the course of neurodegenerative conditions including Parkinson disease and amyotrophic lateral sclerosis. However, beyond its application at the end of life, little is known about the role of palliative care in Huntington disease.Methods In this article, we discuss what is known about palliative care in Huntington disease, specifically related to early disease burden, caregiver burnout, advance care planning, and end of life care.Results We provide a review of the current literature and discuss our own care practices.Discussion We conclude by discussing questions that remain unanswered and positing ideas for future work in the field.

  1. Did the "Woman in the Attic" in Jane Eyre Have Huntington Disease?

    Directory of Open Access Journals (Sweden)

    Elizabeth A. Coon

    2015-07-01

    Full Text Available Background: References to neurologic disorders are frequently found in fictional literature and may precede description in the medical literature. Aim: Our aim was to compare Charlotte Brontë’s depiction of Bertha Mason in Jane Eyre to the tenets set forth in George Huntington’s original essay “On chorea” with the hypothesis that Mason was displaying features of Huntington disease. Results: Charlotte Brontë’s 1847 Victorian novel Jane Eyre features the character Bertha Mason, who is portrayed with a progressive psychiatric illness, violent movements, and possible cognitive decline. Similar to Huntington’s tenets, Mason has a disorder with a strong family history suggestive of autosomal dominant inheritance with onset in adulthood, and culminating in suicide. Conclusion: Brontë’s character had features of Huntington disease as originally described by Huntington. Brontë’s keen characterization may have increased awareness of treatment of neuropsychiatric patients in the Victorian era.

  2. Canyon air flow measurement utilizing ASME standard pitot tube arrays

    International Nuclear Information System (INIS)

    Moncrief, B.R.

    1990-01-01

    The Savannah River Site produces nuclear materials for national defense. In addition to nuclear reactors, the site has separation facilities for reprocessing irradiated nuclear fuel. The chemical separation of highly radioactive materials takes place by remote control in large buildings called canyons. Personnel in these buildings are shielded from radiation by thick concrete walls. Contaminated air is exhausted from the canyons and contaminants are removed by sand filters prior to release to the atmosphere through a stack. When these facilities were built on a crash basis in the early 1950's, inadequate means were provided for pressure and air flow measurement. This presentation describes the challenge we faced in retrofitting a highly radioactive, heavily shielded facility with instrumentation to provide this capability

  3. Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

    Directory of Open Access Journals (Sweden)

    GILBERTO LEVY

    1999-09-01

    Full Text Available Early-onset Huntington's disease (HD occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.Doença de Huntington (DH de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.

  4. Hydraulics of outburst floods spilling over a steep-walled canyon: Implications for paleo-discharges on Mars

    Science.gov (United States)

    Lapotre, Mathieu; Lamb, Michael

    2013-04-01

    Canyons carved by outburst floods are common landforms on Earth and Mars. These canyons are generally found in fractured basalts and jointed sedimentary rocks. Flood-carved canyons commonly have steep headwalls and a roughly constant width, and are often thought to have formed from upstream headwall propagation due to waterfall erosion. Because morphology is readily available from satellite imagery, these canyons offer a unique opportunity to quantify the discharge of rare, catastrophic paleo-floods on Earth and Mars. However, mechanistic relationships that relate canyon size to flood discharge have yet to be developed. We propose that the width of a canyon headwall in fractured rock is set by the spatial distribution of erosion around the rim of the canyon, which is controlled by the distribution of shear stresses induced by the overflowing water as it is focused into the canyon head. We test this hypothesis by performing a series of numerical simulations of flood-water focusing using ANUGA Hydro, a 2D-depth averaged, fully turbulent, hydraulic numerical modeling suite allowing for Froude-number transitions. The numerical simulations were designed to explore five dimensionless variables: the aspect ratio of the canyon (length normalized by width), the canyon width to flood-water width ratio, the canyon width to normal-flow depth ratio, the Froude number, and the topographic gradient upstream of the canyon. Preliminary results show that flow focusing leads to increased shear stresses at the canyon head compared to the sides of the canyon for subcritical floods and higher canyon aspect ratios. This suggests that proto-canyons start growing from a topographic defect in all directions until they reach a critical length for the side walls to dry. Once this critical length is attained, canyons focus most of the flood waters into their heads, and propagate upstream only, maintaining roughly constant widths. Preliminary results suggest that canyon width may be used to

  5. Geomorphic characterization of four shelf-sourced submarine canyons along the U.S. Mid-Atlantic continental margin

    Science.gov (United States)

    Obelcz, Jeffrey; Brothers, Daniel S.; Chaytor, Jason D.; ten Brink, Uri S.; Ross, Steve W.; Brooke, Sandra

    2013-01-01

    Shelf-sourced submarine canyons are common features of continental margins and are fundamental to deep-sea sedimentary systems. Despite their geomorphic and geologic significance, relatively few passive margin shelf-breaching canyons worldwide have been mapped using modern geophysical methods. Between 2007 and 2012 a series of geophysical surveys was conducted across four major canyons of the US Mid-Atlantic margin: Wilmington, Baltimore, Washington, and Norfolk canyons. More than 5700 km2 of high-resolution multibeam bathymetry and 890 line-km of sub-bottom CHIRP profiles were collected along the outer shelf and uppermost slope (depths of 80-1200 m). The data allowed us to compare and contrast the fine-scale morphology of each canyon system. The canyons have marked differences in the morphology and orientation of canyon heads, steepness and density of sidewall gullies, and the character of the continental shelf surrounding canyon rims. Down-canyon axial profiles for Washington, Baltimore and Wilmington canyons have linear shapes, and each canyon thalweg exhibits morphological evidence for recent, relatively small-scale sediment transport. For example, Washington Canyon displays extremely steep wall gradients and contains ~100 m wide, 5–10 m deep, v-shaped incisions down the canyon axis, suggesting modern or recent sediment transport. In contrast, the convex axial thalweg profile, the absence of thalweg incision, and evidence for sediment infilling at the canyon head, suggest that depositional processes strongly influence Norfolk Canyon during the current sea-level high-stand. The north walls of Wilmington, Washington and Norfolk canyons are steeper than the south walls due to differential erosion, though the underlying cause for this asymmetry is not clear. Furthermore, we speculate that most of the geomorphic features observed within the canyons (e.g., terraces, tributary canyons, gullies, and hanging valleys) were formed during the Pleistocene, and show only

  6. Study of line source characteristics for 2-D physical modelling of pollutant dispersion in street canyons

    Energy Technology Data Exchange (ETDEWEB)

    Meroney, Robert N. [Fluid Mechanics and Wind Engineering Program, Civil Engineering Department, Colorado State University Fort Collins, CO (United States); Pavageau, Michel; Rafailidis, Stilianos; Schatzmann, Michael [Meteorologisches Institut, Universitaet Hamburg, Hamburg (Germany)

    1996-08-01

    The University of Hamburg initiated a wind tunnel study of car exhaust dispersion from street canyons in an urban environment to investigate how pollution dispersion is affected by street geometry. Particular emphasis at the beginning of this work was put on the design of a line source to represent traffic exhaust. Pollution dispersion was studied in two dimensions (i.e., infinite-length streets were assumed). The case of an isolated street canyon in open country was examined first. The same street canyon geometry was subsequently studied in an urban environment, i.e., with additional canyons of similar geometry upstream and downstream of the test street. The dynamic and dispersion characteristics of the flow in the two cases were quite different. In the canyon amidst open country we observed better canyon ventilation than in the urban roughness case

  7. The timing of sediment transport down Monterey Submarine Canyon, offshore California

    DEFF Research Database (Denmark)

    Stevens, Thomas; Paull, C.K.; Ussler, W., III

    2014-01-01

    luminescence (OSL) ages of quartz sand deposits and accelerator mass spectrometry 14C ages measured on benthic foraminifera to examine the timing of sediment transport through the axial channel of Monterey Submarine Canyon and Fan, offshore California. The OSL ages date the timing of sediment entry...... dates with water depth provides evidence of mixing and temporary storage of sediment as it moves through the canyon system. The ages also indicate that the frequency of sediment transport events decreases with distance down the canyon channel system. The amalgamated sands near the canyon head yield OSL......While submarine canyons are the major conduits through which sediments are transported from the continents out into the deep sea, the time it takes for sediment to pass down through a submarine canyon system is poorly constrained. Here we report on the first study to couple optically stimulated...

  8. A simple model for calculating air pollution within street canyons

    Science.gov (United States)

    Venegas, Laura E.; Mazzeo, Nicolás A.; Dezzutti, Mariana C.

    2014-04-01

    This paper introduces the Semi-Empirical Urban Street (SEUS) model. SEUS is a simple mathematical model based on the scaling of air pollution concentration inside street canyons employing the emission rate, the width of the canyon, the dispersive velocity scale and the background concentration. Dispersive velocity scale depends on turbulent motions related to wind and traffic. The parameterisations of these turbulent motions include two dimensionless empirical parameters. Functional forms of these parameters have been obtained from full scale data measured in street canyons at four European cities. The sensitivity of SEUS model is studied analytically. Results show that relative errors in the evaluation of the two dimensionless empirical parameters have less influence on model uncertainties than uncertainties in other input variables. The model estimates NO2 concentrations using a simple photochemistry scheme. SEUS is applied to estimate NOx and NO2 hourly concentrations in an irregular and busy street canyon in the city of Buenos Aires. The statistical evaluation of results shows that there is a good agreement between estimated and observed hourly concentrations (e.g. fractional bias are -10.3% for NOx and +7.8% for NO2). The agreement between the estimated and observed values has also been analysed in terms of its dependence on wind speed and direction. The model shows a better performance for wind speeds >2 m s-1 than for lower wind speeds and for leeward situations than for others. No significant discrepancies have been found between the results of the proposed model and that of a widely used operational dispersion model (OSPM), both using the same input information.

  9. Canyon Creek: A late Pleistocene vertebrate locality in interior Alaska

    Science.gov (United States)

    Weber, Florence R.; Hamilton, Thomas D.; Hopkins, David M.; Repenning, Charles A.; Haas, Herbert

    1981-09-01

    The Canyon Creek vertebrate-fossil locality is an extensive road cut near Fairbanks that exposes sediments that range in age from early Wisconsin to late Holocene. Tanana River gravel at the base of the section evidently formed during the Delta Glaciation of the north-central Alaska Range. Younger layers and lenses of fluvial sand are interbedded with arkosic gravel from Canyon Creek that contains tephra as well as fossil bones of an interstadial fauna about 40,000 years old. Solifluction deposits containing ventifacts, wedge casts, and rodent burrows formed during a subsequent period of periglacial activity that took place during the maximum phase of Donnelly Glaciation about 25,000-17,000 years ago. Overlying sheets of eolian sand are separated by a 9500-year-old paleosol that may correlate with a phase of early Holocene spruce expansion through central Alaska. The Pleistocene fauna from Canyon Creek consists of rodents (indicated by burrows), Mammuthus primigenius (woolly mammoth), Equus lambei (Yukon wild ass), Camelops hesternus (western camel), Bison sp. cf. B. crassicornis (large-horned bison), Ovis sp. cf. O. dalli (mountain sheep), Canis sp. cf. C. lupus (wolf), Lepus sp. cf. L. othus or L. arcticus (tundra hare), and Rangifer sp. (caribou). This assemblage suggests an open landscape in which trees and tall shrubs were either absent or confined to sheltered and moist sites. Camelops evidently was present in eastern Beringia during the middle Wisconsin interstadial interval but may have disappeared during the following glacial episode. The stratigraphic section at Canyon Creek appears to demonstrate that the Delta Glaciation of the north-central Alaska Range is at least in part of early Wisconsin age and was separated from the succeeding Donnelly Glaciation by an interstadial rather than interglacial episode.

  10. The down canyon evolution of submarine sediment density flows

    Science.gov (United States)

    Parsons, D. R.; Barry, J.; Clare, M. A.; Cartigny, M.; Chaffey, M. R.; Gales, J. A.; Gwiazda, R.; Maier, K. L.; McGann, M.; Paull, C. K.; O'Reilly, T. C.; Rosenberger, K. J.; Simmons, S.; Sumner, E. J.; Talling, P.; Xu, J.

    2017-12-01

    Submarine density flows, known as turbidity currents, transfer globally significant volumes of terrestrial and shelf sediments, organic carbon, nutrients and fresher-water into the deep ocean. Understanding such flows has wide implications for global organic carbon cycling, the functioning of deep-sea ecosystems, seabed infrastructure hazard assessments, and interpreting geological archives of Earth history. Only river systems transport comparable volumes of sediment over such large areas of the globe. Despite their clear importance, there are remarkably few direct measurements of these oceanic turbidity currents in action. Here we present results from the multi-institution Coordinated Canyon Experiment (CCE) which deployed multiple moorings along the axis of Monterey Canyon (offshore California). An array of six moorings, with downward looking acoustic Doppler current profilers (ADCP) were positioned along the canyon axis from 290 m to 1850 m water depth. The ADCPs reveal the internal flow structure of submarine density flows at each site. We use a novel inversion method to reconstruct the suspended sediment concentration and flow stratification field during each event. Together the six moorings provide the first ever views of the internal structural evolution of turbidity current events as they evolve down system. Across the total 18-month period of deployment at least 15 submarine sediment density flows were measured with velocities up to 8.1 m/sec, with three of these flows extending 50 kms down the canyon beyond the 1850 m water depth mooring. We use these novel data to highlight the controls on ignition, interval structure and collapse of individual events and discuss the implications for the functioning and deposits produced by these enigmatic flows.

  11. Ventilation Processes in a Three-Dimensional Street Canyon

    Czech Academy of Sciences Publication Activity Database

    Nosek, Štěpán; Kukačka, L.; Kellnerová, Radka; Jurčáková, Klára; Jaňour, Zbyněk

    2016-01-01

    Roč. 159, č. 2 (2016), s. 259-284 ISSN 0006-8314 R&D Projects: GA ČR GAP101/12/1554; GA ČR GA15-18964S Institutional support: RVO:61388998 Keywords : Coherent structures * line source * pollution flux measurements * street canyon * wind tunnel Subject RIV: BK - Fluid Dynamics Impact factor: 2.573, year: 2016

  12. LES of flow in the street canyon

    OpenAIRE

    Brechler Josef; Fuka Vladimír

    2012-01-01

    Results of computer simulation of flow over a series of street canyons are presented in this paper. The setup is adapted from an experimental study by [4] with two different shapes of buildings. The problem is simulated by an LES model CLMM (Charles University Large Eddy Microscale Model) and results are analysed using proper orthogonal decomposition and spectral analysis. The results in the channel (layout from the experiment) are compared with results with a free top boundary.

  13. LES of flow in the street canyon

    Directory of Open Access Journals (Sweden)

    Brechler Josef

    2012-04-01

    Full Text Available Results of computer simulation of flow over a series of street canyons are presented in this paper. The setup is adapted from an experimental study by [4] with two different shapes of buildings. The problem is simulated by an LES model CLMM (Charles University Large Eddy Microscale Model and results are analysed using proper orthogonal decomposition and spectral analysis. The results in the channel (layout from the experiment are compared with results with a free top boundary.

  14. Basic repository source term and data sheet report: Lavender Canyon

    International Nuclear Information System (INIS)

    1988-01-01

    This report is one of a series describing studies undertaken in support of the US Department of Energy Civilian Radioactive Waste Management (CRWM) Program. This study contains the derivation of values for environmental source terms and resources consumed for a CRWM repository. Estimates include heavy construction equipment; support equipment; shaft-sinking equipment; transportation equipment; and consumption of fuel, water, electricity, and natural gas. Data are presented for construction and operation at an assumed site in Lavender Canyon, Utah. 3 refs; 6 tabs

  15. Basic repository source term and data sheet report: Davis Canyon

    International Nuclear Information System (INIS)

    1988-01-01

    This report is one of series describing studies undertaken in support of the US Department of Energy Civilian Radioactive Waste Management (CRWM) Program. This study contains the derivation of values for environmental source terms and resources consumed for a CRWM repository. Estimates include heavy construction equipment; support equipment; shaft-sinking equipment; transportation equipment; and consumption of fuel, water electricity, and natural gas. Data are presented for construction and operation at an assumed site in Davis Canyon, Utah. 6 tabs

  16. Peaks, plateaus, canyons, and craters: The complex geometry of simple mid-domain effect models

    DEFF Research Database (Denmark)

    Colwell, Robert K.; Gotelli, Nicholas J.; Rahbek, Carsten

    2009-01-01

    dye algorithm to place assemblages of species of uniform We used a spreading dye algorithm to place assemblages of species of uniform range size in one-dimensional or two-dimensional bounded domains. In some models, we allowed dispersal to introduce range discontinuity. Results: As uniform range size...... increases from small to medium, a flat pattern of species As uniform range size increases from small to medium, a flat pattern of species richness is replaced by a pair of peripheral peaks, separated by a valley (one-dimensional models), or by a cratered ring (two-dimensional models) of species richness...... of a uniform size generate more complex patterns, including peaks, plateaus, canyons, and craters of species richness....

  17. Trees in urban street canyons and their impact on the dispersion of automobile exhausts

    OpenAIRE

    Gromke, Christof; Ruck, Bodo

    2007-01-01

    The aim of the present study is to clarify the influence of trees on the dispersion of automobile exhausts in urban street canyons. For this purpose, measurements have been performed with a small scale wind tunnel model of an idealized, isolated street canyon with model trees placed along the canyon center axis. Sulfur hexafluoride (SF6) was released from a line source embedded in the street surface, simulating vehicle exhaust emissions. The influence of various tree planting arrangements on ...

  18. THE INFLUENCE OF BUOYANCY ON FLOW AND POLLUTANT DISPERSION IN STREET CANYONS

    OpenAIRE

    Buccolieri, Riccardo; Pulvirenti, Beatrice; Di Sabatino, Silvana; Britter, Rex

    2008-01-01

    Abstract: In this paper, the effect of buoyancy on flow and pollutant dispersion within street canyons is studied by means of computational fluid dynamics simulations. We consider a neutral boundary layer approaching a 3D street canyon assuming a wind direction perpendicular to the street canyon. The Boussinesq hypothesis for incompressible fluids is chosen for modelling buoyancy. We distinguish three cases: leeward, ground and windward wall heating. Thermal effects on both the flow ...

  19. Partly standing internal tides in a dendritic submarine canyon observed by an ocean glider

    Science.gov (United States)

    Hall, Rob A.; Aslam, Tahmeena; Huvenne, Veerle A. I.

    2017-08-01

    An autonomous ocean glider is used to make the first direct measurements of internal tides within Whittard Canyon, a large, dendritic submarine canyon system that incises the Celtic Sea continental slope and a site of high benthic biodiversity. This is the first time a glider has been used for targeted observations of internal tides in a submarine canyon. Vertical isopycnal displacement observations at different stations fit a one-dimensional model of partly standing semidiurnal internal tides - comprised of a major, incident wave propagating up the canyon limbs and a minor wave reflected back down-canyon by steep, supercritical bathymetry near the canyon heads. The up-canyon internal tide energy flux in the primary study limb decreases from 9.2 to 2.0 kW m-1 over 28 km (a dissipation rate of 1 - 2.5 ×10-7 Wkg-1), comparable to elevated energy fluxes and internal tide driven mixing measured in other canyon systems. Within Whittard Canyon, enhanced mixing is inferred from collapsed temperature-salinity curves and weakened dissolved oxygen concentration gradients near the canyon heads. It has previously been hypothesised that internal tides impact benthic fauna through elevated near-bottom current velocities and particle resuspension. In support of this, we infer order 20 cm s-1 near-bottom current velocities in the canyon and observe high concentrations of suspended particulate matter. The glider observations are also used to estimate a 1 °C temperature range and 12 μmol kg-1 dissolved oxygen concentration range, experienced twice a day by organisms on the canyon walls, due to the presence of internal tides. This study highlights how a well-designed glider mission, incorporating a series of tide-resolving stations at key locations, can be used to understand internal tide dynamics in a region of complex topography, a sampling strategy that is applicable to continental shelves and slopes worldwide.

  20. Human rhinovirus capsid dynamics is controlled by canyon flexibility

    International Nuclear Information System (INIS)

    Reisdorph, Nichole; Thomas, John J.; Katpally, Umesh; Chase, Elaine; Harris, Ken; Siuzdak, Gary; Smith, Thomas J.

    2003-01-01

    Quantitative enzyme accessibility experiments using nano liquid chromatography electrospray mass spectrometry combined with limited proteolysis and isotope-labeling was used to examine the dynamic nature of the human rhinovirus (HRV) capsid in the presence of three antiviral compounds, a neutralizing Fab, and drug binding cavity mutations. Using these methods, it was found that the antivirals WIN 52084 and picovir (pleconaril) stabilized the capsid, while dansylaziridine caused destabilization. Site-directed mutations in the drug-binding cavity were found to stabilize the HRV14 capsid against proteolytic digestion in a manner similar to WIN 52084 and pleconaril. Antibodies that bind to the NIm-IA antigenic site and penetrate the canyon were also observed to protect the virion against proteolytic cleavage. These results demonstrate that quantifying the effects of antiviral ligands on protein 'breathing' can be used to compare their mode of action and efficacy. In this case, it is apparent that hydrophobic antiviral agents, antibodies, or mutations in the canyon region block viral breathing. Therefore, these studies demonstrate that mobility in the canyon region is a major determinant in capsid breathing

  1. The Influence of Roof Material on Diurnal Urban Canyon Breathing

    Science.gov (United States)

    Abuhegazy, Mohamed; Yaghoobian, Neda

    2017-11-01

    Improvements in building energy use, air quality in urban canyons and in general urban microclimates require understanding the complex interaction between urban morphology, materials, climate, and inflow conditions. Review of the literature indicates that despite a long history of valuable urban microclimate studies, more comprehensive approaches are needed to address energy, and heat and flow transport in urban areas. In this study, a more comprehensive simulation of the diurnally varying street canyon flow and associated heat transport is numerically investigated, using Large-eddy Simulation (LES). We use computational modeling to examine the impact of diurnal variation of the heat fluxes from urban surfaces on the air flow and temperature distribution in street canyons with a focus on the role of roof materials and their temperature footprints. A detailed building energy model with a three-dimensional raster-type geometry provides urban surface heat fluxes as thermal boundary conditions for the LES to determine the key aero-thermodynamic factors that affect urban street ventilation.

  2. Aquatic macroinvertebrates and water quality in Sandia Canyon

    International Nuclear Information System (INIS)

    Bennett, K.

    1994-05-01

    In 1990, field studies of water quality and stream macroinvertebrate communities were initiated in Sandia Canyon at Los Alamos National Laboratory. The studies were designed to establish baseline data and to determine the effects of routine discharges of industrial and sanitary waste. Water quality measurements were taken and aquatic macroinvertebrates sampled at three permanent stations within the canyon. Two of the three sample stations are located where the stream regularly receives industrial and sanitary waste effluents. These stations exhibited a low diversity of macroinvertebrates and slightly degraded water quality. The last sample station, located approximately 0.4 km (0.25 mi) downstream from the nearest wastewater outfall, appears to be in a zone of recovery where water quality parameters more closely resemble those found in natural streams in the Los Alamos area. A large increase in macroinvertebrate diversity was also observed at the third station. These results indicate that effluents discharged into Sandia Canyon have a marked effect on water quality and aquatic macroinvertebrate communities

  3. Survival End Points for Huntington Disease Trials Prior to a Motor Diagnosis.

    Science.gov (United States)

    Long, Jeffrey D; Mills, James A; Leavitt, Blair R; Durr, Alexandra; Roos, Raymund A; Stout, Julie C; Reilmann, Ralf; Landwehrmeyer, Bernhard; Gregory, Sarah; Scahill, Rachael I; Langbehn, Douglas R; Tabrizi, Sarah J

    2017-11-01

    Predictive genetic testing in Huntington disease (HD) enables therapeutic trials in HTT gene expansion mutation carriers prior to a motor diagnosis. Progression-free survival (PFS) is the composite of a motor diagnosis or a progression event, whichever comes first. To determine if PFS provides feasible sample sizes for trials with mutation carriers who have not yet received a motor diagnosis. This study uses data from the 2-phase, longitudinal cohort studies called Track and from a longitudinal cohort study called the Cooperative Huntington Observational Research Trial (COHORT). Track had 167 prediagnosis mutation carriers and 156 noncarriers, whereas COHORT had 366 prediagnosis mutation carriers and noncarriers. Track studies were conducted at 4 sites in 4 countries (Canada, France, England, and the Netherlands) from which data were collected from January 17, 2008, through November 17, 2014. The COHORT was conducted at 38 sites in 3 countries (Australia, Canada, and the United States) from which data were collected from February 14, 2006, through December 31, 2009. Results from the Track data were externally validated with data from the COHORT. The required sample size was estimated for a 2-arm prediagnosis clinical trial. Data analysis took place from May 1, 2016, to June 10, 2017. The primary end point is PFS. Huntington disease progression events are defined for the Unified Huntington's Disease Rating Scale total motor score, total functional capacity, symbol digit modalities test, and Stroop word test. Of Track's 167 prediagnosis mutation carriers, 93 (55.6%) were women, and the mean (SD) age was 40.06 (8.92) years; of the 156 noncarriers, 87 (55.7%) were women, and the mean (SD) age was 45.58 (10.30) years. Of the 366 COHORT participants, 229 (62.5%) were women and the mean (SD) age was 42.21 (12.48) years. The PFS curves of the Track mutation carriers showed good external validity with the COHORT mutation carriers after adjusting for initial progression. For

  4. Late Holocene earthquake history of the Brigham City segment of the Wasatch fault zone at the Hansen Canyon, Kotter Canyon, and Pearsons Canyon trench sites, Box Elder County, Utah

    Science.gov (United States)

    DuRoss, Christopher B.; Personius, Stephen F.; Crone, Anthony J.; McDonald, Greg N.; Briggs, Richard W.

    2012-01-01

    Of the five central segments of the Wasatch fault zone (WFZ) having evidence of recurrent Holocene surface-faulting earthquakes, the Brigham City segment (BCS) has the longest elapsed time since its most recent surface-faulting event (~2.1 kyr) compared to its mean recurrence time between events (~1.3 kyr). Thus, the BCS has the highest time-dependent earthquake probability of the central WFZ. We excavated trenches at three sites––the Kotter Canyon and Hansen Canyon sites on the north-central BCS and Pearsons Canyon site on the southern BCS––to determine whether a surface-faulting earthquake younger than 2.1 ka occurred on the BCS. Paleoseismic data for Hansen Canyon and Kotter Canyon confirm that the youngest earthquake on the north-central BCS occurred before 2 ka, consistent with previous north-central BCS investigations at Bowden Canyon and Box Elder Canyon. At Hansen Canyon, the most recent earthquake is constrained to 2.1–4.2 ka and had 0.6–2.5 m of vertical displacement. At Kotter Canyon, we found evidence for two events at 2.5 ± 0.3 ka and 3.5 ± 0.3 ka, with an average displacement per event of 1.9–2.3 m. Paleoseismic data from Pearsons Canyon, on the previously unstudied southern BCS, indicate that a post-2 ka earthquake ruptured this part of the segment. The Pearsons Canyon earthquake occurred at 1.2 ± 0.04 ka and had 0.1–0.8 m of vertical displacement, consistent with our observation of continuous, youthful scarps on the southern 9 km of the BCS having 1–2 m of late Holocene(?) surface offset. The 1.2-ka earthquake on the southern BCS likely represents rupture across the Weber–Brigham City segment boundary from the penultimate Weber-segment earthquake at about 1.1 ka. The Pearsons Canyon data result in a revised length of the BCS that has not ruptured since 2 ka (with time-dependent probability implications), and provide compelling evidence of at least one segment-boundary failure and multi-segment rupture on the central WFZ. Our

  5. Influence of cetane improvers on the air quality in an urban street canyon

    International Nuclear Information System (INIS)

    Huang, H.; Akutsu, Y.; Arai, M.; Tamura, M.

    2000-01-01

    The concentration distributions of NO x , PM, HC and CO in an urban street canyon have been estimated using a two-dimensional air quality numerical model based on the k-e turbulent model and the atmospheric convection diffusion equation when various cetane improvers were used in diesel fuels. A wind vortex can be found within the street canyon, and the pollutants emitted from the bottom of the street canyon tend to follow the course of the wind field, moving circularly. The addition of cetane improvers can improve the air quality in a street canyon, all of the pollutants were found to decrease with increasing cetane number. (Author)

  6. Network topology and functional connectivity disturbances precede the onset of Huntington's disease.

    Science.gov (United States)

    Harrington, Deborah L; Rubinov, Mikail; Durgerian, Sally; Mourany, Lyla; Reece, Christine; Koenig, Katherine; Bullmore, Ed; Long, Jeffrey D; Paulsen, Jane S; Rao, Stephen M

    2015-08-01

    Cognitive, motor and psychiatric changes in prodromal Huntington's disease have nurtured the emergent need for early interventions. Preventive clinical trials for Huntington's disease, however, are limited by a shortage of suitable measures that could serve as surrogate outcomes. Measures of intrinsic functional connectivity from resting-state functional magnetic resonance imaging are of keen interest. Yet recent studies suggest circumscribed abnormalities in resting-state functional magnetic resonance imaging connectivity in prodromal Huntington's disease, despite the spectrum of behavioural changes preceding a manifest diagnosis. The present study used two complementary analytical approaches to examine whole-brain resting-state functional magnetic resonance imaging connectivity in prodromal Huntington's disease. Network topology was studied using graph theory and simple functional connectivity amongst brain regions was explored using the network-based statistic. Participants consisted of gene-negative controls (n = 16) and prodromal Huntington's disease individuals (n = 48) with various stages of disease progression to examine the influence of disease burden on intrinsic connectivity. Graph theory analyses showed that global network interconnectivity approximated a random network topology as proximity to diagnosis neared and this was associated with decreased connectivity amongst highly-connected rich-club network hubs, which integrate processing from diverse brain regions. However, functional segregation within the global network (average clustering) was preserved. Functional segregation was also largely maintained at the local level, except for the notable decrease in the diversity of anterior insula intermodular-interconnections (participation coefficient), irrespective of disease burden. In contrast, network-based statistic analyses revealed patterns of weakened frontostriatal connections and strengthened frontal-posterior connections that evolved as disease

  7. Deciphering Paria and Little Colorado River flood regimes and their significance in multi-objective adaptive management strategies for Colorado River resources in Grand Canyon

    Science.gov (United States)

    Jain, S.; Topping, D. J.; Melis, T. S.

    2014-12-01

    Planning and decision processes in the Glen Canyon Dam Adaptive Management Program (GCDAMP) strive to balance numerous, often competing, objectives, such as, water supply, hydropower generation, low flow maintenance, sandbars, recreational trout angling, endangered native fish, whitewater rafting, and other sociocultural resources of Glen Canyon National Recreation Area and Grand Canyon National Park. In this context, use of monitored and predictive information on warm-season Paria River floods (JUL-OCT, at point-to-regional scales) has been identified as lead information for a new 10-year long controlled flooding experiment (termed the High-Flow Experiment Protocol) intended to determine management options for rebuilding and maintaining sandbars below Glen Canyon Dam; an adaptive strategy that can potentially facilitate improved planning and dam operations. In this work, we focus on a key concern identified by the GCDAMP, related to the timing and volume of warm season tributary sand input from the Paria River into the Colorado River in Grand Canyon National Park. The Little Colorado River is an important secondary source of sand inputs to Grand Canyon, but its lower segment is also critical spawning habitat for the endangered humpback chub. Fish biologists have reported increased abundance of chub juveniles in this key tributary in summers following cool-season flooding (DEC-FEB), but little is known about chub spawning substrates and behavior or the role that flood frequency in this tributary may play in native fish population dynamics in Grand Canyon. Episodic and intraseasonal variations (with links to equatorial and sub-tropical Pacific sea surface temperature variability) in southwest hydroclimatology are investigated to understand the magnitude, timing and spatial scales of warm- and cool-season floods from these two important tributaries of the semi-arid Colorado Plateau. Coupled variations of floods (magnitude and timing) from these rivers are also

  8. UV Radiation in an Urban Canyon in Southeast Queensland

    Science.gov (United States)

    McKinley, A. R.; Moore, M. R.; Kimlin, M. G.

    2006-12-01

    Ultraviolet radiation (UV) has the possibility to both harm and to benefit human beings when unprotected exposure occurs. After receiving small amounts of UV our bodies begin to synthesise vitamin D, which is essential for maintaining healthy bones, however excessive UV exposure can result in a variety of damaging outcomes ranging from sunburn to skin cancer and cataracts. For this reason it is very important to understand the different environments in which people encounter UV so as to better prepare the public to make smart and healthy sun exposure decisions. Each day more and more people are moving into large cities around the world and spending their time inside the urban canyon, however UV measurements are generally taken at scientific stations in open areas or on top of tall buildings, meaning that at times the environmental characteristics measured may not accurately represent those found at street-level in these highly urbanized areas. Urban canyons are home to both very tall buildings and tropospheric air pollution, each of which reduces the amount of UV reaching street-level. This study measured the varying difference between UV measurements taken at street-level and at a standard UV monitoring site on top of a building outside of the urban canyon. Investigation was conducted in the central business district (CBD) of Brisbane, Australia, which models the CBDs of large cities around the world in that it boasts a great number of tall buildings, including many skyscrapers. Data was collected under clear sky conditions at five different street-level sites in the CBD (on either side of two streets running perpendicular to one another (four sites) and in a public square) and then compared to that obtained on the same day at the Queensland University of Technology's Australian Sun and Health Research Laboratory (ASHRL), which is located 2.5 kilometres outside Brisbane's CBD. Minimum erythemal dose (MED) data was collected at each location and it was found that

  9. A randomized, double-blind, placebo-controlled study of latrepirdine in patients with mild to moderate Huntington disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth

    2013-01-01

    BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect of latrepir......BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect...... of latrepirdine on cognition and global function in patients with mild to moderate Huntington disease. DESIGN Randomized, double-blind, placebo-controlled study. SETTING Sixty-four research centers in Australia, Europe, and North America. PATIENTS Four hundred three patients with mild to moderate Huntington...... between those randomized to latrepirdine (68.5%) and placebo (68.0%). CONCLUSION In patients with mild to moderate Huntington disease and cognitive impairment, treatment with latrepirdine for 6 months was safe and well tolerated but did not improve cognition or global function relative to placebo. TRIAL...

  10. A Report Generator Volume 2

    Science.gov (United States)

    1988-01-01

    her Nicholle) ( korsakoffs (proper-noun singlp neuter) korsakoffs korekoffs) (huntingtons (proper-noun sing3p neuter) huntingtons huntingtons...hemisphere)) (sub-class (value gerstmann- syndrome 1-constructional-dyspraxia reading-coup aphasia)) (type (value lobe)) (dda (value (location...class)) (dda (value (function damage memory) (location brain))) (importance (value 10)) (damage (value 8))) ( korsakoffs (super-class (value stm)) (sub

  11. Redistributed orebodies of Poison Canyon, Sec. 18 and 19, T. 13 N., R. 9 W., McKinley County

    International Nuclear Information System (INIS)

    Tessendorf, T.N.

    1980-01-01

    Since the early 1950's, the Poison Canyon mine has been considered a classic example of uranium geology. Owing to present economic condtions, a close examination of the redistributed mineralization is taking place. Because of the evolution of the structure and geomorphology of Poison Canyon, the primary mineralization went through further oxidation and reduction. Enriched solutions of uranium migrated downdip through permeable sands. These solutions were controlled by north-trending fracture patterns, with some vertical movement along major faults. The uranium collected in structural and lithological traps, forming amoeba-like orebodies with the higher grade mineralization located in the fractures. First-generation redistributed ore is primarily coffinite. Forming later is second-generation redistributed ore, which is mainly tyuyamunite. The latter formed from further oxidation and redistribution of the primary and first-generation mineralization, combined with an increasing nearness to surface. The authigenic minerals in the redistributed mineralization are found in carbon-deficient sands. The redistributed minerals are locally associated with pascoite, although this mineral is rare. The radiometric equilibrium of the primary minerals differs from that of the redistributed minerals. The uranium has been leached from the primary minerals making chemical values less than radiometric values. The redistributed minerals are chemically greater than radiometric, producing a favorable equilibrium. The percent extraction in the mill process is greater for the redistributed ore than for the primary ore. The paragenetic position of the different minerals has a direct bearing on these observations

  12. Fractal properties of fractured sandstones of the Guartela Canyon, Parana Basin - Brazil; Propriedades fractais de arenitos fraturados do Canyon Guartela, Formacao Furnas, Bacia do Parana

    Energy Technology Data Exchange (ETDEWEB)

    Souza, Jeferson de; Figueira, Isabela Francoso Rebutini; Santos, Thais Borba [Universidade Federal do Rio Grande do Norte (PPGG/DG/UFRN), Natal (Brazil). Dept. de Geologia. Programa de Pos-Graducao em Geologia; Rostirolla, Sidnei Pires [Universidade Federal do Rio Grande do Norte (DG/UFRN), Natal (Brazil). Dept. de Geologia; Pierin, Andre Ramiro; Spisila, Andre Luis [Universidade Federal do Rio Grande do Norte (DG/UFRN), Natal (Brazil). Dept. de Geologia. Programa de Iniciacao Cientifica

    2008-03-15

    The statistical and geometrical properties of fracture systems were obtained by analyzing remote sense images and outcrop data, in the Region of Guartela Canyon, in the central-eastern of Parana State. The probability distributions of fractures, with their parameters and attributes, were obtained through extensive statistical exploration of data. These parameters were used as input data for generating 3-D stochastic fractures models through the 'discrete fracture network - DFN' method. The modeling is performed by using the code FRED. To study the persistence of statistical parameters in multiple scales were used remote sensing images (SRTM, Landsat TM7 and aerial photos), covering a scale range from outcrops (few meters) to basin scales (hundreds of kilometers). The results indicated the presence of power-law (fractal) statistics for the spatial and size distributions. Fractals distributions were found for all sets studied, in some cases with different fractal exponents. The implications of fractal behavior for the generation of discrete fracture network, and consequently for the hydraulic properties, are briefly discussed. (author)

  13. Measured and modelled concentrations and vertical profiles of airborne particulate matter within the boundary layer of a street canyon

    International Nuclear Information System (INIS)

    Colls, J.J.; Micallef, A.

    1999-01-01

    Concentrations and vertical profiles of various fractions of airborne particulate matter (suspended particulate matter (SPM), PM 10 and PM 2.5 ) have been measured over the first three metres from ground in a street canyon. Measurements were carried out using automated near real-time apparatus called the Kinetic Sequential Sampling (KSS) system. KSS system is essentially an electronically-controlled lift carrying a real-time particle monitor for sampling air sequentially, at different heights within the breathing zone, which includes all heights within the surface layer of a street canyon at which people may breathe. Data is automatically logged at the different receptor levels, for the determination of the average vertical concentration profile of airborne particulate matter. For measuring the airborne particle concentration, a Grimm Dust Monitor 1.104/5 was used. The recorded data also allows for time series analysis of airborne particulate matter concentration at different heights. Time series data and hourly-average vertical concentration profiles in the boundary layer of the confines of a street are thought to be mainly determined by traffic emissions and traffic associated processes. Hence the measured data were compared with results of a street canyon emission-dispersion model in time and space. This Street Level Air Quality (SLAQ) model employs the plume-box technique and includes modules for simulating vehicle-generated effects such as thermally- and mechanically-generated turbulence and resuspension of road dust. Environmental processes, such as turbulence resulting from surface sensible heat and the formation of sulphate aerosol from sulphur dioxide exhaust emissions, are taken into account. The paper presents an outline description of the measuring technique and model used, and a comparison of the measured and modelled data

  14. Inventory and Evaluation of Cultural Resources, Bolsa Chica Mesa and Huntington Beach Mesa, Orange County, California

    Science.gov (United States)

    1989-09-30

    Excelentisimo Conde de Monterey, Virrey Que Era dela Nueva Espana. In Monarchia Indiana, edited by J. de Torquemada, pp. 693-725. Madrid. 101 102 Baumhoff, M...biological bacterias , this includes the destruction of canyons, hills, mountains and the flora and fauna in these areas. Road construction, real

  15. Modelling Black Carbon concentrations in two busy street canyons in Brussels using CANSBC

    Science.gov (United States)

    Brasseur, O.; Declerck, P.; Heene, B.; Vanderstraeten, P.

    2015-01-01

    This paper focused on modelling Black Carbon (BC) concentrations in two busy street canyons, the Crown and Belliard Street in Brussels. The used original Operational Street Pollution Model was adapted to BC by eliminating the chemical module and is noted here as CANSBC. Model validations were performed using temporal BC data from the fixed measurement network in Brussels. Subsequently, BC emissions were adjusted so that simulated BC concentrations equalled the observed ones, averaged over the whole period of simulation. Direct validations were performed for the Crown Street, while BC model calculations for the Belliard Street were validated indirectly using the linear relationship between BC and NOx. Concerning the Crown Street, simulated and observed half-hourly BC concentrations correlated well (r = 0.74) for the period from July 1st, 2011 till June 30th, 2013. In particular, CANSBC performed very well to simulate the monthly and diurnal evolutions of averaged BC concentrations, as well as the difference between weekdays and weekends. This means that the model correctly handled the meteorological conditions as well as the variation in traffic emissions. Considering dispersion, it should however be noted that BC concentrations are better simulated under stable than under unstable conditions. Even if the correlation on half-hourly NOx concentrations was slightly lower (r = 0.60) than the one of BC, indirect validations of CANSBC for the Belliard Street yielded comparable results and conclusions as described above for the Crown Street. Based on our results, it can be stated that CANSBC is suitable to accurately simulate BC concentrations in the street canyons of Brussels, under the following conditions: (i) accurate vehicle counting data is available to correctly estimate traffic emissions, and (ii) vehicle speeds are measured in order to improve emission estimates and to take into account the impact of the turbulence generated by moving vehicles on the local

  16. 77 FR 59607 - Black Canyon Hydro, LLC; Notice of Environmental Site Review

    Science.gov (United States)

    2012-09-28

    ... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Project No. 14110-001] Black Canyon Hydro, LLC; Notice of Environmental Site Review On Wednesday, October 3, 2012, at 3 p.m., Commission staff will be participating in an environmental site review for the proposed Black Canyon Hydroelectric Project. All interested participants should mee...

  17. 75 FR 10308 - Fire Management Plan, Final Environmental Impact Statement, Record of Decision, Grand Canyon...

    Science.gov (United States)

    2010-03-05

    ... DEPARTMENT OF THE INTERIOR National Park Service Fire Management Plan, Final Environmental Impact... Statement for the Fire Management Plan, Grand Canyon National Park. SUMMARY: Pursuant to the National... the Record of Decision for the Fire Management Plan, Grand Canyon National Park, Arizona. On January...

  18. Effects of trees on the dilution of vehicle exhaust emissions in urban street canyons

    NARCIS (Netherlands)

    Gromke, C.B.; Ruck, B.

    2009-01-01

    In order to investigate the natural ventilation and air quality of urban street canyons with trees, boundary layer wind tunnel studies at a small-scale model have been performed. Concentrations in street canyons with a tracer gas emitting line source at the ground level and one row of trees arranged

  19. Strategic guidelines for street canyon geometry to achieve sustainable street air quality

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Andy T.; So, Ellen S.P.; Samad, Subash C. [Hong Kong Univ., Dept. of Mechanical Engineering, Hong Kong (China)

    2001-08-01

    This paper is concerned with the motion of air within the urban street canyon and is directed towards a deeper understanding of pollutant dispersion with respect to various simple canyon geometries and source positions. Taking into account the present days typical urban configurations, three principal flow regimes 'isolated roughness flow', 'skimming flow' and 'wake interference flow' (Boundary Layer Climates, 2nd edition, Methuen, London) and their corresponding pollutant dispersion characteristics are studied for various canopies aspect ratios, namely relative height (h{sub 2}/H{sub 1}), canyon height to width ratio (h/w) and canyon length to height ratio (l/h). A field-size canyon has been analysed through numerical simulations using the standard k-{sup {epsilon}} turbulence closure model. It is found that the pollutant transport and diffusion is strongly dependent upon the type of flow regime inside the canyon and exchange between canyon and the above roof air. Some rules of thumbs have been established to get urban canyon geometries for efficient dispersion of pollutants. (Author)

  20. Captured in Stone: Women in the Rock Art of Canyon de Chelly.

    Science.gov (United States)

    Travis, Tara

    1997-01-01

    Describes the pictographs (painted images on stone) and petroglyphs (pecked images on stone) found in the Canyon de Chelly National Monument in Arizona. Canyon de Chelly includes one of the largest concentrations of American Indian rock art in the southwest. Discusses the depiction of women in these images. (MJP)

  1. 33 CFR 165.1155 - Security Zone; Diablo Canyon Nuclear Power Plant, Avila Beach, California.

    Science.gov (United States)

    2010-07-01

    ... Nuclear Power Plant, Avila Beach, California. 165.1155 Section 165.1155 Navigation and Navigable Waters... Coast Guard District § 165.1155 Security Zone; Diablo Canyon Nuclear Power Plant, Avila Beach... surface to bottom, within a 2,000 yard radius of Diablo Canyon Nuclear Power Plant centered at position 35...

  2. Draft environmental assessment: Lavender Canyon site, Utah. Nuclear Waste Policy Act (Section 112)

    International Nuclear Information System (INIS)

    1984-12-01

    In February 1983, the US Department of Energy (DOE) identified the Lavender Canyon site in Utah, as one of nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Lavender Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for Nuclear Waste Repositories. These evaluations are reported in this draft environmental assessment (EA), which is being issued for public review and comment. The DOE findings and determinations that are based on these evaluations are preliminary and subject to public review and comment. A final EA will be prepared after considering the comments received. On the basis of the evaluations contained in this draft EA, the DOE has found that the Lavender Canyon site is not disqualified under the guidelines. The site is contained in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. This setting contains one other potentially acceptable site - the Davis Canyon site. Although the Lavender Canyon site appears to be suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. On the basis of these findings, the DOE is proposing to nominate the Davis Canyon site rather than the Lavender Canyon site as one of the five sites suitable for characterization

  3. Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study

    Science.gov (United States)

    Piano, Carla; Losurdo, Anna; Della Marca, Giacomo; Solito, Marcella; Calandra-Buonaura, Giovanna; Provini, Federica; Bentivoglio, Anna Rita; Cortelli, Pietro

    2015-01-01

    Study Objectives: To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD). Design: Cross-sectional cohort study. Setting: Sleep laboratory. Patients: Thirty HD patients, 16 women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y). Interventions: Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale [UHDMRS]), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG). Measurements and Results: The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m2. No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P Marca G, Solito M, Calandra-Buonaura G, Provini F, Bentivoglio AR, Cortelli P. Polysomnographic findings and clinical correlates in Huntington disease: a cross-sectional cohort study. SLEEP 2015;38(9):1489–1495. PMID:25845698

  4. Nonparametric estimation for censored mixture data with application to the Cooperative Huntington's Observational Research Trial.

    Science.gov (United States)

    Wang, Yuanjia; Garcia, Tanya P; Ma, Yanyuan

    2012-01-01

    This work presents methods for estimating genotype-specific distributions from genetic epidemiology studies where the event times are subject to right censoring, the genotypes are not directly observed, and the data arise from a mixture of scientifically meaningful subpopulations. Examples of such studies include kin-cohort studies and quantitative trait locus (QTL) studies. Current methods for analyzing censored mixture data include two types of nonparametric maximum likelihood estimators (NPMLEs) which do not make parametric assumptions on the genotype-specific density functions. Although both NPMLEs are commonly used, we show that one is inefficient and the other inconsistent. To overcome these deficiencies, we propose three classes of consistent nonparametric estimators which do not assume parametric density models and are easy to implement. They are based on the inverse probability weighting (IPW), augmented IPW (AIPW), and nonparametric imputation (IMP). The AIPW achieves the efficiency bound without additional modeling assumptions. Extensive simulation experiments demonstrate satisfactory performance of these estimators even when the data are heavily censored. We apply these estimators to the Cooperative Huntington's Observational Research Trial (COHORT), and provide age-specific estimates of the effect of mutation in the Huntington gene on mortality using a sample of family members. The close approximation of the estimated non-carrier survival rates to that of the U.S. population indicates small ascertainment bias in the COHORT family sample. Our analyses underscore an elevated risk of death in Huntington gene mutation carriers compared to non-carriers for a wide age range, and suggest that the mutation equally affects survival rates in both genders. The estimated survival rates are useful in genetic counseling for providing guidelines on interpreting the risk of death associated with a positive genetic testing, and in facilitating future subjects at risk

  5. Current status of PET imaging in Huntington's disease

    International Nuclear Information System (INIS)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios

    2016-01-01

    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading ''Huntington Disease'' combined with text and key words ''Huntington Disease'', ''Neuroimaging'' and ''PET''. Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([ 18 F]FDG and [ 15 O]H 2 O), presynaptic ([ 18 F]fluorodopa, [ 11 C]β-CIT and [ 11 C]DTBZ) and postsynaptic ([ 11 C]SCH22390, [ 11 C]FLB457 and [ 11 C]raclopride) dopaminergic function, phosphodiesterases ([ 18 F]JNJ42259152, [ 18 F]MNI-659 and [ 11 C]IMA107), and adenosine ([ 18 F]CPFPX), cannabinoid ([ 18 F]MK-9470), opioid ([ 11 C]diprenorphine) and GABA ([ 11 C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed for monitoring potential neuroprotective and preventive treatment

  6. Short G-rich oligonucleotides as a potential therapeutic for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Parekh-Olmedo Hetal

    2006-10-01

    Full Text Available Abstract Background Huntington's Disease (HD is an inherited autosomal dominant genetic disorder in which neuronal tissue degenerates. The pathogenesis of the disease appears to center on the development of protein aggregates that arise initially from the misfolding of the mutant HD protein. Mutant huntingtin (Htt is produced by HD genes that contain an increased number of glutamine codons within the first exon and this expansion leads to the production of a protein that misfolds. Recent studies suggest that mutant Htt can nucleate protein aggregation and interfere with a multitude of normal cellular functions. Results As such, efforts to find a therapy for HD have focused on agents that disrupt or block the mutant Htt aggregation pathway. Here, we report that short guanosine monotonic oligonucleotides capable of adopting a G-quartet structure, are effective inhibitors of aggregation. By utilizing a biochemical/immunoblotting assay as an initial screen, we identified a 20-mer, all G-oligonucleotide (HDG as an active molecule. Subsequent testing in a cell-based assay revealed that HDG was an effective inhibitor of aggregation of a fusion protein, comprised of a mutant Htt fragment and green fluorescent protein (eGFP. Taken together, our results suggest that a monotonic G-oligonucleotide, capable of adopting a G-quartet conformation is an effective inhibitor of aggregation. This oligonucleotide can also enable cell survival in PC12 cells overexpressing a mutant Htt fragment fusion gene. Conclusion Single-stranded DNA oligonucleotides capable of forming stable G-quartets can inhibit aggregation of the mutant Htt fragment protein. This activity maybe an important part of the pathogenecity of Huntington's Disease. Our results reveal a new class of agents that could be developed as a therapeutic approach for Huntington's Disease.

  7. 75 FR 26788 - Public Land Order No. 7742; Withdrawal of Public Land for the Manning Canyon Tailings Repository; UT

    Science.gov (United States)

    2010-05-12

    ... 79765] Public Land Order No. 7742; Withdrawal of Public Land for the Manning Canyon Tailings Repository... period of 5 years to protect the integrity of the Manning Canyon Tailings Repository and surrounding... withdrawal is to protect public health and safety and the Federal investment in the Manning Canyon Tailings...

  8. 76 FR 14745 - Notice To Rescind a Notice of Intent To Prepare an Environmental Impact Statement, Ada and Canyon...

    Science.gov (United States)

    2011-03-17

    ... To Prepare an Environmental Impact Statement, Ada and Canyon Counties, ID AGENCY: Federal Highway... prepare an Environmental Impact Statement for a proposed highway project in Ada and Canyon County, Idaho... Highway 44 from Exit 25 at Interstate 84 in Canyon County to Ballantyne Lane in Ada County. The project is...

  9. Aerodynamic effects of trees on pollutant concentration in street canyons.

    Science.gov (United States)

    Buccolieri, Riccardo; Gromke, Christof; Di Sabatino, Silvana; Ruck, Bodo

    2009-09-15

    This paper deals with aerodynamic effects of avenue-like tree planting on flow and traffic-originated pollutant dispersion in urban street canyons by means of wind tunnel experiments and numerical simulations. Several parameters affecting pedestrian level concentration are investigated, namely plant morphology, positioning and arrangement. We extend our previous work in this novel aspect of research to new configurations which comprise tree planting of different crown porosity and stand density, planted in two rows within a canyon of street width to building height ratio W/H=2 with perpendicular approaching wind. Sulfur hexafluoride was used as tracer gas to model the traffic emissions. Complementary to wind tunnel experiments, 3D numerical simulations were performed with the Computational Fluid Dynamics (CFD) code FLUENT using a Reynolds Stress turbulence closure for flow and the advection-diffusion method for concentration calculations. In the presence of trees, both measurements and simulations showed considerable larger pollutant concentrations near the leeward wall and slightly lower concentrations near the windward wall in comparison with the tree-less case. Tree stand density and crown porosity were found to be of minor importance in affecting pollutant concentration. On the other hand, the analysis indicated that W/H is a more crucial parameter. The larger the value of W/H the smaller is the effect of trees on pedestrian level concentration regardless of tree morphology and arrangement. A preliminary analysis of approaching flow velocities showed that at low wind speed the effect of trees on concentrations is worst than at higher speed. The investigations carried out in this work allowed us to set up an appropriate CFD modelling methodology for the study of the aerodynamic effects of tree planting in street canyons. The results obtained can be used by city planners for the design of tree planting in the urban environment with regard to air quality issues.

  10. Marble Canyon 10 x 20 NTMS area Arizona: data report

    International Nuclear Information System (INIS)

    Heffner, J.D.

    1980-07-01

    Results of ground water and stream/surface sediment reconnaissance (HSSR) in the National Topographic Map Series (NTMS) Marble Canyon 1 0 x 2 0 quadrangle are presented. The target sampling density for all media collected was one site per 12 square kilometers. This resulted in 884 sediment samples being collected; however, dry conditions and sparse population resulted in the collection of only 2 ground water samples. Grand Canyon National Park, Glen Canyon National Recreation Area, and much Indian tribal land in the southern half of the quadrangle were not sampled. Neutron activation analysis (NAA) results are given for uranium and 16 other elements in sediments, and for uranium and 9 other elements in ground water. Mass spectrometry results are given for helium in ground water. Field measurements for sediment samples are presented in tables and maps. Statistical summaries of data and a brief description of results are given. A generalized geologic map and a summary of the geology of the area are included. Data from ground water include: water chemistry measurements (pH, conductivity, and alkalinity); physical measurements (water temperature, and scintillometer readings); and elemental analyses (U, Al, Br, Cl, Dy, F, He, Mg, Mn, Na, and V). Data from sediment sites include: water chemistry measurements (where available) for pH, conductivity, and alkalinity; and elemental analyses(U, Th, Hf, Al, Ce, Dy, Eu, Fe, La, Lu, Mn, Sc, Sm, Na, Ti, V, and Yb). Sample site descriptors (stream characteristics, vegetation, etc.) are also tabulated. Histograms, cumulative frequency, and areal distribution plots for most elements; Log U/Th, Log U/Hf, and Log U/(Th + Hf) ratios; and scintillometer readings are included

  11. Psychiatric and cognitive symptoms in Huntington's disease are modified by polymorphisms in catecholamine regulating enzyme genes

    DEFF Research Database (Denmark)

    Vinther-Jensen, T; Nielsen, Troels Tolstrup; Budtz-Jørgensen, E

    2016-01-01

    Huntington's disease (HD) is an autosomal dominantly inherited neurodegenerative disorder characterized by motor, psychiatric, and cognitive manifestations. HD is caused by a CAG repeat expansion in the Huntingtin (HTT) gene but the exact pathogenesis remains unknown. Dopamine imbalance has......-described cohort of Danish HD gene-expansion carriers. We show that cognitive impairment and psychiatric symptoms in HD are modified by polymorphisms in the monoamine oxidase A (MAOA) and catechol-O-methyltransferase (COMT) genes and by the 4p16.3 B haplotype. These results support the theory of dopamine imbalance...

  12. Derivation of Huntington Disease affected Genea046 human embryonic stem cell line

    Directory of Open Access Journals (Sweden)

    Biljana Dumevska

    2016-03-01

    Full Text Available The Genea046 human embryonic stem cell line was derived from a donated, fully commercially consented ART blastocyst, carrying HTT gene CAG expansion of 45 repeats, indicative of Huntington Disease. Following ICM outgrowth on inactivated human feeders, karyotype was confirmed as 46, XX by CGH and STR analysis demonstrated a female Allele pattern. The hESC line had pluripotent cell morphology, 85% of cells expressed Nanog, 92% Oct4, 75% Tra1–60 and 99% SSEA4 and demonstrated Alkaline Phosphatase activity. The cell line was negative for Mycoplasma and visible contamination.

  13. El papel de la terapia ocupacional en la enfermedad de Huntington

    OpenAIRE

    Hernández Lozano, David; Fernández Hawrylak, María; Grau Rubio, Claudia

    2014-01-01

    se analiza el papel del terapeuta ocupacional en el trabajo con las personas afectadas por la enfermedad de Huntington. se realizó una investigación por objetivos a través del diseño y puesta en práctica de un programa de intervención encaminado a mitigar el deterioro producido por la enfermedad mediante el entrenamiento en actividades de la vida diaria y de estimulación cognitiva, y a desarrollar competencias en la familia. la investigación se desarrolló en la Asociación de corea de Huntingt...

  14. Assessing and Modulating Kynurenine Pathway Dynamics in Huntington's Disease: Focus on Kynurenine 3-Monooxygenase.

    Science.gov (United States)

    Sathyasaikumar, Korrapati V; Breda, Carlo; Schwarcz, Robert; Giorgini, Flaviano

    2018-01-01

    The link between disturbances in kynurenine pathway (KP) metabolism and Huntington's disease (HD) pathogenesis has been explored for a number of years. Several novel genetic and pharmacological tools have recently been developed to modulate key regulatory steps in the KP such as the reaction catalyzed by the enzyme kynurenine 3-monooxygenase (KMO). This insight has offered new options for exploring the mechanistic link between this metabolic pathway and HD, and provided novel opportunities for the development of candidate drug-like compounds. Here, we present an overview of the field, focusing on some novel approaches for interrogating the pathway experimentally.

  15. A Case of Juvenile Huntington Disease in a 6-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Jun-Sang Sunwoo

    2010-10-01

    Full Text Available Huntington disease is a neurodegenerative disorder distinguished by the triad of dominant inheritance, choreoathetosis and dementia, usually with onset in the fourth and fifth decades. It is caused by an unstable cytosine-adenine-guanine (CAG trinucleotide repeat expansion in the gene IT15 in locus 4p16.3. Juvenile HD that constitutes about 3% to 10% of all patients is clinically different from adult-onset form and characterized by a larger number of CAG repeats typically exceeding 60. We report a case of a 6-year-old boy with myoclonic seizure and 140 CAG repeats confirmed by molecular genetic analysis.

  16. Populating a Control Point Database: A cooperative effort between the USGS, Grand Canyon Monitoring and Research Center and the Grand Canyon Youth Organization

    Science.gov (United States)

    Brown, K. M.; Fritzinger, C.; Wharton, E.

    2004-12-01

    The Grand Canyon Monitoring and Research Center measures the effects of Glen Canyon Dam operations on the resources along the Colorado River from Glen Canyon Dam to Lake Mead in support of the Grand Canyon Adaptive Management Program. Control points are integral for geo-referencing the myriad of data collected in the Grand Canyon including aerial photography, topographic and bathymetric data used for classification and change-detection analysis of physical, biologic and cultural resources. The survey department has compiled a list of 870 control points installed by various organizations needing to establish a consistent reference for data collected at field sites along the 240 mile stretch of Colorado River in the Grand Canyon. This list is the foundation for the Control Point Database established primarily for researchers, to locate control points and independently geo-reference collected field data. The database has the potential to be a valuable mapping tool for assisting researchers to easily locate a control point and reduce the occurrance of unknowingly installing new control points within close proximity of an existing control point. The database is missing photographs and accurate site description information. Current site descriptions do not accurately define the location of the point but refer to the project that used the point, or some other interesting fact associated with the point. The Grand Canyon Monitoring and Research Center (GCMRC) resolved this problem by turning the data collection effort into an educational exercise for the participants of the Grand Canyon Youth organization. Grand Canyon Youth is a non-profit organization providing experiential education for middle and high school aged youth. GCMRC and the Grand Canyon Youth formed a partnership where GCMRC provided the logistical support, equipment, and training to conduct the field work, and the Grand Canyon Youth provided the time and personnel to complete the field work. Two data

  17. Ancho Canyon RF Collect, March 2, 2017: Final Report

    Energy Technology Data Exchange (ETDEWEB)

    Junor, William [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Layne, John Preston [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Gamble, Thomas Kirk [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Quintana, Bobby Arthur [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Snelson-Gerlicher, Catherine Mary [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Goorley, John Timothy [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2017-09-21

    We report the results from the March 2, 2017, Ancho Canyon RF collection. While bright electromagnetic signals were seen nearby the firing point, there were no detections of signals from the explosively-fired fuse at a collection point about 570m distant on the East Mesa. However, "liveness" tests of the East Mesa data acquisition system and checks of the timing both suggest that the collection system was working correctly. We examine possible reasons for the lack of detection. Principal among these is that the impulsive signal may be small compared to the radio frequency background on the East Mesa.

  18. Late Holocene sea ice conditions in Herald Canyon, Chukchi Sea

    Science.gov (United States)

    Pearce, C.; O'Regan, M.; Rattray, J. E.; Hutchinson, D. K.; Cronin, T. M.; Gemery, L.; Barrientos, N.; Coxall, H.; Smittenberg, R.; Semiletov, I. P.; Jakobsson, M.

    2017-12-01

    Sea ice in the Arctic Ocean has been in steady decline in recent decades and, based on satellite data, the retreat is most pronounced in the Chukchi and Beaufort seas. Historical observations suggest that the recent changes were unprecedented during the last 150 years, but for a longer time perspective, we rely on the geological record. For this study, we analyzed sediment samples from two piston cores from Herald Canyon in the Chukchi Sea, collected during the 2014 SWERUS-C3 Arctic Ocean Expedition. The Herald Canyon is a local depression across the Chukchi Shelf, and acts as one of the main pathways for Pacific Water to the Arctic Ocean after entering through the narrow and shallow Bering Strait. The study site lies at the modern-day seasonal sea ice minimum edge, and is thus an ideal location for the reconstruction of past sea ice variability. Both sediment cores contain late Holocene deposits characterized by high sediment accumulation rates (100-300 cm/kyr). Core 2-PC1 from the shallow canyon flank (57 m water depth) is 8 meter long and extends back to 4200 cal yrs BP, while the upper 3 meters of Core 4-PC1 from the central canyon (120 mwd) cover the last 3000 years. The chronologies of the cores are based on radiocarbon dates and the 3.6 ka Aniakchak CFE II tephra, which is used as an absolute age marker to calculate the marine radiocarbon reservoir age. Analysis of biomarkers for sea ice and surface water productivity indicate stable sea ice conditions throughout the entire late Holocene, ending with an abrupt increase of phytoplankton sterols in the very top of both sediment sequences. The shift is accompanied by a sudden increase in coarse sediments (> 125 µm) and a minor change in δ13Corg. We interpret this transition in the top sediments as a community turnover in primary producers from sea ice to open water biota. Most importantly, our results indicate that the ongoing rapid ice retreat in the Chukchi Sea of recent decades was unprecedented during the

  19. Impact of roof height non-uniformity on pollutant transport between a street canyon and intersections.

    Science.gov (United States)

    Nosek, Štěpán; Kukačka, Libor; Jurčáková, Klára; Kellnerová, Radka; Jaňour, Zbyněk

    2017-08-01

    This paper presents an extension of our previous wind-tunnel study (Nosek et al., 2016) in which we highlighted the need for investigation of the removal mechanisms of traffic pollution from all openings of a 3D street canyon. The extension represents the pollution flux (turbulent and advective) measurements at the lateral openings of three different 3D street canyons for the winds perpendicular and oblique to the along-canyon axis. The pollution was simulated by emitting a passive gas (ethane) from a homogeneous ground-level line source positioned along the centreline of the investigated street canyons. The street canyons were formed by courtyard-type buildings of two different regular urban-array models. The first model has a uniform building roof height, while the second model has a non-uniform roof height along each building's wall. The mean flow and concentration fields at the canyons' lateral openings confirm the findings of other studies that the buildings' roof-height variability at the intersections plays an important role in the dispersion of the traffic pollutants within the canyons. For the perpendicular wind, the non-uniform roof-height canyon appreciably removes or entrains the pollutant through its lateral openings, contrary to the uniform canyon, where the pollutant was removed primarily through the top. The analysis of the turbulent mass transport revealed that the coherent flow structures of the lateral momentum transport correlate with the ventilation processes at the lateral openings of all studied canyons. These flow structures coincide at the same areas and hence simultaneously transport the pollutant in opposite directions. Copyright © 2017 Elsevier Ltd. All rights reserved.

  20. Following the canyon to reach and remove olfactory groove meningiomas.

    Science.gov (United States)

    Stefini, Roberto; Zenga, Francesco; Giacomo, Esposito; Bolzoni, Andrea; Tartara, Fulvio; Spena, Giannantonio; Ambrosi, Claudia; Fontanella, Marco M

    2017-04-01

    Olfactory groove meningiomas (OGMs) represent approximately 10% of all intracranial meningiomas. They arise in the olfactory fossa, a variable depression delimited by the lateral lamella and perpendicular plate. The cribriform plate with the lateral lamella and ethmoidal and orbital roof could be viewed as a 'canyon' with the frontal sinus as the main entrance. Between January 2000 and December 2013, 32 consecutive patients underwent removal of OGMs through this 'canyon' at the Department of Neurosurgery of Brescia and Turin. Complete removal was achieved in all patients with this trans-frontal sinus subcranial approach (Simpson grade I; mean lesion volume, 46.6 cm3). Five patients (15.6%) experienced nasal CSF leakage, treated with external lumbar drain positioning for 4 days and resolved in all cases but one, which was re-operated. Two patients (6.2%) during the CSF leakage experienced meningitis at day 7 after surgery, both successfully treated by intravenous antibiotic therapy. After one month, one patient developed hydrocephalus, treated with a ventricular peritoneal shunt. In one patient, traction on the OGM caused bleeding of the callosomarginal artery, which was coagulated with superior frontal gyrus ischemia without neurological consequences. Glasgow Outcome Scale Score at 6 months was V in 29 patients, IV in one patient, and I in two patients. Advantages with this approach may include easy and early control of blood supply from its insertion in the skull base, minimal frontal lobe retraction, preservation of the frontal veins draining to the sagittal sinus, and a satisfactory aesthetic outcome.

  1. An Improved Simulation of the Diurnally Varying Street Canyon Flow

    Science.gov (United States)

    Yaghoobian, Neda; Kleissl, Jan; Paw U, Kyaw Tha

    2012-11-01

    The impact of diurnal variation of temperature distribution over building and ground surfaces on the wind flow and scalar transport in street canyons is numerically investigated using the PArallelized LES Model (PALM). The Temperature of Urban Facets Indoor-Outdoor Building Energy Simulator (TUF-IOBES) is used for predicting urban surface heat fluxes as boundary conditions for a modified version of PALM. TUF-IOBES dynamically simulates indoor and outdoor building surface temperatures and heat fluxes in an urban area taking into account weather conditions, indoor heat sources, building and urban material properties, composition of the building envelope (e.g. windows, insulation), and HVAC equipment. Temperature (and heat flux) distribution over urban surfaces of the 3-D raster-type geometry of TUF-IOBES makes it possible to provide realistic, high resolution boundary conditions for the numerical simulation of flow and scalar transport in an urban canopy. Compared to some previous analyses using uniformly distributed thermal forcing associated with urban surfaces, the present analysis shows that resolving non-uniform thermal forcings can provide more detailed and realistic patterns of the local air flow and pollutant dispersion in urban canyons.

  2. Geologic map of the Paintbrush Canyon Area, Yucca Mountain, Nevada

    International Nuclear Information System (INIS)

    Dickerson, R.P.; Drake, R.M. II

    1998-01-01

    This geologic map is produced to support site characterization studies of Yucca Mountain, Nevada, site of a potential nuclear waste storage facility. The area encompassed by this map lies between Yucca Wash and Fortymile Canyon, northeast of Yucca Mountain. It is on the southern flank of the Timber Mountain caldera complex within the southwest Nevada volcanic field. Miocene tuffs and lavas of the Calico Hills Formation, the Paintbrush Group, and the Timber Mountain Group crop out in the area of this map. The source vents of the tuff cones and lava domes commonly are located beneath the thickest deposits of pyroclastic ejecta and lava flows. The rocks within the mapped area have been deformed by north- and northwest-striking, dominantly west-dipping normal faults and a few east-dipping normal faults. Faults commonly are characterized by well developed fault scarps, thick breccia zones, and hanging-wall grabens. Latest movement as preserved by slickensides on west-dipping fault scarps is oblique down towards the southwest. Two of these faults, the Paintbrush Canyon fault and the Bow Ridge fault, are major block-bounding faults here and to the south at Yucca Mountain. Offset of stratigraphic units across faults indicates that faulting occurred throughout the time these volcanic units were deposited

  3. Geologic map of the Paintbrush Canyon Area, Yucca Mountain, Nevada

    Energy Technology Data Exchange (ETDEWEB)

    Dickerson, R.P. [Geological Survey, Denver, CO (United States); Drake, R.M. II [Pacific Western Technologies, Ltd., Lakewood, CO (United States)

    1998-11-01

    This geologic map is produced to support site characterization studies of Yucca Mountain, Nevada, site of a potential nuclear waste storage facility. The area encompassed by this map lies between Yucca Wash and Fortymile Canyon, northeast of Yucca Mountain. It is on the southern flank of the Timber Mountain caldera complex within the southwest Nevada volcanic field. Miocene tuffs and lavas of the Calico Hills Formation, the Paintbrush Group, and the Timber Mountain Group crop out in the area of this map. The source vents of the tuff cones and lava domes commonly are located beneath the thickest deposits of pyroclastic ejecta and lava flows. The rocks within the mapped area have been deformed by north- and northwest-striking, dominantly west-dipping normal faults and a few east-dipping normal faults. Faults commonly are characterized by well developed fault scarps, thick breccia zones, and hanging-wall grabens. Latest movement as preserved by slickensides on west-dipping fault scarps is oblique down towards the southwest. Two of these faults, the Paintbrush Canyon fault and the Bow Ridge fault, are major block-bounding faults here and to the south at Yucca Mountain. Offset of stratigraphic units across faults indicates that faulting occurred throughout the time these volcanic units were deposited.

  4. Predictive Temperature Equations for Three Sites at the Grand Canyon

    Science.gov (United States)

    McLaughlin, Katrina Marie Neitzel

    Climate data collected at a number of automated weather stations were used to create a series of predictive equations spanning from December 2009 to May 2010 in order to better predict the temperatures along hiking trails within the Grand Canyon. The central focus of this project is how atmospheric variables interact and can be combined to predict the weather in the Grand Canyon at the Indian Gardens, Phantom Ranch, and Bright Angel sites. Through the use of statistical analysis software and data regression, predictive equations were determined. The predictive equations are simple or multivariable best fits that reflect the curvilinear nature of the data. With data analysis software curves resulting from the predictive equations were plotted along with the observed data. Each equation's reduced chi2 was determined to aid the visual examination of the predictive equations' ability to reproduce the observed data. From this information an equation or pair of equations was determined to be the best of the predictive equations. Although a best predictive equation for each month and season was determined for each site, future work may refine equations to result in a more accurate predictive equation.

  5. Actinide speciation in the shallow aquifer of Mortandad Canyon

    International Nuclear Information System (INIS)

    Nelson, D.M.; Polzer, W.L.

    1983-01-01

    Treated waste effluent at Los Alamos has been released to the environment in Mortandad Canyon since 1963. A study has been initiated to investigate the relative mobilities of the actinides in the shallow aquifer of Mortandad Canyon. Speciation of radionuclides and their adsorption by sediment are important parameters in the evaluation of those mobilities. Plutonium concentrations and oxidation states were measured in water collected from four test wells (MCO 4, 5, 6, 7.5). A regular decrease in 239 240 Pu concentration was observed with increasing distance from the discharge point. The large difference between the concentrations discharged (30 to 1000 pCi/l for period 1977-1982) and those in the wells (0.02 to 5.4 pCi/l) suggests that progressive loss of plutonium from the water as it moves through the alluvium is probably more important in regulating the concentrations than the variability of concentrations in the discharged effluent. The K/sub D/s for 228 Th were also determined and found to be similar to those for plutonium. The factors regulating the concentration of dissolved plutonium are probably the same as those regulating 228 Th. In contrast the K/sub D/s of 241 Am decrease regularly with distance from the discharge point and are about 100 times lower than those of plutonium and thorium for water in wells farthest from the discharge point

  6. A sand budget for Marble Canyon, Arizona: implications for long-term monitoring of sand storage change

    Science.gov (United States)

    Grams, Paul E.

    2013-01-01

    Recent U.S. Geological Survey research is providing important insights into how best to monitor changes in the amount of tributary-derived sand stored on the bed of the Colorado River and in eddies in Marble Canyon, Arizona. Before the construction of Glen Canyon Dam and other dams upstream, sandbars in Glen, Marble, and Grand Canyons were replenished each year by sediment-rich floods. Sand input into the Colorado River is crucial to protecting endangered native fish, animals, and plants and cultural and recreational resources along the river in Glen Canyon National Recreation Area and Grand Canyon National Park.

  7. Megafauna of vulnerable marine ecosystems in French mediterranean submarine canyons: Spatial distribution and anthropogenic impacts

    Science.gov (United States)

    Fabri, M.-C.; Pedel, L.; Beuck, L.; Galgani, F.; Hebbeln, D.; Freiwald, A.

    2014-06-01

    Vulnerable Marine Ecosystems (VME) in the deep Mediterranean Sea have been identified by the General Fisheries Commission for the Mediterranean as consisting of communities of Scleractinia (Lophelia pertusa and Madrepora oculata), Pennatulacea (Funiculina quadrangularis) and Alcyonacea (Isidella elongata). This paper deals with video data recorded in the heads of French Mediterranean canyons. Quantitative observations were extracted from 101 video films recorded during the MEDSEACAN cruise in 2009 (Aamp/Comex). Qualitative information was extracted from four other cruises (two Marum/Comex cruises in 2009 and 2011 and two Ifremer cruises in 1995 and 2010) to support the previous observations in the Cassidaigne and Lacaze-Duthiers canyons. All the species, fishing impacts and litter recognized in the video films recorded from 180 to 700 m depth were mapped using GIS. The abundances and distributions of benthic fishing resources (marketable fishes, Aristeidae, Octopodidae), Vulnerable Marine Species, trawling scars and litter of 17 canyons were calculated and compared, as was the open slope between the Stoechades and Toulon canyons. Funiculina quadrangularis was rarely observed, being confined for the most part to the Marti canyon and, I. elongata was abundant in three canyons (Bourcart, Marti, Petit-Rhône). These two cnidarians were encountered in relatively low abundances, and it may be that they have been swept away by repeated trawling. The Lacaze-Duthiers and Cassidaigne canyons comprised the highest densities and largest colony sizes of scleractinian cold-water corals, whose distribution was mapped in detail. These colonies were often seen to be entangled in fishing lines. The alcyonacean Callogorgia verticillata was observed to be highly abundant in the Bourcart canyon and less abundant in several other canyons. This alcyonacean was also severely affected by bottom fishing gears and is proposed as a Vulnerable Marine Species. Our studies on anthropogenic

  8. Continuous deep sedation, physician-assisted suicide, and euthanasia in Huntington's disorder.

    Science.gov (United States)

    Lindblad, Anna; Juth, Niklas; Fürst, Carl Johan; Lynöe, Niels

    2010-11-01

    To investigate the attitudes among Swedish physicians and the general public towards continuous deep sedation (CDS) as an alternative treatment for a competent, not imminently dying patient with Huntington's disorder requesting physician-assisted suicide (PAS) and euthanasia. A questionnaire was distributed to 1200 physicians in Sweden and 1201 individuals in Stockholm. It consisted of three parts: 1) A vignette about a competent patient with Huntington's disease requesting PAS. When no longer competent, relatives request euthanasia on behalf of the patient. Responders were asked about their attitudes towards these requests and whether CDS would be an acceptable alternative. 2) General questions about PAS and euthanasia. 3) Background variables. The response rate was 56% (physicians) and 52% (general public). The majority of the general public and a fairly large proportion of physicians reported more liberal views on CDS than are expressed in current Swedish and international recommendations. In light of the results, we suggest that there is a need for a broader discussion about the recommendations for CDS, with a special focus on the needs of patients with progressive neurodegenerative disorders.

  9. Insights into gait disorders: walking variability using phase plot analysis, Huntington's disease.

    Science.gov (United States)

    Collett, Johnny; Esser, Patrick; Khalil, Hanan; Busse, Monica; Quinn, Lori; DeBono, Katy; Rosser, Anne; Nemeth, Andrea H; Dawes, Helen

    2014-09-01

    Huntington's disease (HD) is a progressive inherited neurodegenerative disorder. Identifying sensitive methodologies to quantitatively measure early motor changes have been difficult to develop. This exploratory observational study investigated gait variability and symmetry in HD using phase plot analysis. We measured the walking of 22 controls and 35 HD gene carriers (7 premanifest (PreHD)), 16 early/mid (HD1) and 12 late stage (HD2) in Oxford and Cardiff, UK. The unified Huntington's disease rating scale-total motor scores (UHDRS-TMS) and disease burden scores (DBS) were used to quantify disease severity. Data was collected during a clinical walk test (8.8 or 10 m) using an inertial measurement unit attached to the trunk. The 6 middle strides were used to calculate gait variability determined by spatiotemporal parameters (co-efficient of variation (CoV)) and phase plot analysis. Phase plots considered the variability in consecutive wave forms from vertical movement and were quantified by SDA (spatiotemporal variability), SDB (temporal variability), ratio ∀ (ratio SDA:SDB) and Δangleβ (symmetry). Step time CoV was greater in manifest HD (p0.05). Phase plot analysis identified differences between manifest HD and controls for SDB, Ratio ∀ and Δangle (all pplot analysis may be a sensitive method of detecting gait changes in HD and can be performed quickly during clinical walking tests. Copyright © 2014 Elsevier B.V. All rights reserved.

  10. Electronic Transport in Single-Stranded DNA Molecule Related to Huntington's Disease

    Science.gov (United States)

    Sarmento, R. G.; Silva, R. N. O.; Madeira, M. P.; Frazão, N. F.; Sousa, J. O.; Macedo-Filho, A.

    2018-04-01

    We report a numerical analysis of the electronic transport in single chain DNA molecule consisting of 182 nucleotides. The DNA chains studied were extracted from a segment of the human chromosome 4p16.3, which were modified by expansion of CAG (cytosine-adenine-guanine) triplet repeats to mimics Huntington's disease. The mutated DNA chains were connected between two platinum electrodes to analyze the relationship between charge propagation in the molecule and Huntington's disease. The computations were performed within a tight-binding model, together with a transfer matrix technique, to investigate the current-voltage (I-V) of 23 types of DNA sequence and compare them with the distributions of the related CAG repeat numbers with the disease. All DNA sequences studied have a characteristic behavior of a semiconductor. In addition, the results showed a direct correlation between the current-voltage curves and the distributions of the CAG repeat numbers, suggesting possible applications in the development of DNA-based biosensors for molecular diagnostics.

  11. Adenyl cyclase activator forskolin protects against Huntington's disease-like neurodegenerative disorders

    Directory of Open Access Journals (Sweden)

    Sidharth Mehan

    2017-01-01

    Full Text Available Long term suppression of succinate dehydrogenase by selective inhibitor 3-nitropropionic acid has been used in rodents to model Huntington's disease where mitochondrial dysfunction and oxidative damages are primary pathological hallmarks for neuronal damage. Improvements in learning and memory abilities, recovery of energy levels, and reduction of excitotoxicity damage can be achieved through activation of Adenyl cyclase enzyme by a specific phytochemical forskolin. In this study, intraperitoneal administration of 10 mg/kg 3-nitropropionic acid for 15 days in rats notably reduced body weight, worsened motor cocordination (grip strength, beam crossing task, locomotor activity, resulted in learning and memory deficits, greatly increased acetylcholinesterase, lactate dehydrogenase, nitrite, and malondialdehyde levels, obviously decreased adenosine triphosphate, succinate dehydrogenase, superoxide dismutase, catalase, and reduced glutathione levels in the striatum, cortex and hippocampus. Intragastric administration of forskolin at 10, 20, 30 mg/kg dose-dependently reversed these behavioral, biochemical and pathological changes caused by 3-nitropropionic acid. These results suggest that forskolin exhibits neuroprotective effects on 3-nitropropionic acid-induced Huntington's disease-like neurodegeneration.

  12. Nonparametric modeling and analysis of association between Huntington's disease onset and CAG repeats.

    Science.gov (United States)

    Ma, Yanyuan; Wang, Yuanjia

    2014-04-15

    Huntington's disease (HD) is a neurodegenerative disorder with a dominant genetic mode of inheritance caused by an expansion of CAG repeats on chromosome 4. Typically, a longer sequence of CAG repeat length is associated with increased risk of experiencing earlier onset of HD. Previous studies of the association between HD onset age and CAG length have favored a logistic model, where the CAG repeat length enters the mean and variance components of the logistic model in a complex exponential-linear form. To relax the parametric assumption of the exponential-linear association to the true HD onset distribution, we propose to leave both mean and variance functions of the CAG repeat length unspecified and perform semiparametric estimation in this context through a local kernel and backfitting procedure. Motivated by including family history of HD information available in the family members of participants in the Cooperative Huntington's Observational Research Trial (COHORT), we develop the methodology in the context of mixture data, where some subjects have a positive probability of being risk free. We also allow censoring on the age at onset of disease and accommodate covariates other than the CAG length. We study the theoretical properties of the proposed estimator and derive its asymptotic distribution. Finally, we apply the proposed methods to the COHORT data to estimate the HD onset distribution using a group of study participants and the disease family history information available on their family members. Copyright © 2013 John Wiley & Sons, Ltd.

  13. [Predictors of mental and physical quality of life in Huntington's disease].

    Science.gov (United States)

    Brugger, F; Hepperger, C; Hametner, E-M; Holl, A K; Painold, A; Schusterschitz, C; Bonelli, R; Holas, C; Wenning, G K; Poewe, W; Seppi, K

    2015-02-01

    The assessment of health-related quality of life (hrQoL) is an important tool in therapy studies and in the treatment of patients with Huntington's disease (HD). In the absence of causal interventions, HD therapy targets the alleviation of symptoms aiming to improve impaired hrQoL. The aim of this study was to determine the impact of disease characteristics on hrQoL in HD. A total of 80 genetically confirmed HD patients underwent an assessment using the Unified Huntington's Disease Rating Scale, the Beck Depression Inventory, the Hamilton Rating Scale and the SF-36, a scale for the assessment of physical and mental QoL. Multiple regression analysis revealed that health-related physical and mental QoL was considerably influenced by the functional capacity. The mental QoL also correlated with the degree of depressive symptoms, age and the number of CAG repeats. However, there was no statistical relation between QoL and motor and cognitive abilities. This study underlines the relationship between function capacity and depressive symptoms with mental and physical QoL. This is the first time that hrQoL has been investigated in a German speaking cohort. The results are in accordance with previous studies of hrQoL in HD.

  14. Neurotensin receptor binding levels in basal ganglia are not altered in Huntington's chorea or schizophrenia

    International Nuclear Information System (INIS)

    Palacios, J.M.; Chinaglia, G.; Rigo, M.; Ulrich, J.; Probst, A.

    1991-01-01

    Autoradiographic techniques were used to examine the distribution and levels of neurotensin receptor binding sites in the basal ganglia and related regions of the human brain. Monoiodo ( 125 I-Tyr3)neurotensin was used as a ligand. High amounts of neurotensin receptor binding sites were found in the substantia nigra pars compacta. Lower but significant quantities of neurotensin receptor binding sites characterized the caudate, putamen, and nucleus accumbens, while very low quantities were seen in both medial and lateral segments of the globus pallidus. In Huntington's chorea, the levels of neurotensin receptor binding sites were found to be comparable to those of control cases. Only slight but not statistically significant decreases in amounts of receptor binding sites were detected in the dorsal part of the head and in the body of caudate nucleus. No alterations in the levels of neurotensin receptor binding sites were observed in the substantia nigra pars compacta and reticulata. These results suggest that a large proportion of neurotensin receptor binding sites in the basal ganglia are located on intrinsic neurons and on extrinsic afferent fibers that do not degenerate in Huntington's disease

  15. Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model

    DEFF Research Database (Denmark)

    Aidt, Frederik Heurlin; Nielsen, Signe Marie Borch; Kanters, Jørgen

    2013-01-01

    Metabolic dysfunction and mitochondrial involvement are recognised as part of the pathology in Huntington's Disease (HD). Post-mortem examinations of the striatum from end-stage HD patients have shown a decrease in the in vitro activity of complexes II, III and IV of the electron transport system...

  16. Transgenic Rat Model of Huntington's Disease: A Histopathological Study and Correlations with Neurodegenerative Process in the Brain of HD Patients.

    Czech Academy of Sciences Publication Activity Database

    Mazurová, Y.; Anděrová, Miroslava; Němečková, I.; Bezrouk, A.

    2014-01-01

    Roč. 2014, Aug 03 (2014), s. 291531 ISSN 2314-6133 R&D Projects: GA ČR(CZ) GBP304/12/G069 Grant - others:GA MŠk(CZ) Prvouk P37 Institutional support: RVO:68378041 Keywords : Huntington's Disease * neurodegenerative process in the brain Subject RIV: FH - Neurology Impact factor: 1.579, year: 2014

  17. Assessment of motor function, sensory motor gating and recognition memory in a novel BACHD transgenic rat model for Huntington disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Nguyen, Huu Phuc; Schreiber, Rudy; Ellenbroek, Bart

    2013-01-01

    Rationale: Huntington disease (HD) is frequently first diagnosed by the appearance of motor symptoms; the diagnosis is subsequently confirmed by the presence of expanded CAG repeats (> 35) in the HUNTINGTIN (HTT) gene. A BACHD rat model for HD carrying the human full length mutated HTT with 97

  18. Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington's Disease Gene-expansion Carriers

    DEFF Research Database (Denmark)

    Unmack Larsen, Ida; Vinther-Jensen, Tua; Gade, Anders

    2015-01-01

    Executive functions (EF) and psychomotor speed (PMS) has been widely studied in Huntington's disease (HD). Most studies have focused on finding markers of disease progression by comparing group means at different disease stages. Our aim was to investigate performances on nine measures of EF and PMS...

  19. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    John P Miller

    Full Text Available A genome-scale RNAi screen was performed in a mammalian cell-based assay to identify modifiers of mutant huntingtin toxicity. Ontology analysis of suppressor data identified processes previously implicated in Huntington's disease, including proteolysis, glutamate excitotoxicity, and mitochondrial dysfunction. In addition to established mechanisms, the screen identified multiple components of the RRAS signaling pathway as loss-of-function suppressors of mutant huntingtin toxicity in human and mouse cell models. Loss-of-function in orthologous RRAS pathway members also suppressed motor dysfunction in a Drosophila model of Huntington's disease. Abnormal activation of RRAS and a down-stream effector, RAF1, was observed in cellular models and a mouse model of Huntington's disease. We also observe co-localization of RRAS and mutant huntingtin in cells and in mouse striatum, suggesting that activation of R-Ras may occur through protein interaction. These data indicate that mutant huntingtin exerts a pathogenic effect on this pathway that can be corrected at multiple intervention points including RRAS, FNTA/B, PIN1, and PLK1. Consistent with these results, chemical inhibition of farnesyltransferase can also suppress mutant huntingtin toxicity. These data suggest that pharmacological inhibition of RRAS signaling may confer therapeutic benefit in Huntington's disease.

  20. Monitoring riparian-vegetation composition and cover along the Colorado River downstream of Glen Canyon Dam, Arizona

    Science.gov (United States)

    Palmquist, Emily C.; Ralston, Barbara E.; Sarr, Daniel A.; Johnson, Taylor C.

    2018-06-05

    Vegetation in the riparian zone (the area immediately adjacent to streams, such as stream banks) along the Colorado River downstream of Glen Canyon Dam, Arizona, supports many ecosystem and societal functions. In both Glen Canyon and Grand Canyon, this ecosystem has changed over time in response to flow alterations, invasive species, and recreational use. Riparian-vegetation cover and composition are likely to continue to change as these pressures persist and new ones emerge. Because this system is a valuable resource that is known to change in response to flow regime and other disturbances, a long-term monitoring protocol has been designed with three primary objectives:Annually measure and summarize the status (composition and cover) of native and non-native vascular-plant species within the riparian zone of the Colorado River between Glen Canyon Dam and Lake Mead.At 5-year intervals, assess change in vegetation composition and cover in the riparian zone, as related to geomorphic setting and dam operations, particularly flow regime.Collect data in a manner that can be used by multiple stakeholders, particularly the basinwide monitoring program overseen by the National Park Service’s Northern Colorado Plateau Network Inventory and Monitoring program.A protocol for the long-term monitoring of riparian vegetation is described in detail and standard operating procedures are included herein for all tasks. Visual estimates of foliar and ground covers are collected in conjunction with environmental measurements to assess correlations of foliar cover with abiotic and flow variables. Sample quadrats are stratified by frequency of inundation, geomorphic feature, and by river segment to account for differences in vegetation type. Photographs of sites are also taken to illustrate qualitative characteristics of the site at the time of sampling. Procedures for field preparation, generating random samples, data collection, data management, collecting and managing unknown

  1. First molecular modeling report on novel arylpyrimidine kynurenine monooxygenase inhibitors through multi-QSAR analysis against Huntington's disease: A proposal to chemists!

    Science.gov (United States)

    Amin, Sk Abdul; Adhikari, Nilanjan; Jha, Tarun; Gayen, Shovanlal

    2016-12-01

    Huntington's disease (HD) is caused by mutation of huntingtin protein (mHtt) leading to neuronal cell death. The mHtt induced toxicity can be rescued by inhibiting the kynurenine monooxygenase (KMO) enzyme. Therefore, KMO is a promising drug target to address the neurodegenerative disorders such as Huntington's diseases. Fiftysix arylpyrimidine KMO inhibitors are structurally explored through regression and classification based multi-QSAR modeling, pharmacophore mapping and molecular docking approaches. Moreover, ten new compounds are proposed and validated through the modeling that may be effective in accelerating Huntington's disease drug discovery efforts. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Pollutant Concentrations in Street Canyons of Different Aspect Ratio with Avenues of Trees for Various Wind Directions

    Science.gov (United States)

    Gromke, Christof; Ruck, Bodo

    2012-07-01

    This study summarizes the effects of avenues of trees in urban street canyons on traffic pollutant dispersion. We describe various wind-tunnel experiments with different tree-avenue models in combination with variations in street-canyon aspect ratio W/ H (with W the street-canyon width and H the building height) and approaching wind direction. Compared to tree-free street canyons, in general, higher pollutant concentrations are found. Avenues of trees do not suppress canyon vortices, although the air ventilation in canyons is hindered significantly. For a perpendicular wind direction, increases in wall-average and wall-maximum concentrations at the leeward canyon wall and decreases in wall-average concentrations at the windward wall are found. For oblique and perpendicular wind directions, increases at both canyon walls are obtained. The strongest effects of avenues of trees on traffic pollutant dispersion are observed for oblique wind directions for which also the largest concentrations at the canyon walls are found. Thus, the prevailing assumption that attributes the most harmful dispersion conditions to a perpendicular wind direction does not hold for street canyons with avenues of trees. Furthermore, following dimensional analysis, an estimate of the normalized wall-maximum traffic pollutant concentration in street canyons with avenues of trees is derived.

  3. Fluctuating helical asymmetry and morphology of snails (Gastropoda in divergent microhabitats at 'Evolution Canyons I and II,' Israel.

    Directory of Open Access Journals (Sweden)

    Shmuel Raz

    Full Text Available Developmental instability of shelled gastropods is measured as deviations from a perfect equiangular (logarithmic spiral. We studied six species of gastropods at 'Evolution Canyons I and II' in Carmel and the Galilee Mountains, Israel, respectively. The xeric, south-facing, 'African' slopes and the mesic, north-facing, 'European' slopes have dramatically different microclimates and plant communities. Moreover, 'Evolution Canyon II' receives more rainfall than 'Evolution Canyon I.'We examined fluctuating asymmetry, rate of whorl expansion, shell height, and number of rotations of the body suture in six species of terrestrial snails from the two 'Evolution Canyons.' The xeric 'African' slope should be more stressful to land snails than the 'European' slope, and 'Evolution Canyon I' should be more stressful than 'Evolution Canyon II.' Only Eopolita protensa jebusitica showed marginally significant differences in fluctuating helical asymmetry between the two slopes. Contrary to expectations, asymmetry was marginally greater on the 'European' slope. Shells of Levantina spiriplana caesareana at 'Evolution Canyon I,' were smaller and more asymmetric than those at 'Evolution Canyon II.' Moreover, shell height and number of rotations of the suture were greater on the north-facing slopes of both canyons.Our data is consistent with a trade-off between drought resistance and thermoregulation in snails; Levantina was significantly smaller on the 'African' slope, for increasing surface area and thermoregulation, while Eopolita was larger on the 'African' slope, for reducing water evaporation. In addition, 'Evolution Canyon I' was more stressful than Evolution Canyon II' for Levantina.

  4. Geomorphic Thresholds of Submarine Canyons Along the U.S. Atlantic Continental Margin

    Science.gov (United States)

    Brothers, D. S.; ten Brink, U. S.; Andrews, B. D.; Chaytor, J. D.

    2011-12-01

    Vast networks of submarine canyons and associated channels are incised into the U.S. Atlantic continental slope and rise. Submarine canyons form by differential erosion and deposition, primarily from sedimentary turbidity flows. Theoretical and laboratory studies have investigated the initiation of turbidity flows and their capacity to erode and entrain sedimentary material at distances far from the shelf edge. The results have helped understand the nature of turbidite deposits on the continental slope and rise. Nevertheless, few studies have examined the linkages between down-canyon sediment transport and the morphology of canyon/channel networks using mesoscale analyses of swath bathymetry data. We present quantitative analysis of 100-m resolution multibeam bathymetry data spanning ~616,000 km2 of the slope and rise between Georges Banks and the Blake Plateau (New England to North Carolina). Canyons are categorized as shelf-indenting or slope-confined based on spatial scale, vertical relief and connection with terrestrial river systems during sea level low stands. Shelf-indenting canyons usually represent the trunk-canyon of submerged channel networks. On the rise, shelf-indenting canyons have relatively well-developed channel-levees and sharp inner-thalwag incision suggesting much higher frequency and volume of turbidity flows. Because of the similarities between submarine canyon networks and terrestrial river systems, we apply methods originally developed to study fluvial morphology. Along-canyon profiles are extracted from the bathymetry data and the power-law relationship between thalwag gradient and drainage area is examined for more than 180 canyons along an ~1200 km stretch of the US Atlantic margin. We observe distinct thresholds in the power-law relationship between drainage area and gradient. Almost all canyons with heads on the upper slope contain at least two linear segments when plotted in log-log form. The first segment along the upper slope is flat

  5. Experimental simulation of air quality in street canyon under changes of building orientation and aspect ratio.

    Science.gov (United States)

    Yassin, Mohamed F; Ohba, Masaake

    2012-09-01

    To assist validation of numerical simulations of urban pollution, air quality in a street canyon was investigated using a wind tunnel as a research tool under neutral atmospheric conditions. We used tracer gas techniques from a line source without buoyancy. Ethylene (C(2)H(4)) was used as the tracer gas. The street canyon model was formed of six parallel building rows of the same length. The flow and dispersion field was analyzed and measured using a hot-wire anemometer with split fiber probe and fast flame ionization detector. The diffusion flow field in the boundary layer within the street canyon was examined at different locations, with varying building orientations (θ=90°, 112.5°, 135° and 157.5°) and street canyon aspect ratios (W/H=1/2, 3/4 and 1) downwind of the leeward side of the street canyon model. Results show that velocity increases with aspect ratio, and with θ>90°. Pollutant concentration increases as aspect ratio decreases. This concentration decreases exponentially in the vertical direction, and decreases as θ increases from 90°. Measured pollutant concentration distributions indicate that variability of building orientation and aspect ratio in the street canyon are important for estimating air quality in the canyon. The data presented here can be used as a comprehensive database for validation of numerical models.

  6. On the pollutant removal, dispersion, and entrainment over two-dimensional idealized street canyons

    Science.gov (United States)

    Liu, Chun-Ho; Wong, Colman C. C.

    2014-01-01

    Pollutant dispersion over urban areas is not that well understood, in particular at the street canyon scale. This study is therefore conceived to examine how urban morphology modifies the pollutant removal, dispersion, and entrainment over urban areas. An idealized computational domain consisting of 12 two-dimensional (2D) identical street canyons of unity aspect ratio is employed. The large-eddy simulation (LES) is used to calculate the turbulent flows and pollutant transport in the urban boundary layer (UBL). An area source of uniform pollutant concentration is applied on the ground of the first street canyon. A close examination on the roof-level turbulence reveals patches of low-speed air masses in the streamwise flows and narrow high-speed downdrafts in the shear layer. Different from the flows over a smooth surface, the turbulence intensities are peaked near the top of the building roughness. The pollutant is rather uniformly distributed inside a street canyon but disperses quickly in the UBL over the buildings. Partitioning the vertical pollutant flux into its mean and turbulent components demystifies that the pollutant removal is mainly governed by turbulence. Whereas, mean wind carries pollutant into and out of a street canyon simultaneously. In addition to wind speed promotion, turbulent mixing is thus required to dilute the ground-level pollutants, which are then removed from the street canyon to the UBL. Atmospheric flows slow down rapidly after the leeward buildings, leading to updrafts carrying pollutants away from the street canyons (the basic pollutant removal mechanism).

  7. Rapid formation of a modern bedrock canyon by a single flood event

    Science.gov (United States)

    Lamb, Michael P.; Fonstad, Mark A.

    2010-07-01

    Deep river canyons are thought to form slowly over geological time (see, for example, ref. 1), cut by moderate flows that reoccur every few years. In contrast, some of the most spectacular canyons on Earth and Mars were probably carved rapidly during ancient megaflood events. Quantification of the flood discharge, duration and erosion mechanics that operated during such events is hampered because we lack modern analogues. Canyon Lake Gorge, Texas, was carved in 2002 during a single catastrophic flood. The event offers a rare opportunity to analyse canyon formation and test palaeo-hydraulic-reconstruction techniques under known topographic and hydraulic conditions. Here we use digital topographic models and visible/near-infrared aerial images from before and after the flood, discharge measured during the event, field measurements and sediment-transport modelling to show that the flood moved metre-sized boulders, excavated ~7m of limestone and transformed a soil-mantled valley into a bedrock canyon in just ~3days. We find that canyon morphology is strongly dependent on rock type: plucking of limestone blocks produced waterfalls, inner channels and bedrock strath terraces, whereas abrasion of cemented alluvium sculpted walls, plunge pools and streamlined islands. Canyon formation was so rapid that erosion might have been limited by the ability of the flow to transport sediment. We suggest that our results might improve hydraulic reconstructions of similar megafloods on Earth and Mars.

  8. Modern landscape processes affecting archaeological sites along the Colorado River corridor downstream of Glen Canyon Dam, Glen Canyon National Recreation Area, Arizona

    Science.gov (United States)

    East, Amy E.; Sankey, Joel B.; Fairley, Helen C.; Caster, Joshua J.; Kasprak, Alan

    2017-08-29

    The landscape of the Colorado River through Glen Canyon National Recreation Area formed over many thousands of years and was modified substantially after the completion of Glen Canyon Dam in 1963. Changes to river flow, sediment supply, channel base level, lateral extent of sedimentary terraces, and vegetation in the post-dam era have modified the river-corridor landscape and have altered the effects of geologic processes that continue to shape the landscape and its cultural resources. The Glen Canyon reach of the Colorado River downstream of Glen Canyon Dam hosts many archaeological sites that are prone to erosion in this changing landscape. This study uses field evaluations from 2016 and aerial photographs from 1952, 1973, 1984, and 1996 to characterize changes in potential windblown sand supply and drainage configuration that have occurred over more than six decades at 54 archaeological sites in Glen Canyon and uppermost Marble Canyon. To assess landscape change at these sites, we use two complementary geomorphic classification systems. The first evaluates the potential for aeolian (windblown) transport of river-derived sand from the active river channel to higher elevation archaeological sites. The second identifies whether rills, gullies, or arroyos (that is, overland drainages that erode the ground surface) exist at the archaeological sites as well as the geomorphic surface, and therefore the relative base level, to which those flow paths drain. Results of these assessments are intended to aid in the management of irreplaceable archaeological resources by the National Park Service and stakeholders of the Glen Canyon Dam Adaptive Management Program.

  9. Facies and depositional model of Almada Canyon, Almada Basin, Bahia, Brazil; Facies e modelo deposicional do Canyon de Almada, Bacia de Almada, Bahia

    Energy Technology Data Exchange (ETDEWEB)

    D' Avila, Roberto Salvador Francisco; Souza Cruz, Carlos Emanoel de; Oliveira Filho, Jose Souto; Jesus, Candida Menezes de; Cesero, Pedro de; Dias Filho, Dorval Carvalho; Lima, Claudio Coelho de; Queiroz, Claudia Lima de; Santos, Saulo Ferreira; Ferreira, Eduardo Araripe [PETROBRAS, Santos, SP (Brazil). Unidade de Negocio de Exploracao]. E-mail: rdavila@petrobras.com.br

    2004-11-01

    In the continental portion of the Almada Basin outcrops of canyon filling deposits are represented by turbidite channels and associated facies from Urucutuca Formation. The canyon - semi-exhumated - eroded basement and pre-Cenomanian sedimentary rocks. The field study of the outcrops and cores obtained in adjacent perforations lead to the understanding of the facies and processes that controlled the deposition of these channeled turbidite that can be compared to the reservoirs of many oil fields in Brazil. The Almada canyon is a submarine conduct of tectonic origin that was enlarged by the repeated passing of turbidity currents. During the rift phase and the Albian period, compressive events reactivated old N E and N W faults in the basement as trans current fault systems. The continuation of these stresses, from the Cenomanian to the Maastrichtian, developed normal faults that controlled a submarine canyon that connected the continent, where an estuary was formed between the mountains, to the deep marine region of the basin. The canyon has received sediments brought by catastrophic fluvial floods coming from the surrounding mountains, which formed hyperpicnal flows that have evolved as turbidity currents, thus causing erosion of the substrate and carrying a huge volume of sediments to the basin. A part of that load was deposited in the canyon and formed turbidite channels filled by conglomerates, sandstones and shales. These moderately to highly efficient turbidite are intercalated to pro delta pelites and low density turbid plumes deposits, which have mostly been re mobilized as slump and debris flows (chaotic deposits). Pelites were accumulated mainly in the normal fluvial sedimentation phases, when the sandy sediment was retained next to the canyon head and were reworked by the tides on the upper part of the estuary. (author)

  10. Draft environmental assessment: Davis Canyon site, Utah. Nuclear Waste Policy Act (Section 112)

    International Nuclear Information System (INIS)

    1984-12-01

    In February 1983, the US Department of Energy (DOE) identified the Davis Canyon site in Utah, as one of nine potentially acceptable sites for a mined geologic repository for spent nuclear fuel and high-level radioactive waste. To determine their suitability, the Davis Canyon site and the eight other potentially acceptable sites have been evaluated in accordance with the DOE's General Guidelines for the Recommendation of Sites for Nuclear Waste Repositories. These evaluations are reported in this draft environmental assessment (EA), which is being issued for public review and comment. The DOE findings and determinations that are based on these evaluations are preliminary and subject to public review and comment. A final EA will be prepared after considering the comments received. On the basis of the evaluations reported in this draft EA, the DOE has found that the Davis Canyon site is not disqualified under the guidelines. The site is in the Paradox Basin, which is one of five distinct geohydrologic settings considered for the first repository. This setting contains one other potentially acceptable site - the Lavender Canyon site. Although the Lavender Canyon site appears to be suitable for site characterization, the DOE has concluded that the Davis Canyon site is the preferred site in the Paradox Basin. Furthermore, the DOE finds that the site is suitable for site characterization because the evidence does not support a conclusion that the site will not be able to meet each of the qualifying conditions specified in the guidelines. On the basis of these findings, the DOE is proposing to nominate the Davis Canyon site as one of five sites suitable for characterization. Having compared the Davis Canyon site with the other four sites proposed for nomination, the DOE has determined that the Davis Canyon site is not one of the three preferred sites for recommendation to the President as candidates for characterization

  11. Long-term surveillance plan for the Burro Canyon disposal cell, Slick Rock, Colorado

    International Nuclear Information System (INIS)

    1996-11-01

    This long-term surveillance plant (LTSP) describes the US Department of energy's (DOE) long-term care program for the Uranium Mill Tailings Remediation Action (UMTRA) Project's burro Canyon disposal cell in San Miguel County, Colorado. This LTSP describes the long-term surveillance program the DOE will implement to ensure that the Burro Canyon disposal cell performs as designed. The program is based on site inspections to identify threats to disposal cell integrity. No ground water monitoring will be required at the Burro Canyon disposal cell because the ground water protection strategy is supplemental standards based on low-yield from the upper-most aquifer

  12. Impact of roof height non-uniformity on pollutant transport between a street canyon and intersections

    International Nuclear Information System (INIS)

    Nosek, Štěpán; Kukačka, Libor; Jurčáková, Klára; Kellnerová, Radka; Jaňour, Zbyněk

    2017-01-01

    This paper presents an extension of our previous wind-tunnel study (Nosek et al., 2016) in which we highlighted the need for investigation of the removal mechanisms of traffic pollution from all openings of a 3D street canyon. The extension represents the pollution flux (turbulent and advective) measurements at the lateral openings of three different 3D street canyons for the winds perpendicular and oblique to the along-canyon axis. The pollution was simulated by emitting a passive gas (ethane) from a homogeneous ground-level line source positioned along the centreline of the investigated street canyons. The street canyons were formed by courtyard-type buildings of two different regular urban-array models. The first model has a uniform building roof height, while the second model has a non-uniform roof height along each building's wall. The mean flow and concentration fields at the canyons' lateral openings confirm the findings of other studies that the buildings' roof-height variability at the intersections plays an important role in the dispersion of the traffic pollutants within the canyons. For the perpendicular wind, the non-uniform roof-height canyon appreciably removes or entrains the pollutant through its lateral openings, contrary to the uniform canyon, where the pollutant was removed primarily through the top. The analysis of the turbulent mass transport revealed that the coherent flow structures of the lateral momentum transport correlate with the ventilation processes at the lateral openings of all studied canyons. These flow structures coincide at the same areas and hence simultaneously transport the pollutant in opposite directions. - Highlights: • The pollutant transport strongly depends on the roof-height arrangement. • The non-uniform canyons also remove the pollutants through their lateral openings. • The higher the upstream wall, the more pollutant is removed through the top. • The lateral coherent structures correlate

  13. Fish Passage Assessment: Big Canyon Creek Watershed, Technical Report 2004.

    Energy Technology Data Exchange (ETDEWEB)

    Christian, Richard

    2004-02-01

    This report presents the results of the fish passage assessment as outlined as part of the Protect and Restore the Big Canyon Creek Watershed project as detailed in the CY2003 Statement of Work (SOW). As part of the Northwest Power Planning Council's Columbia Basin Fish and Wildlife Program (FWP), this project is one of Bonneville Power Administration's (BPA) many efforts at off-site mitigation for damage to salmon and steelhead runs, their migration, and wildlife habitat caused by the construction and operation of federal hydroelectric dams on the Columbia River and its tributaries. The proposed restoration activities within the Big Canyon Creek watershed follow the watershed restoration approach mandated by the Fisheries and Watershed Program. Nez Perce Tribal Fisheries/Watershed Program vision focuses on protecting, restoring, and enhancing watersheds and treaty resources within the ceded territory of the Nez Perce Tribe under the Treaty of 1855 with the United States Federal Government. The program uses a holistic approach, which encompasses entire watersheds, ridge top to ridge top, emphasizing all cultural aspects. We strive toward maximizing historic ecosystem productive health, for the restoration of anadromous and resident fish populations. The Nez Perce Tribal Fisheries/Watershed Program (NPTFWP) sponsors the Protect and Restore the Big Canyon Creek Watershed project. The NPTFWP has the authority to allocate funds under the provisions set forth in their contract with BPA. In the state of Idaho vast numbers of relatively small obstructions, such as road culverts, block thousands of miles of habitat suitable for a variety of fish species. To date, most agencies and land managers have not had sufficient, quantifiable data to adequately address these barrier sites. The ultimate objective of this comprehensive inventory and assessment was to identify all barrier crossings within the watershed. The barriers were then prioritized according to the

  14. Periodontitis determining the onset and progression of Huntington's disease: review of the literature

    Directory of Open Access Journals (Sweden)

    María Lourdes Rodríguez Coyago

    2015-10-01

    Full Text Available La enfermedad de Hungtinton es un trastorno neurodegenerativo, causado por la expansión de un triplete de citosina, adenina, guanina en el gen de la huntingtina. Se manifiesta con un deterioro físico, cognitivo y psiquiátrico a distintas edades en el adulto, con un pronóstico fatal. Además del número de repeticiones del triplete, existirían otros factores que explicarían el inicio de esta enfermedad a más temprana edad. Se sabe que la neuroinflamación es un protagonista en los trastornos neurodegenerativos, no siendo la enfermedad de Huntington una excepción. La neuroinflamación exacerba el daño neuronal producido por la mutación, al existir activación aberrante de la célula microglía, disfunción de astrocitos y células dendríticas; compromiso de la barrera hematoencefálica y activación de complemento, todas ellas como efecto directo e indirecto de la mutante y otros estímulos como infecciones crónicas. Es el interés del presente trabajo analizar la periodontitis, como modelo de infección bucodental crónica y fuente de inflamación sistémica. Hipotetizamos que el potencial rol de la periodontitis en la enfermedad de Huntington y los mecanismos por los cuales contribuiría a la manifestación temprana y progreso de dicha enfermedad, para lo cual se consideraron revisiones sistemáticas, metanálisis y estudios experimentales publicados tanto en español como en inglés obtenidos del PubMed y SciELO. Son diversos los mecanismos que generan inflamación en el cerebro de estos pacientes, adquiriendo especial protagonismo los mecanismos de la inmunidad innata. Las infecciones buco dentarias crónicas, como la enfermedad periodontal, pueden constituir un factor exacerbante de la neuroinflamación que per se asocia la enfermedad de Huntington.

  15. Current status of PET imaging in Huntington's disease

    Energy Technology Data Exchange (ETDEWEB)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios [King' s College London, Neurodegeneration Imaging Group, Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), Camberwell, London (United Kingdom)

    2016-06-15

    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading ''Huntington Disease'' combined with text and key words ''Huntington Disease'', ''Neuroimaging'' and ''PET''. Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([{sup 18}F]FDG and [{sup 15}O]H{sub 2}O), presynaptic ([{sup 18}F]fluorodopa, [{sup 11}C]β-CIT and [{sup 11}C]DTBZ) and postsynaptic ([{sup 11}C]SCH22390, [{sup 11}C]FLB457 and [{sup 11}C]raclopride) dopaminergic function, phosphodiesterases ([{sup 18}F]JNJ42259152, [{sup 18}F]MNI-659 and [{sup 11}C]IMA107), and adenosine ([{sup 18}F]CPFPX), cannabinoid ([{sup 18}F]MK-9470), opioid ([{sup 11}C]diprenorphine) and GABA ([{sup 11}C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed

  16. Diablo Canyon: the challenges of the Californian nuclear

    International Nuclear Information System (INIS)

    Avrin, Anne-Perrine; Zweibaum, Nicolas

    2014-01-01

    This article addresses the Californian nuclear plant of Diablo Canyon which, due to the shutting down of the two reactors of another plant, could be the last nuclear plant to operate in California as this state voted an interdiction of construction of any new nuclear plant until a permanent solution has been found for radioactive waste storage. It is outlined that this nuclear plant is the most controlled nuclear plant throughout the USA, presents an unchallenged security level despite it is built at the vicinity of seismic fault lines. It outlines that the problem of waste storage is far from being solved in California, and discusses the future role of nuclear energy in California within the context of global warming

  17. Is there excess argon in the Fish Canyon magmatic system?

    Science.gov (United States)

    Wilkinson, C. M.; Sherlock, S.; Kelley, S. P.; Charlier, B. L.

    2010-12-01

    Some phenocrysts from the Fish Canyon Tuff (San Juan volcanic field, south-western Colorado, USA) have yielded anomalously old 40Ar/39Ar apparent ages and yet the sanidine ages are sufficiently reproducible to allow its use as an international standard. The eruption age of the Fish Canyon tuff has recently been determined by high precision analysis and recalibration of the decay constants based on the sanidine standard at 28.305 ± 0.036 Ma [1], slightly younger than the generally accepted U-Pb age. Previously, minerals from the tuff have been used in various geochronological studies e.g., fission-track; U-Pb; Rb-Sr; K-Ar and 40Ar/39Ar, but U-Pb zircon ages which range 28.37 - 28.61 Ma appear to be older than the sanidine and other minerals, including biotite, yield older ages (27.41 - 28.25 Ma for biotite) [2]. In the Fish Canyon volcanic system, the erupted products are thought to exist in the magma chamber for significant periods prior to eruption [3] and then pass rapidly from a high temperature magmatic environment (where Ar is free to re-equilibrate among the minerals), to effectively being quenched upon eruption (where Ar becomes immobile). Artificially elevated ages, older than eruption age, have been identified in some 40Ar/39Ar geochronological studies (e.g. [4]). These older ages may either reflect; 1) argon accumulation in pheno- or xenocrysts (by radioactive decay of parent 40K), 2) excess argon (40ArE) incorporated into a mineral during crystallisation (via diffusion into the mineral lattice or hosted within fluid or melt inclusions) or 3) inherited radiogenic argon (the dated material contains a component older than the age of eruption) [5]. To better understand the effects of 40ArE on 40Ar/39Ar apparent ages we have conducted a detailed study of intra-grain grain age variations by UV-LAMP Ar-analysis. Analysis of polished thick sections has been performed in-situ using a 213nm laser and Nu Instruments Noblesse which is able to discriminate against

  18. Microbial ecology of deep-water mid-Atlantic canyons

    Science.gov (United States)

    Kellogg, Christina A.

    2011-01-01

    The research described in this fact sheet will be conducted from 2012 to 2014 as part of the U.S. Geological Survey's DISCOVRE (DIversity, Systematics, and COnnectivity of Vulnerable Reef Ecosystems) Program. This integrated, multidisciplinary effort will be investigating a variety of topics related to unique and fragile deep-sea ecosystems from the microscopic level to the ecosystem level. One goal is to improve understanding, at the microbiological scale, of the benthic communities (including corals) that reside in and around mid-Atlantic canyon habitats and their associated environments. Specific objectives include identifying and characterizing the microbial associates of deep-sea corals, characterizing the microbial biofilms on hard substrates to better determine their role in engineering the ecosystem, and adding a microbial dimension to benthic community structure and function assessments by characterizing micro-eukaryotes, bacteria, and archaea in deep-sea sediments.

  19. Evaluation of air quality and noise impact assessments, Davis Canyon

    International Nuclear Information System (INIS)

    1986-05-01

    In this report, several issues are identified regarding the air quality and noise assessments presented in the final salt repository environmental assessment (EA) prepared by the US Department of Energy for the Davis Canyon, Utah, site. Necessary revisions to the data and methods used to develop the EA impact assessment are described. Then, a comparative evaluation is presented in which estimated impacts based upon the revised data and methods are compared with the impacts published in the EA. The evaluation indicates that the conclusions of the EA air quality and noise impact sections would be unchanged. Consequently, the guideline findings presented in Chapter 6 of the EA are also unchanged by the revised analysis. 50 refs., 16 tabs

  20. Achieving quality excellence at the Diablo Canyon Nuclear Power Plant

    International Nuclear Information System (INIS)

    Skidmore, S.M.; Taggart, D.A.

    1988-01-01

    Quality assurance methods at the Diablo Canyon plant were transformed from the then typical industry practices that often alienated professional and technical people, as well as craftsmen and their foremen, to a cooperative method that allowed plant personnel to work together as a team. It has created an attitude to do it right the first time. The roles of quality professionals were expanded to include teaching and coaching to facilitate enhanced communication between and within functional organizations. This included regular presentations to managers and line personnel in an informal group participative atmosphere. These presentations have become widely known at the plant as quality awareness tailboard sessions. These presentations are intended to increase personnel sensitivity to the subject of quality and quality management. Economic achievement of excellence in quality is essential to remain competitive in today's marketplace. The proactive team-oriented approach of quality assurance achieves the bottom line of high quality with concurrently enhanced productivity and cost-effectiveness

  1. RECALIBRATION OF H CANYON ONLINE SPECTROPHOTOMETER AT EXTENDED URANIUM CONCENTRATION

    International Nuclear Information System (INIS)

    Lascola, R

    2008-01-01

    The H Canyon online spectrophotometers are calibrated for measurement of the uranium and nitric acid concentrations of several tanks in the 2nd Uranium Cycle.[1] The spectrometers, flow cells, and prediction models are currently optimized for a process in which uranium concentrations are expected to range from 0-15 g/L and nitric acid concentrations from 0.05-6 M. However, an upcoming processing campaign will involve 'Super Kukla' material, which has a lower than usual enrichment of fissionable uranium. Total uranium concentrations will be higher, spanning approximately 0-30 g/L U, with no change in the nitric acid concentrations. The new processing conditions require the installation of new flow cells with shorter path lengths. As the process solutions have a higher uranium concentration, the shorter path length is required to decrease the absorptivity to values closer to the optimal range for the instrument. Also, new uranium and nitric acid prediction models are required to span the extended uranium concentration range. The models will be developed for the 17.5 and 15.4 tanks, for which nitric acid concentrations will not exceed 1 M. The restricted acid range compared to the original models is anticipated to reduce the measurement uncertainty for both uranium and nitric acid. The online spectrophotometers in H Canyon Second Uranium Cycle were modified to allow measurement of uranium and nitric acid for the Super Kukla processing campaign. The expected uranium concentrations, which are higher than those that have been recently processed, required new flow cells with one-third the optical path length of the existing cells. Also, new uranium and nitric acid calibrations were made. The estimated reading uncertainties (2σ) for Tanks 15.4 and 17.5 are ∼5% for uranium and ∼25% for nitric acid

  2. Formation of Box Canyon, Idaho, by megaflood: implications for seepage erosion on Earth and Mars.

    Science.gov (United States)

    Lamb, Michael P; Dietrich, William E; Aciego, Sarah M; Depaolo, Donald J; Manga, Michael

    2008-05-23

    Amphitheater-headed canyons have been used as diagnostic indicators of erosion by groundwater seepage, which has important implications for landscape evolution on Earth and astrobiology on Mars. Of perhaps any canyon studied, Box Canyon, Idaho, most strongly meets the proposed morphologic criteria for groundwater sapping because it is incised into a basaltic plain with no drainage network upstream, and approximately 10 cubic meters per second of seepage emanates from its vertical headwall. However, sediment transport constraints, 4He and 14C dates, plunge pools, and scoured rock indicate that a megaflood (greater than 220 cubic meters per second) carved the canyon about 45,000 years ago. These results add to a growing recognition of Quaternary catastrophic flooding in the American northwest, and may imply that similar features on Mars also formed by floods rather than seepage erosion.

  3. Data Quality Objectives Summary Report for the 221-U Canyon Disposition Alternatives

    International Nuclear Information System (INIS)

    Miller, M.S.; Oaces, L.E.; Baxter, J.; Brown, T.M.; Enoke, D.E.; Carlson, D.; Rugg, J.E.

    1997-08-01

    The 221-U Canyon Disposition Alternatives Data Quality Objective (DQO) Process identifies the sampling and analytical requirements necessary to support future detailed evaluation of alternatives via the CERCLA process, for final disposition of the 221-U Canyon Facility. Viable alternatives for the disposition of the 221-U Facility have been identified in a CERCLA Phase I Feasibility Study (FS) (DOE-RL 1997) for the Canyon Disposition Initiative (CDI). The scope of this DQO Process is limited to the 221-U Process Canyon Building and equipment contained within the facility. Associated stacks, filters, solvent handling, vaults, and storage facilities external to the 221-U Building are not addressed in this DQO. This DQO focuses on the 221-U Building because it provides the greatest potential source of contaminant volumes and concentrations and the physical structure poses the greatest challenge for disposition decisions

  4. 76 FR 23623 - Backcountry Management Plan, Environmental Impact Statement, Grand Canyon National Park, Arizona

    Science.gov (United States)

    2011-04-27

    ...-7945, [email protected] or Rachel Bennett, Environmental Protection Specialist, P.O. Box 129, Grand Canyon, Arizona 86023, 928-638-7326, Rachel[email protected] . SUPPLEMENTARY INFORMATION: If you wish to...

  5. Deepwater Canyons 2012: Pathways to the Abyss on NOAA Ship Nancy Foster between 20120815 and 20121001

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Mid-Atlantic Deep-Water Canyons project is co-funded by the Bureau of Ocean Energy Management (BOEM) and NOAA's Office of Ocean Exploration and Research (which...

  6. Integrated Project Management Planning for the Deactivation of the Savannah River Site F-Canyon Complex

    Energy Technology Data Exchange (ETDEWEB)

    Clark, T.G.

    2000-12-01

    This paper explains the planning process that is being utilized by the Westinghouse Savannah River Company to take the F-Canyon Complex facilities from operations to a deactivated condition awaiting final decommissioning.

  7. Fire modeling for Building 221-T - T Plant Canyon Deck and Railroad Tunnel

    International Nuclear Information System (INIS)

    Oar, D.L.

    1994-01-01

    This report was prepared by Hughes Associates, Inc. to document the results of fire models for building 221-T Canyon Deck and Railroad Tunnel. Backup data is contained in document No. WHC-SD-CP-ANAL-010, Rev. 0

  8. Lightning protection for the process canyons at the Savannah River site

    International Nuclear Information System (INIS)

    McAfee, D.E.

    1995-01-01

    Westinghouse Savannah River Company (WSRC) has performed Lightning Studies for the existing Process Canyons at the Savannah River Site (SRS). These studies were initiated to verify the lightning protection systems for the facilities and to compare the installations to the National Fire Protection (NFPA) Standard 780, Lighting Protection Code, 1992. The original study of the F-Canyon was initiated to develop answers to concerns raised by the Defense Nuclear Facility Safety Board (DNFSB). Once this study was completed it was determined that a similar study for H-Canyon would be prudent; followed by an evaluation of the Defense Waste Processing Facility (DWPF) Vitrification Building (S-Canyon). This paper will provide an overview of the nature of lightning and the principals of lightning protection. This will provide the reader with a basic understanding of the phenomena of lighting and its potential for damaging structures, components, and injuring personnel in or near the structure

  9. Submarine canyons along the upper Sardinian slope (Central Western Mediterranean) as repositories for derelict fishing gears.

    Science.gov (United States)

    Cau, Alessandro; Alvito, Andrea; Moccia, Davide; Canese, Simonepietro; Pusceddu, Antonio; Rita, Cannas; Angiolillo, Michela; Follesa, Maria C

    2017-10-15

    By means of ROV surveys, we assessed the quantity, composition and bathymetric distribution of marine litter in 17 sites along the Sardinian continental margin (Central Western Mediterranean) at depths ranging from 100 to 480m. None of the investigated sites was litter free, but the mean density of litter (0.0175±0.0022itemsm -2 ) was lower than that reported from other Tyrrhenian regions. The difference in the total litter density among sites was negligible, but the density of derelict fishing gear (DFG) items (most of which ascribable to small scale fishery) in submarine canyons was higher in submarine canyons than in other habitats. Our result suggest that submarine canyons (known to be highly vulnerable ecosystems) act as major repositories of DFGs, and, therefore, we anticipate the need of specific measures aimed at minimizing the loss and abandonment of DFGs in submarine canyons. Copyright © 2017 Elsevier Ltd. All rights reserved.

  10. Giant landslides and turbidity currents in the Agadir Canyon Region, NW-Africa

    Science.gov (United States)

    Krastel, Sebastian; Wynn, Russell B.; Stevenson, Christopher; Feldens, Peter; Mehringer, Lisa; Schürer, Anke

    2017-04-01

    Coring and drilling of the Moroccan Turbidite System off NW-Africa revealed a long sequence of turbidites, mostly sourced from the Moroccan continental margin and the volcanic Canary Islands. The largest individual flow deposits in the Moroccan Turbidite System contain sediment volumes >100 km3, although these large-scale events are relatively infrequent with a recurrence interval of 10,000 years (over the last 200,000 years). The largest siliciclastic flow in the last 200,000 years was the 'Bed 5 event', which transported 160 km3 of sediment up to 2000 km from the Agadir Canyon region to the southwest Madeira Abyssal Plain. While the Moroccan Turbidite System is extremely well investigated, almost no data from the source region, i.e. the Agadir Canyon, are available. Understanding why some submarine landslides remain as coherent blocks of sediment throughout their passage downslope, while others mix and disintegrate almost immediately after initial failure, is a major scientific challenge, which was addressed in the Agadir Canyon source region during RV Maria S. Merian Cruise MSM32 in late 2013. A major landslide area was identified 200 km south of the Agadir Canyon. A landslide was traced from this failure area to the Agadir Canyon. This landslide entered the canyon in about 2500 m water depth. Despite a significant increase in slope angle, the landslide did not disintegrate into a turbidity current when entering the canyon but moved on as landslide for at least another 200 km down the canyon. The age of the landslide ( 145 ka) does not correspond to any major turbidte deposit in the Moroccan Turbidite System, further supporting the fact that the landslide did not disintegrate into a major turbidity current. A core taken about 350 m above the thalweg in the head region of Agadir Canyon shows a single coarse-grained turbidite, which resembles the composition of the Bed 5 event in the Madeira Abyssal Plain. Hence, the Bed 5 turbidite originated as a failure in the

  11. Modulation at Age of Onset in Tunisian Huntington Disease Patients: Implication of New Modifier Genes

    Directory of Open Access Journals (Sweden)

    Dorra Hmida-Ben Brahim

    2014-01-01

    Full Text Available Huntington’s disease (HD is an autosomal dominant neurodegenerative disorder. The causative mutation is an expansion of more than 36 CAG repeats in the first exon of IT15 gene. Many studies have shown that the IT15 interacts with several modifier genes to regulate the age at onset (AO of HD. Our study aims to investigate the implication of CAG expansion and 9 modifiers in the age at onset variance of 15 HD Tunisian patients and to establish the correlation between these modifiers genes and the AO of this disease. Despite the small number of studied patients, this report consists of the first North African study in Huntington disease patients. Our results approve a specific effect of modifiers genes in each population.

  12. Neuropsychological correlates of brain atrophy in Huntington's disease: a magnetic resonance imaging study

    International Nuclear Information System (INIS)

    Starkstein, S.E.; Brandt, J.; Bylsma, F.; Peyser, C.; Folstein, M.; Folstein, S.E.

    1992-01-01

    Magnetic resonance imaging and a comprehensive cognitive evaluation were carried out in a series of 29 patients with mild to moderate Huntington's disease (HD). A factor analysis of the neuropsychological test scores provided three factors: A memory/speed-of-processing factor, a 'frontal' factor, and a response inhibition factor. The memory/speed factor correlated significantly with measures of caudate atrophy, frontal atrophy, and atrophy of the left (but not the right) sylvian cistern. There were no significant correlations between the 'frontal' or response inhibition factors and measures of cortical or subcortical brain atrophy. Our findings confirm that subcortical atrophy is significantly correlated with specific cognitive deficits in HD, and demonstrate that cortical atrophy also has important association with the cognitive deficits of patients with HD. (orig.)

  13. Liver function in Huntington's disease assessed by blood biochemical analyses in a clinical setting

    DEFF Research Database (Denmark)

    Nielsen, Signe Marie Borch; Vinther-Jensen, Tua; Nielsen, Jørgen E.

    2016-01-01

    dysfunction are seen. Blood levels of a wide range of hormones, metabolites and proteins have been analyzed in HD patients, identifying several changes associated with the disease. However, a comprehensive panel of liver function tests (LFT) has not been performed. We investigated a cohort of manifest......Huntington's disease (HD) is a dominantly inherited, progressive neurological disorder caused by a CAG repeat elongation in the huntingtin gene. In addition to motor-, psychiatric- A nd cognitive dysfunction, peripheral disease manifestations in the form of metabolic changes and cellular...... and premanifest HD gene-expansion carriers and controls, using a clinically applied panel of LFTs. Here, we demonstrate that the level of alkaline phosphatase is increased in manifest HD gene-expansion carriers compared to premanifest HD gene-expansion carriers and correlate with increased disease severity...

  14. Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients.

    Science.gov (United States)

    van Roon-Mom, Willeke M C; Roos, Raymund A C; de Bot, Susanne T

    2018-04-01

    On December 11 of 2017, Ionis Pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their HTTRx Phase 1/2a study in Huntington disease (HD) patients. The results from this Ionis trial have gained much attention from the patient community and the oligonucleotide therapeutics field, since it is the first trial targeting the cause of HD, namely the mutant huntingtin protein, using antisense oligonucleotides (ASOs). The press release also states that the primary endpoints of the study (safety and tolerability) were met, but does not contain data. This news follows the approval of another therapeutic ASO nusinersen (trade name Spinraza) for a neurological disease, spinal muscular atrophy, by the U.S. Food and Drug Administration and European Medicines Agency, in 2016 and 2017, respectively. Combined, this offers hope for the development of the HTTRx therapy for HD patients.

  15. Map showing selected surface-water data for the Huntington 30 x 60-minute quadrangle, Utah

    Science.gov (United States)

    Price, Don

    1984-01-01

    This is one of a series of maps that describe the geology and related natural resources of the Huntington 30 x 60-minute quadrangle, Utah. Streamflow records used to compile this map were collected by the U.S. Geological Survey in cooperation with the Utah Department of Natural Resources, Division of Water Rights, and the Utah Department of Transportation. The principal runoff-producing area shown on the map was delineated from a work map (scale 1:250,000) compiled to estimate water yields in Utah (Bagley and others, 1964). Sources of information about recorded floods resulting from cloudbursts included Woolley (1946) and Butler and Marsell (1972); sources of information about the chemical quality of streamflow included Mundorff (1972) and Mundorff and Thompson (1982).

  16. Therapeutic strategies for circadian rhythm and sleep disturbances in Huntington disease.

    Science.gov (United States)

    van Wamelen, Daniel J; Roos, Raymund Ac; Aziz, Nasir A

    2015-12-01

    Aside from the well-known motor, cognitive and psychiatric signs and symptoms, Huntington disease (HD) is also frequently complicated by circadian rhythm and sleep disturbances. Despite the observation that these disturbances often precede motor onset and have a high prevalence, no studies are available in HD patients which assess potential treatments. In this review, we will briefly outline the nature of circadian rhythm and sleep disturbances in HD and subsequently focus on potential treatments based on findings in other neurodegenerative diseases with similarities to HD, such as Parkinson and Alzheimer disease. The most promising treatment options to date for circadian rhythm and sleep disruption in HD include melatonin (agonists) and bright light therapy, although further corroboration in clinical trials is warranted.

  17. Age at onset in Huntington's disease: replication study on the association of HAP1.

    Science.gov (United States)

    Karadima, Georgia; Dimovasili, Christina; Koutsis, Georgios; Vassilopoulos, Demetris; Panas, Marios

    2012-11-01

    In recent years two association studies investigating the HAP1 T441M (rs4523977) polymorphism as a potential modifying factor of the age at onset (AAO) of Huntington's disease (HD), have been reported. Initially evidence for association was found between the M441 risk allele and the AAO. Subsequently, a second study, although failing to replicate these findings, found evidence for association between the same risk allele and AAO of motor symptoms (mAAO). In the present study, the role of the HAP1 T441M polymorphism as a modifier of the AAO in HD was investigated in a cohort of 298 Greek HD patients. In this cohort the CAG repeat number accounted for 55% of the variance in AAO. No association was found between the HAP1 T441M polymorphism and the AAO of HD. © 2012 Elsevier Ltd. All rights reserved.

  18. Brain energy metabolism and dopaminergic function in Huntington's disease measured in vivo using positron emission tomography

    International Nuclear Information System (INIS)

    Leenders, K.L.; Frackowiak, R.S.; Quinn, N.; Marsden, C.D.

    1986-01-01

    A 48-year-old man with typical Huntington's disease was investigated with computed tomography (CT) and positron emission tomography. Regional cerebral blood flow, oxygen extraction, oxygen and glucose utilization, L-Dopa uptake, and dopamine (D2) receptor binding were measured using several positron-labelled tracers. CT showed slight atrophy of the head of caudate but no cortical atrophy, although distinct frontal lobe dysfunction was present on psychometric testing. Oxygen and glucose metabolism and cerebral blood flow were decreased in the striata and to a lesser extent in frontal cortex. Cerebral blood flow was in the low normal range throughout the remainder of the brain. A normal metabolic ratio was found in all regions, since the changes in glucose utilization paralleled those in oxygen consumption. The capacity of the striatum to store dopamine as assessed by L-[ 18 F]-fluorodopa uptake was normal, but dopamine (D2) receptor binding was decreased when compared to normal subjects

  19. Tics as an initial manifestation of juvenile Huntington's disease: case report and literature review.

    Science.gov (United States)

    Cui, Shi-Shuang; Ren, Ru-Jing; Wang, Ying; Wang, Gang; Chen, Sheng-Di

    2017-08-08

    Huntington's disease (HD) is an autosomal dominant disorder, typically characterized by chorea due to a trinucleotide repeat expansion in the HTT gene, although the clinical manifestations of patients with juvenile HD (JHD) are atypical. A 17-year-old boy with initial presentation of tics attended our clinic and his DNA analysis demonstrated mutation in the HTT gene (49 CAG repeats). After treatment, his symptoms improved. Furthermore, we performed literature review through searching the databases and summarized clinical features in 33 JHD patients. The most prevalent symptoms are ataxia, and two cases reported that tics as initial and prominent manifestation in JHD. Among them, 88% patients carried CAG repeats beyond 60 and most of them have family history. This case here illustrates the variable range of clinical symptoms of JHD and the necessity of testing for the HD mutation in young patients with tics with symptoms unable to be explained by Tourette's syndrome (TS).

  20. Social Cognition, Executive Functions and Self-Report of Psychological Distress in Huntington's Disease

    DEFF Research Database (Denmark)

    Larsen, Ida Unmack; Vinther-Jensen, Tua; Nielsen, Jørgen Erik

    2016-01-01

    OBJECTIVE: Huntington's disease (HD) is characterized by motor symptoms, psychiatric symptoms and cognitive impairment in, inter alia, executive functions and social cognition. The aim of this study was to investigate the relationship between subjective feeling of psychological distress using...... a self-report questionnaire and performances on tests of executive functions and social cognition in a large consecutive cohort of HD patients. METHOD: 50 manifest HD patients were tested in social cognition and executive functions and each answered a self-report questionnaire about current status...... psychological distress was significantly associated with worse performances on social cognitive tests (mean absolute correlation .34) and that there were no significant correlations between perceived psychological distress and performance on tests of executive functions. The correlations between perceived...

  1. Oxidative Stress and Huntington's Disease: The Good, The Bad, and The Ugly.

    Science.gov (United States)

    Kumar, Amit; Ratan, Rajiv R

    2016-10-01

    Redox homeostasis is crucial for proper cellular functions, including receptor tyrosine kinase signaling, protein folding, and xenobiotic detoxification. Under basal conditions, there is a balance between oxidants and antioxidants. This balance facilitates the ability of oxidants, such as reactive oxygen species, to play critical regulatory functions through a direct modification of a small number of amino acids (e.g. cysteine) on signaling proteins. These signaling functions leverage tight spatial, amplitude, and temporal control of oxidant concentrations. However, when oxidants overwhelm the antioxidant capacity, they lead to a harmful condition of oxidative stress. Oxidative stress has long been held to be one of the key players in disease progression for Huntington's disease (HD). In this review, we will critically review this evidence, drawing some intermediate conclusions, and ultimately provide a framework for thinking about the role of oxidative stress in the pathophysiology of HD.

  2. Aerial gamma ray and magnetic survey, Huntington quadrangle: Ohio, West Virginia and Kentucky. Final report

    International Nuclear Information System (INIS)

    1981-04-01

    The Huntington quadrangle of Kentucky, Ohio, and West Virginia covers 7250 square miles of the easternmost Midwestern Physiographic Province. Paleozoic exposures dominate the surface. These Paleozoics deepen toward the east from approximately 500 feet to a maximum depth of 8000 feet. Precambrian basement is thought to underlie the entire area. No known uranium deposits exist in the area. One hundred anomalies were found using the standard statistical analysis. Some high uranium concentration anomalies that may overlie the stratigraphic equivalent of the Devonian-Mississippian New Albany or Chattanooga Shales may represent significant levels of naturally occurring uranium. Future studies should concentrate on this unit. Magnetic data are largely in concurrence with existing structural interpretations but suggest some complexities in the underlying Precambrian

  3. Is gardening a stimulating activity for people with advanced Huntington's disease?

    Science.gov (United States)

    Spring, Josephine A; Viera, Marc; Bowen, Ceri; Marsh, Nicola

    2014-11-01

    This study evaluated adapted gardening as an activity for people with advanced Huntington's disease (HD) and explored its therapeutic aspects. Visitors and staff completed a questionnaire and participated in structured interviews to capture further information, whereas a pictorial questionnaire was designed for residents with communication difficulties. Staff reported that gardening was a constructive, outdoor activity that promoted social interaction, physical activity including functional movement and posed cognitive challenges. Half the staff thought the activity was problem free and a third used the garden for therapy. Visitors used the garden to meet with residents socially. Despite their disabilities, HD clients enjoyed growing flourishing flowers and vegetables, labelling plants, being outside in the sun and the quiet of the garden. The garden is valued by all three groups. The study demonstrates the adapted method of gardening is a stimulating and enjoyable activity for people with advanced HD. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  4. Gardening with Huntington's disease clients--creating a programme of winter activities.

    Science.gov (United States)

    Spring, Josephine Anne; Baker, Mark; Dauya, Loreane; Ewemade, Ivie; Marsh, Nicola; Patel, Prina; Scott, Adrienne; Stoy, Nicholas; Turner, Hannah; Viera, Marc; Will, Diana

    2011-01-01

    A programme of garden-related indoor activities was developed to sustain a gardening group for people with mid to late stage Huntington's disease during the winter. The activities were devised by the horticulturist, working empirically, involving the services occupational therapist, physiotherapist, occupational therapy art technician, computer room, recreation and leisure staff. The programme was strongly supported by the nursing and care staff. Feedback on the effectiveness of the activities was sought from the clients, team members and unit staff. The clients' interest in gardening was sustained by a multidisciplinary programme of indoor growing and using plant products in creative activities, computing and group projects. The clients enjoyed all activities except one that they said lacked contact with plants. The inexpensive programme of activities enabled creativity and self-expression, stimulated social contact and helped with therapeutic goals of the clients. In addition, it engaged the multi-disciplinary team and the unit staff, was practical and enhanced the environment.

  5. Colorado Canyons National Conservation Area 2003 visitor use survey: Completion report

    Science.gov (United States)

    Ponds, Phadrea; Gillette, Shana C.; Koontz, Lynne

    2004-01-01

    This report represents the analysis of research conducted by the U.S. Geological Survey (USGS) for the Bureau of Land Management (BLM). The purpose is to provide socio-economic and recreational use information that can be used in the development of a Resource Management Plan (RMP) for the Colorado Canyons National Conservation Area (CCNCA). The results reported here deal primarily with recreation-based activities in four areas: Kokopelli Loops, Rabbit Valley, Loma Boat Launch, and Devil’s Canyon.

  6. Large-Eddy Simulation of Flow and Pollutant Transport in Urban Street Canyons with Ground Heating

    OpenAIRE

    Li, Xian-Xiang; Britter, Rex E.; Koh, Tieh Yong; Norford, Leslie Keith; Liu, Chun-Ho; Entekhabi, Dara; Leung, Dennis Y. C.

    2009-01-01

    Our study employed large-eddy simulation (LES) based on a one-equation subgrid-scale model to investigate the flow field and pollutant dispersion characteristics inside urban street canyons. Unstable thermal stratification was produced by heating the ground of the street canyon. Using the Boussinesq approximation, thermal buoyancy forces were taken into account in both the Navier–Stokes equations and the transport equation for subgrid-scale turbulent kinetic energy (TKE). The LESs were valida...

  7. Mortandad Canyon: Elemental concentrations in vegetation, streambank soils, and stream sediments - 1979

    International Nuclear Information System (INIS)

    Ferenbaugh, R.W.; Gladney, E.S.

    1997-06-01

    In 1979, stream sediments, streambank soils, and streambank vegetation were sampled at 100 m intervals downstream of the outfall of the TA-50 radioactive liquid waste treatment facility in Mortandad Canyon. Sampling was discontinued at a distance of 3260 m at the location of the sediment traps in the canyon. The purpose of the sampling was to investigate the effect of the residual contaminants in the waste treatment facility effluent on elemental concentrations in various environmental media

  8. The functional implications of motor, cognitive, psychiatric, and social problem-solving states in Huntington's disease.

    Science.gov (United States)

    Van Liew, Charles; Gluhm, Shea; Goldstein, Jody; Cronan, Terry A; Corey-Bloom, Jody

    2013-01-01

    Huntington's disease (HD) is a genetic, neurodegenerative disorder characterized by motor, cognitive, and psychiatric dysfunction. In HD, the inability to solve problems successfully affects not only disease coping, but also interpersonal relationships, judgment, and independent living. The aim of the present study was to examine social problem-solving (SPS) in well-characterized HD and at-risk (AR) individuals and to examine its unique and conjoint effects with motor, cognitive, and psychiatric states on functional ratings. Sixty-three participants, 31 HD and 32 gene-positive AR, were included in the study. Participants completed the Social Problem-Solving Inventory-Revised: Long (SPSI-R:L), a 52-item, reliable, standardized measure of SPS. Items are aggregated under five scales (Positive, Negative, and Rational Problem-Solving; Impulsivity/Carelessness and Avoidance Styles). Participants also completed the Unified Huntington's Disease Rating Scale functional, behavioral, and cognitive assessments, as well as additional neuropsychological examinations and the Symptom Checklist-90-Revised (SCL-90R). A structural equation model was used to examine the effects of motor, cognitive, psychiatric, and SPS states on functionality. The multifactor structural model fit well descriptively. Cognitive and motor states uniquely and significantly predicted function in HD; however, neither psychiatric nor SPS states did. SPS was, however, significantly related to motor, cognitive, and psychiatric states, suggesting that it may bridge the correlative gap between psychiatric and cognitive states in HD. SPS may be worth assessing in conjunction with the standard gamut of clinical assessments in HD. Suggestions for future research and implications for patients, families, caregivers, and clinicians are discussed.

  9. Somatostatin receptor 1 and 5 double knockout mice mimic neurochemical changes of Huntington's disease transgenic mice.

    Directory of Open Access Journals (Sweden)

    Padmesh S Rajput

    Full Text Available Selective degeneration of medium spiny neurons and preservation of medium sized aspiny interneurons in striatum has been implicated in excitotoxicity and pathophysiology of Huntington's disease (HD. However, the molecular mechanism for the selective sparing of medium sized aspiny neurons and vulnerability of projection neurons is still elusive. The pathological characteristic of HD is an extensive reduction of the striatal mass, affecting caudate putamen. Somatostatin (SST positive neurons are selectively spared in HD and Quinolinic acid/N-methyl-D-aspartic acid induced excitotoxicity, mimic the model of HD. SST plays neuroprotective role in excitotoxicity and the biological effects of SST are mediated by five somatostatin receptor subtypes (SSTR1-5.To delineate subtype selective biological responses we have here investigated changes in SSTR1 and 5 double knockout mice brain and compared with HD transgenic mouse model (R6/2. Our study revealed significant loss of dopamine and cAMP regulated phosphoprotein of 32 kDa (DARPP-32 and comparable changes in SST, N-methyl-D-aspartic acid receptors subtypes, calbindin and brain nitric oxide synthase expression as well as in key signaling proteins including calpain, phospho-extracellular-signal-regulated kinases1/2, synapsin-IIa, protein kinase C-α and calcineurin in SSTR1/5(-/- and R6/2 mice. Conversely, the expression of somatostatin receptor subtypes, enkephalin and phosphatidylinositol 3-kinases were strain specific. SSTR1/5 appears to be important in regulating NMDARs, DARPP-32 and signaling molecules in similar fashion as seen in HD transgenic mice.This is the first comprehensive description of disease related changes upon ablation of G- protein coupled receptor gene. Our results indicate that SST and SSTRs might play an important role in regulation of neurodegeneration and targeting this pathway can provide a novel insight in understanding the pathophysiology of Huntington's disease.

  10. Large-Eddy Simulation of Flow and Pollutant Transport in Urban Street Canyons with Ground Heating

    Science.gov (United States)

    Li, Xian-Xiang; Britter, Rex E.; Koh, Tieh Yong; Norford, Leslie K.; Liu, Chun-Ho; Entekhabi, Dara; Leung, Dennis Y. C.

    2010-11-01

    Our study employed large-eddy simulation (LES) based on a one-equation subgrid-scale model to investigate the flow field and pollutant dispersion characteristics inside urban street canyons. Unstable thermal stratification was produced by heating the ground of the street canyon. Using the Boussinesq approximation, thermal buoyancy forces were taken into account in both the Navier-Stokes equations and the transport equation for subgrid-scale turbulent kinetic energy (TKE). The LESs were validated against experimental data obtained in wind-tunnel studies before the model was applied to study the detailed turbulence, temperature, and pollutant dispersion characteristics in the street canyon of aspect ratio 1. The effects of different Richardson numbers ( Ri) were investigated. The ground heating significantly enhanced mean flow, turbulence, and pollutant flux inside the street canyon, but weakened the shear at the roof level. The mean flow was observed to be no longer isolated from the free stream and fresh air could be entrained into the street canyon at the roof-level leeward corner. Weighed against higher temperature, the ground heating facilitated pollutant removal from the street canyon.

  11. Effect of stable stratification on dispersion within urban street canyons: A large-eddy simulation

    Science.gov (United States)

    Li, Xian-Xiang; Britter, Rex; Norford, Leslie K.

    2016-11-01

    This study employs a validated large-eddy simulation (LES) code with high tempo-spatial resolution to investigate the effect of a stably stratified roughness sublayer (RSL) on scalar transport within an urban street canyon. The major effect of stable stratification on the flow and turbulence inside the street canyon is that the flow slows down in both streamwise and vertical directions, a stagnant area near the street level emerges, and the vertical transport of momentum is weakened. Consequently, the transfer of heat between the street canyon and overlying atmosphere also gets weaker. The pollutant emitted from the street level 'pools' within the lower street canyon, and more pollutant accumulates within the street canyon with increasing stability. Under stable stratification, the dominant mechanism for pollutant transport within the street canyon has changed from ejections (flow carries high-concentration pollutant upward) to unorganized motions (flow carries high-concentration pollutant downward), which is responsible for the much lower dispersion efficiency under stable stratifications.

  12. Event-driven sediment flux in Hueneme and Mugu submarine canyons, southern California

    Science.gov (United States)

    Xu, J. P.; Swarzenski, P.W.; Noble, M.; Li, A.-C.

    2010-01-01

    Vertical sediment fluxes and their dominant controlling processes in Hueneme and Mugu submarine canyons off south-central California were assessed using data from sediment traps and current meters on two moorings that were deployed for 6 months during the winter of 2007. The maxima of total particulate flux, which reached as high as 300+ g/m2/day in Hueneme Canyon, were recorded during winter storm events when high waves and river floods often coincided. During these winter storms, wave-induced resuspension of shelf sediment was a major source for the elevated sediment fluxes. Canyon rim morphology, rather than physical proximity to an adjacent river mouth, appeared to control the magnitude of sediment fluxes in these two submarine canyon systems. Episodic turbidity currents and internal bores enhanced sediment fluxes, particularly in the lower sediment traps positioned 30 m above the canyon floor. Lower excess 210Pb activities measured in the sediment samples collected during periods of peak total particulate flux further substantiate that reworked shelf-, rather than newly introduced river-borne, sediments supply most of the material entering these canyons during storms.

  13. Impact of aspect ratio and solar heating on street canyon air temperature

    International Nuclear Information System (INIS)

    Memon, R.A.; Lal, K.

    2011-01-01

    The results obtained from RNG (Re-Normalization Group) version of k-and turbulence model are reported in this study. The model is adopted to elucidate the impact of different building aspect ratios (i.e., ratio of building-height-to-street-canyon-width) and solar heating on temperatures in street canyon. The validation of Navier-Stokes and energy an sport equations showed that the model prediction for air-temperature and ambient wind provides reasonable accuracy. The model was applied on AR (Aspect Ratios) one to eight and surface temperature difference (delta and theta/sub s-a/)) of 2 -8. Notably, air-temperatures were higher in high AR street canyons in particular on the leeward side of the street canyon. Further investigation showed that the difference between the air-temperature 'high and low AR street canyons (AR) was positive and high with higher delta and theta/sub s-a/) conversely, the AR become negative and low gradually with lower values of delta and theta(/sub s-a/). These results could be very beneficial for the city and regional planners, civil engineers Id HVAC experts who design street canyons and strive for human thermal comfort with minimum possible energy requirements. (author)

  14. Earthquakes drive large-scale submarine canyon development and sediment supply to deep-ocean basins.

    Science.gov (United States)

    Mountjoy, Joshu J; Howarth, Jamie D; Orpin, Alan R; Barnes, Philip M; Bowden, David A; Rowden, Ashley A; Schimel, Alexandre C G; Holden, Caroline; Horgan, Huw J; Nodder, Scott D; Patton, Jason R; Lamarche, Geoffroy; Gerstenberger, Matthew; Micallef, Aaron; Pallentin, Arne; Kane, Tim

    2018-03-01

    Although the global flux of sediment and carbon from land to the coastal ocean is well known, the volume of material that reaches the deep ocean-the ultimate sink-and the mechanisms by which it is transferred are poorly documented. Using a globally unique data set of repeat seafloor measurements and samples, we show that the moment magnitude ( M w ) 7.8 November 2016 Kaikōura earthquake (New Zealand) triggered widespread landslides in a submarine canyon, causing a powerful "canyon flushing" event and turbidity current that traveled >680 km along one of the world's longest deep-sea channels. These observations provide the first quantification of seafloor landscape change and large-scale sediment transport associated with an earthquake-triggered full canyon flushing event. The calculated interevent time of ~140 years indicates a canyon incision rate of 40 mm year -1 , substantially higher than that of most terrestrial rivers, while synchronously transferring large volumes of sediment [850 metric megatons (Mt)] and organic carbon (7 Mt) to the deep ocean. These observations demonstrate that earthquake-triggered canyon flushing is a primary driver of submarine canyon development and material transfer from active continental margins to the deep ocean.

  15. Transport and deposition of plutonium-contaminated sediments by fluvial processes, Los Alamos Canyon, New Mexico

    International Nuclear Information System (INIS)

    Graf, W.L.

    1996-01-01

    Between 1945 and 1952 the development of nuclear weapons at Los Alamos National Laboratory, New Mexico, resulted in the disposal of plutonium into the alluvium of nearby Acid and (to a lesser degree) DP Canyons. The purpose of this paper is to explore the connection between the disposal sites and the main river, a 20 km link formed by the fluvial system of Acid, Pueblo, DP, and Los Alamos Canyons. Empirical data from 15 yr of annual sediment sampling throughout the canyon system has produced 458 observations of plutonium concentration in fluvial sediments. These data show that, overall, mean plutonium concentrations in fluvial sediment decline from 10,000 fCi/g near the disposal area to 100 fCi/g at the confluence of the canyon system and the Rio Grande. Simulations using a computer model for water, sediment, and plutonium routing in the canyon system show that discharges as large as the 25 yr event would fail to develop enough transport capacity to completely remove the contaminated sediments from Pueblo Canyon. Lesser flows would move some materials to the Rio Grande by remobilization of stored sediments. The simulations also show that the deposits and their contaminants have a predictable geography because they occur where stream power is low, hydraulic resistance is high, and the geologic and/or geomorphic conditions provide enough space for storage. 38 refs., 13 figs., 1 tab

  16. Bedrock Canyons Carved by the Largest Known Floods on Earth and Mars

    Science.gov (United States)

    Lamb, M. P.; Lapôtre, M. G. A.; Larsen, I. J.; Williams, R. M. E.

    2017-12-01

    The surface of Earth is a dynamic and permeable interface where the rocky crust is sculpted by ice, wind and water resulting in spectacular mountain ranges, vast depositional basins and environments that support life. These landforms and deposits contain a rich, yet incomplete, record of Earth history that we are just beginning to understand. Some of the most dramatic landforms are the huge bedrock canyons carved by catastrophic floods. On Mars, similar bedrock canyons, known as Outflow Channels, are the most important indicators of large volumes of surface water in the past. Despite their importance and now decades of observations of canyon morphology, we lack a basic understanding of how the canyons formed, which limits our ability to reconstruct flood discharge, duration and water volume. In this presentation I will summarize recent work - using mechanistic numerical models and field observations - that suggests that bedrock canyons carved by megafloods rapidly evolve to a size and shape such that boundary shear stresses just exceed that required to entrain fractured blocks of rock. The threshold shear stress constraint allows for quantitative reconstruction of the largest known floods on Earth and Mars, and implies far smaller discharges than previous methods that assume flood waters fully filled the canyons to high water marks.

  17. Klondike III/Biglow Canyon Wind Integration Project; Final Environmental Impact Statement, September 2006.

    Energy Technology Data Exchange (ETDEWEB)

    United States. Bonneville Power Administration

    2006-09-01

    BPA has been asked by PPM Energy, Inc. to interconnect 300 megawatts (MW) of electricity generated from the proposed Klondike III Wind Project to the Federal Columbia River Transmission System. Orion Energy LLC has also asked BPA to interconnect 400 MW of electricity from its proposed Biglow Canyon Wind Farm, located north and east of the proposed Klondike III Wind Project. (Portland General Electric recently bought the rights to develop the proposed Biglow Canyon Wind Farm from Orion Energy, LLC.) Both wind projects received Site Certificates from the Oregon Energy Facility Siting Council on June 30, 2006. To interconnect these projects, BPA would need to build and operate a 230-kV double-circuit transmission line about 12 miles long, expand one substation and build one new substation. The wind projects would require wind turbines, substation(s), access roads, and other facilities. Two routes for the transmission line are being considered. Both begin at PPM's Klondike Schoolhouse Substation then travel north (Proposed Action) or north and westerly (Middle Alternative) to a new BPA 230-kV substation next to BPA's existing John Day 500-kV Substation. BPA is also considering a No Action Alternative in which BPA would not build the transmission line and would not interconnect the wind projects. The proposed BPA and wind projects would be located on private land, mainly used for agriculture. If BPA decides to interconnect the wind projects, construction of the BPA transmission line and substation(s) could commence as early as the winter of 2006-07. Both wind projects would operate for much of each year for at least 20 years. The proposed projects would generally create no or low impacts. Wildlife resources and local visual resources are the only resources to receive an impact rating other than ''none'' or ''low''. The low to moderate impacts to wildlife are from the expected bird and bat mortality and the cumulative

  18. Geologic map and upper Paleozoic stratigraphy of the Marble Canyon area, Cottonwood Canyon quadrangle, Death Valley National Park, Inyo County, California

    Science.gov (United States)

    Stone, Paul; Stevens, Calvin H.; Belasky, Paul; Montañez, Isabel P.; Martin, Lauren G.; Wardlaw, Bruce R.; Sandberg, Charles A.; Wan, Elmira; Olson, Holly A.; Priest, Susan S.

    2014-01-01

    This geologic map and pamphlet focus on the stratigraphy, depositional history, and paleogeographic significance of upper Paleozoic rocks exposed in the Marble Canyon area in Death Valley National Park, California. Bedrock exposed in this area is composed of Mississippian to lower Permian (Cisuralian) marine sedimentary rocks and the Jurassic Hunter Mountain Quartz Monzonite. These units are overlain by Tertiary and Quaternary nonmarine sedimentary deposits that include a previously unrecognized tuff to which we tentatively assign an age of late middle Miocene (~12 Ma) based on tephrochronologic analysis, in addition to the previously recognized Pliocene tuff of Mesquite Spring. Mississippian and Pennsylvanian rocks in the Marble Canyon area represent deposition on the western continental shelf of North America. Mississippian limestone units in the area (Tin Mountain, Stone Canyon, and Santa Rosa Hills Limestones) accumulated on the outer part of a broad carbonate platform that extended southwest across Nevada into east-central California. Carbonate sedimentation was interrupted by a major eustatic sea-level fall that has been interpreted to record the onset of late Paleozoic glaciation in southern Gondwana. Following a brief period of Late Mississippian clastic sedimentation (Indian Springs Formation), a rise in eustatic sea level led to establishment of a new carbonate platform that covered most of the area previously occupied by the Mississippian platform. The Pennsylvanian Bird Spring Formation at Marble Canyon makes up the outer platform component of ten third-order (1 to 5 m.y. duration) stratigraphic sequences recently defined for the regional platform succession. The regional paleogeography was fundamentally changed by major tectonic activity along the continental margin beginning in middle early Permian time. As a result, the Pennsylvanian carbonate shelf at Marble Canyon subsided and was disconformably overlain by lower Permian units (Osborne Canyon and

  19. Dissolution of Material and Test reactor Fuel in an H-Canyon Dissolver

    Energy Technology Data Exchange (ETDEWEB)

    Daniel, W. E. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL); Rudisill, T. S. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL); O' Rourke, P. E. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL)

    2017-01-26

    In an amended record of decision for the management of spent nuclear fuel (SNF) at the Savannah River Site, the US Department of Energy has authorized the dissolution and recovery of U from 1000 bundles of Al-clad SNF. The SNF is fuel from domestic and foreign research reactors and is typically referred to as Material Test Reactor (MTR) fuel. Bundles of MTR fuel containing assemblies fabricated from U-Al alloys (or other U compounds) are currently dissolved using a Hg-catalyzed HNO3 flowsheet. Since the development of the existing flowsheet, improved experimental methods have been developed to more accurately characterize the offgas composition and generation rate during laboratory dissolutions. Recently, these new techniques were successfully used to develop a flowsheet for the dissolution of High Flux Isotope Reactor (HFIR) fuel. Using the data from the HFIR dissolution flowsheet development and necessary laboratory experiments, the Savannah River National Laboratory (SRNL) was requested to define flowsheet conditions for the dissolution of MTR fuels. With improved offgas characterization techniques, SRNL will be able define the number of bundles of fuel which can be charged to an H-Canyon dissolver with much less conservatism.

  20. Huntington's Disease

    Science.gov (United States)

    ... monitor a disease) for HD. A large and related NINDS-supported study aims to identify additional genetic factors in people that influence the course of the disease. Other research hopes to identify variations in the genomes of individuals with HD that may point to new targets ...

  1. California State Waters Map Series—Monterey Canyon and vicinity, California

    Science.gov (United States)

    Dartnell, Peter; Maier, Katherine L.; Erdey, Mercedes D.; Dieter, Bryan E.; Golden, Nadine E.; Johnson, Samuel Y.; Hartwell, Stephen R.; Cochrane, Guy R.; Ritchie, Andrew C.; Finlayson, David P.; Kvitek, Rikk G.; Sliter, Ray W.; Greene, H. Gary; Davenport, Clifton W.; Endris, Charles A.; Krigsman, Lisa M.; Dartnell, Peter; Cochran, Susan A.

    2016-06-10

    IntroductionIn 2007, the California Ocean Protection Council initiated the California Seafloor Mapping Program (CSMP), designed to create a comprehensive seafloor map of high-resolution bathymetry, marine benthic habitats, and geology within the 3-nautical-mile limit of California’s State Waters. The CSMP approach is to create highly detailed seafloor maps through collection, integration, interpretation, and visualization of swath bathymetry data, acoustic backscatter, seafloor video, seafloor photography, high-resolution seismic-reflection profiles, and bottom-sediment sampling data. The map products display seafloor morphology and character, identify potential marine benthic habitats, and illustrate both the surficial seafloor geology and shallow subsurface geology.The Monterey Canyon and Vicinity map area lies within Monterey Bay in central California. Monterey Bay is one of the largest embayments along the west coast of the United States, spanning 36 km from its northern to southern tips (in Santa Cruz and Monterey, respectively) and 20 km along its central axis. Not only does it contain one of the broadest sections of continental shelf along California’s coast, it also contains Monterey Canyon, one of the largest and deepest submarine canyons in the world. Note that the California’s State Waters limit extends farther offshore between Santa Cruz and Monterey so that it encompasses all of Monterey Bay.The coastal area within the map area is lightly populated. The community of Moss Landing (population, 204) hosts the largest commercial fishing fleet in Monterey Bay in its harbor. The map area also includes parts of the cities of Marina (population, about 20,000) and Castroville (population, about 6,500). Fertile lowlands of the Salinas River and Pajaro River valleys largely occupy the inland part of the map area, and land use is primarily agricultural.The offshore part of the map area lies completely within the Monterey Bay National Marine Sanctuary. The

  2. An Investigation of Amphitheater-Headed Canyon Distribution, Morphology Variation, and Longitudinal Profile Controls in Escalante and Tarantula Mesa, Utah.

    Science.gov (United States)

    Ryan, A. J.; Whipple, K. X.

    2014-12-01

    Amphitheater-headed canyons are primarily distinguished from typical fluvial channels by their abrupt headwall terminations. A key goal in the study of river canyons is to establish a reliable link between form and formation processes. This is of particular significance for Mars, where, if such links can be established, amphitheater-headed canyons could be used to determine ancient erosion mechanisms and, by inference, climate conditions. Type examples in arid regions on Earth, such as in Escalante River, Utah, previously have been interpreted as products of groundwater seepage erosion. We investigate amphitheater-headed canyons in Escalante and Tarantula Mesa where variations in canyon head morphology may hold clues for the relative roles of rock properties and fluvial and groundwater processes. In lower Escalante, amphitheaters are only present where canyons have breached the Navajo Sandstone - Kayenta Formation contact. In some canyons, amphitheater development appears to have been inhibited by an abundance of coarse bedload. In Tarantula Mesa, canyons have a variety of headwalls, from amphitheaters to stepped knickzones. Headwall morphology distribution is directly related to the spatially variable presence of knickpoint-forming, fine-grained interbeds within cliff-forming sandstones. Amphitheaters only form where the sandstone unit is undisrupted by these interbeds. Finally, most canyons in Escalante and Tarantula Mesa, regardless of substrate lithology, amphitheater presence, or groundwater spring intensity, are well described by a slope-area power law relationship with regionally constant concavity and normalized steepness indices. This suggests that all channels here are subject to the same erosion rates, independent of groundwater weathering intensity. Thus: 1) variations in canyon headwall form do not necessary relate to differences in fluvial history, 2) stratigraphic variations are clearly of importance in sedimentary canyon systems, and 3) although

  3. Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure?

    Science.gov (United States)

    Morea, Veronica; Bidollari, Eris; Colotti, Gianni; Fiorillo, Annarita; Rosati, Jessica; De Filippis, Lidia; Squitieri, Ferdinando; Ilari, Andrea

    2017-07-01

    Huntington's disease (HD) or Huntington's chorea is the most common inherited, dominantly transmitted, neurodegenerative disorder. It is caused by increased CAG repeats number in the gene coding for huntingtin (Htt) and characterized by motor, behaviour and psychiatric symptoms, ultimately leading to death. HD patients also exhibit alterations in glucose and energetic metabolism, which result in pronounced weight loss despite sustained calorie intake. Glucose metabolism decreases in the striatum of all the subjects with mutated Htt, but affects symptom presentation only when it drops below a specific threshold. Recent evidence points at defects in glucose uptake by the brain, and especially by neurons, as a relevant component of central glucose hypometabolism in HD patients. Here we review the main features of glucose metabolism and transport in the brain in physiological conditions and how these processes are impaired in HD, and discuss the potential ability of strategies aimed at increasing intracellular energy levels to counteract neurological and motor degeneration in HD patients.

  4. Street canyon ventilation control by proper planning and development

    Directory of Open Access Journals (Sweden)

    Balakin Vladimir Vasil'evich

    2014-05-01

    Full Text Available The objective of street canyon ventilation control in major streets is a tool of air pollution prevention in them, protection of housing areas from excessive wind or preservation and intensification of existing wind speed in case of insufficient ventilation. The maximum permissible concentration of car exhaust pollutants with wind speed within comfortable and permissible values by physiological and hygienic criteria, are ensured as from 40 to 70 % of thoroughfares in major cities. The dependence of air pollution level on wind speed is comparable to its dependence on traffic intensity and ratio of buildings height (H to street width. But one has to take into account that, if the wind blows across the street, vortices form within the street canyon, which results in higher concentration of car exhaust pollutants near the downwind buildings. The objective of this work is to find the functional dependences of wind speed in a major street on its width and density of buildings, and also to find out which street configurations are favorable for formation of closed air circulation within it, resulting in insufficient aeration. The experimental research was done on a site for large-scale modeling of built-up urban territory, using cup anemometers. The coefficients of dependence of wind speed within a street on the types of buildings and on the street width were obtained. Characteristics of street layouts for control of aeration were determined. Building density rates for maximizing or optimizing the wind speed were determined. Street layouts are considered where stable vortices form between the buildings. For example, vortices within the street canyon’s cross-section appear when buildings squarish in ground plan situated far apart are replaced by oblong ones with the minimum allowed intervals of 15 meters between them (for 5-storeyed buildings; or intervals equal to the buildings’ height, or where the buildings are long and close together. With

  5. SRTM Colored Height and Shaded Relief: Pinon Canyon region, Colorado

    Science.gov (United States)

    2001-01-01

    Erosional features are prominent in this view of southern Colorado taken by the Shuttle Radar Topography Mission (SRTM). The area covers about 20,000 square kilometers and is located about 50 kilometers south of Pueblo, Colorado. The prominent mountains near the left edge of the image are the Spanish Peaks, remnants of a 20 million year old volcano. Rising 2,100 meters (7,000 ft) above the plains to the east, these igneous rock formations with intrusions of eroded sedimentary rock historically served as guiding landmarks for travelers on the Mountain Branch of the Santa Fe Trail.Near the center of the image is the Pinon Canyon Maneuver Site, a training area for soldiers of the U.S. Army from nearby Fort Carson. The site supports a diverse ecosystem with large numbers of big and small game, fisheries, non-game wildlife, forest, range land and mineral resources. It is bounded on the east by the dramatic topography of the Purgatoire River Canyon, a 100 meter (328 foot) deep scenic red canyon with flowing streams, sandstone formations, and exposed geologic processes.Two visualization methods were combined to produce this image: shading and color coding of topographic height. The shade image was derived by computing topographic slope in the northwest-southeast direction. Southern slopes appear bright and northern slopes appear dark. Color coding is directly related to topographic height, with blue and green at the lower elevations, rising through yellow and brown to white at the highest elevations.Elevation data used in this image was acquired by the Shuttle Radar Topography Mission (SRTM) aboard the Space Shuttle Endeavour, launched on February 11, 2000. SRTM used the same radar instrument that comprised the Spaceborne Imaging Radar-C/X-Band Synthetic Aperture Radar (SIR-C/X-SAR)that flew twice on the Space Shuttle Endeavour in 1994. SRTM was designed to collect three-dimensional measurements of the Earth's surface. To collect the 3-D data, engineers added a 60-meter

  6. Morphology and sediment dynamics of the Capbreton canyon (Bay of Biscay, SW France)

    Science.gov (United States)

    Gaudin, M.; Umr 5805; Ifremer Team

    2003-04-01

    The Canyon of Capbreton extending in the Bay of Biscay (SW France) is the deepest canyon in the world. Its structure and morphology was studied using new multibeam bathymetry, acoustic imagery and high-resolution seismic data. The canyon head appears only 250 m away from the coast line and runs westward parallel to the north coast of Spain for 160 km due to structural control, then turns northward, widens and abruptly disappears in the continental rise by 3500 m water depth. Its northern margin is flat and progrades clearly westward. Conversely the southern margin is steep and progrades towards the north (i.e. towards the canyon). Down to 800 m water depth, the canyon deeply incises the continental shelf and the axial channel is meandering (sinuosity of 1.9). The canyon shows both major and minor stream beds, perched tributary valleys, nested terraces and abandoned meanders. The terraces have three morphologies: (1) flat, (2) with a raised side or (3) with a horseshoe structure. These morphologies have been interpreted as overbank deposits or nested levees (1 and 2) or as the result of meander abandon (3). Terraces of types (1) and (2) contain mainly fine deposits resulting from decantation of the top of turbulent surges that flow in the canyon. Westward (800 to 2000 m water depth) the main talweg remains sinuous (1.7). On the southern margin, several straight or slightly sinuous S-N tributary valleys are followed by alignments of pockmarks that also indicate a structural control. On the northern margin, a single large tributary valley with a sinuous central talweg, flowing from the upper Aquitaine continental slope, is interpreted as a giant slump scar due to sediment instability. This valley is bordered to the west by a topographic high with sediment waves on the external flank that might be interpreted as a sedimentary levee. The canyon recorded a recent turbidite activity. An 18 cm-thick turbidite was deposited at 650 m water depth by a turbidity current

  7. Macrofaunal Patterns in and around du Couedic and Bonney Submarine Canyons, South Australia.

    Directory of Open Access Journals (Sweden)

    Kathleen E Conlan

    Full Text Available Two South Australian canyons, one shelf-incising (du Couedic and one slope-limited (Bonney were compared for macrofaunal patterns on the shelf and slope that spanned three water masses. It was hypothesized that community structure would (H1 significantly differ by water mass, (H2 show significant regional differences and (H3 differ significantly between interior and exterior of each canyon. Five hundred and thirty-one species of macrofauna ≥ 1 mm were captured at 27 stations situated in depth stratified transects inside and outside the canyons from 100 to 1500 m depth. The macrofauna showed a positive relationship to depth in abundance, biomass, species richness and community composition while taxonomic distinctness and evenness remained high at all depths. Biotic variation on the shelf was best defined by variation in bottom water primary production while sediment characteristics and bottom water oxygen, temperature and nutrients defined biotic variation at greater depth. Community structure differed significantly (p<0.01 among the three water masses (shelf-flowing South Australian current, upper slope Flinders current and lower slope Antarctic Intermediate Water (H1. Although community differences between the du Couedic and Bonney regions were marginally above significance at p = 0.05 (H2, over half of the species captured were unique to each region. This supports the evidence from fish and megafaunal distributions that the du Couedic and Bonney areas are in different bioregions. Overall, the canyon interiors were not significantly different in community composition from the exterior (H3. However, both canyons had higher abundance and/or biomass, increased species dominance, different species composition and coarser sediments near the canyon heads compared to outside the canyons at the same depth (500 m, suggestive of heightened currents within the canyons that influence community composition there. At 1000-1500 m, the canyon interiors were

  8. Numerical Simulation of Recent Turbidity Currents in the Monterey Canyon System, Offshore California

    Science.gov (United States)

    Heimsund, S.; Xu, J.; Nemec, W.

    2007-12-01

    The method of computational fluid dynamics (CFD) has been used, in the form of a 3D numerical model (Flow- 3D®), to perform a full-scale simulation of turbidity currents measured in December 2002 by three moorings in the Soquel and Monterey canyons. The model was verified by simulation of laboratory flows, and was upscaled to the Monterey Canyon system on the basis of high-resolution bathymetric data and flow measurements. The measured velocity profiles were sufficient to assess the flow thickness, initial velocity and duration in the canyon head zone. A computational grid with a highest feasible resolution was used, and both bathymetry and hydrostatic pressure were accounted for. The volumetric sediment concentration and exact grain- size composition of the flows were unknown, and thus a range of values for the initial concentration and bed roughness were assumed and assessed on a trial-and-error basis. The simulations reveal the behavior of a turbidity current along its descent path, including its local hydraulic characteristics (the 3D field of velocity, sediment concentration, shear stress, strain rate, and dynamic viscosity, as well as the magnitude of velocity and turbulent shear). The results confirm that the velocity structure of turbidity current is highly sensitive to variation in seafloor topography. The December 17th flow in the Soquel Canyon appears to have lost capacity by dilution over a relatively short distance and shown significant velocity fluctuations, which is attributed to the rugged topography of the canyon floor. A major loss of momentum occurred when the flow plunged at high angle into the Monterey Canyon, crashing against its bend's southern wall. The December 20th flow in the Monterey Canyon, in contrast, developed a considerably longer body and strongly accelerated towards the canyon's sharp second bend before crashing against its western wall. The mooring data show a down-canyon decline of velocity and suggest gradual waning, but the

  9. Huntington's disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads database.

    Science.gov (United States)

    Kalathur, Ravi Kiran Reddy; Hernández-Prieto, Miguel A; Futschik, Matthias E

    2012-06-28

    Huntington's disease (HD) is a fatal progressive neurodegenerative disorder caused by the expansion of the polyglutamine repeat region in the huntingtin gene. Although the disease is triggered by the mutation of a single gene, intensive research has linked numerous other genes to its pathogenesis. To obtain a systematic overview of these genes, which may serve as therapeutic targets, CHDI Foundation has recently established the HD Research Crossroads database. With currently over 800 cataloged genes, this web-based resource constitutes the most extensive curation of genes relevant to HD. It provides us with an unprecedented opportunity to survey molecular mechanisms involved in HD in a holistic manner. To gain a synoptic view of therapeutic targets for HD, we have carried out a variety of bioinformatical and statistical analyses to scrutinize the functional association of genes curated in the HD Research Crossroads database. In particular, enrichment analyses were performed with respect to Gene Ontology categories, KEGG signaling pathways, and Pfam protein families. For selected processes, we also analyzed differential expression, using published microarray data. Additionally, we generated a candidate set of novel genetic modifiers of HD by combining information from the HD Research Crossroads database with previous genome-wide linkage studies. Our analyses led to a comprehensive identification of molecular mechanisms associated with HD. Remarkably, we not only recovered processes and pathways, which have frequently been linked to HD (such as cytotoxicity, apoptosis, and calcium signaling), but also found strong indications for other potentially disease-relevant mechanisms that have been less intensively studied in the context of HD (such as the cell cycle and RNA splicing, as well as Wnt and ErbB signaling). For follow-up studies, we provide a regularly updated compendium of molecular mechanism, that are associated with HD, at http://hdtt.sysbiolab.eu Additionally

  10. Study of protein O-GlcNAcylation in the brain tissue in Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Ondrušková, N.; Rodinová, M.; Kratochvílová, H.; Klempíř, J.; Roth, J.; Motlík, Jan; Radoslav, M.; Zeman, J.; Hansíková, H.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 20-20 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk(CZ) 7F14308; GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * glycosylation * N-acetylglucosamine Subject RIV: FH - Neurology

  11. Pre-clinical evaluation of AAV5-miHTT gene therapy of Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Konstantinová, P.; Miniarikova, J.; Blits, B.; Zimmer, V.; Spoerl, A.; Southwell, A.; Hayden, M.; van Deventer, S.; Deglon, N.; Motlík, Jan; Juhás, Štefan; Juhásová, Jana; Richard, Ch.; Petry, H.

    2015-01-01

    Roč. 78, Supl 2 (2015), s. 8-8 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * gene therapy * AAV5-miHTT Subject RIV: EB - Genetics ; Molecular Biology

  12. Decreased mitochondrial density and ultrastructural changes of mitochondria in cultivated skin fibroblasts of patients with Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Rodinová, M.; Marková, M.; Kratochvílová, H.; Kučerová, I.; Tesařová, M.; Lišková, Irena; Klempíř, J.; Roth, J.; Zeman, J.; Hansíková, H.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 20-21 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * fibroblasts * mitochondrial ultrastructure Subject RIV: FH - Neurology

  13. The effects of wildfire on the peak streamflow magnitude and frequency, Frijoles and Capulin Canyons, Bandelier National Monument, New Mexico

    Science.gov (United States)

    Veenhuis, J.E.

    2004-01-01

    In June of 1977, the La Mesa fire burned 15,270 acres in and around Frijoles Canyon, Bandelier National Monument and the adjacent Santa Fe National Forest, New Mexico. The Dome fire occurred in April of 1996 in Bandelier National Monument, burned 16,516 acres in Capulin Canyon and the surrounding Dome Wilderness area. Both canyons are characterized by extensive archeological artifacts, which could be threatened by increased runoff and accelerated rates of erosion after a wildfire. The U.S. Geological Survey (USGS) in cooperation with the National Park Service monitored the fires' effects on streamflow in both canyons. Copyright 2004 ASCE.

  14. Environmental and human impact on the sedimentary dynamic in the Rhone Delta subaquatic canyons (France-Switzerland)

    Science.gov (United States)

    Arantegui, A.; Corella, J. P.; Loizeau, J. L.; Anselmetti, F. S.; Girardclos, S.

    2012-04-01

    Deltas are very sensitive environments and highly vulnerable to variations in water discharge and the amount of suspended sediment load provided by the delta-forming currents. Human activities in the watershed, such as building of dams and irrigation ditches, or river bed deviations, may affect the discharge regime and sediment input, thus affecting delta growth. Underwater currents create deeply incised canyons cutting into the delta lobes. Understanding the sedimentary processes in these subaquatic canyons is crucial to reconstruct the fluvial evolution and human impact on deltaic environments and to carry out a geological risk assessment related to mass movements, which may affect underwater structures and civil infractructure. Recently acquired high-resolution multibeam bathymetry on the Rhone Delta in Lake Geneva (Sastre et al. 2010) revealed the complexity of the underwater morphology formed by active and inactive canyons first described by Forel (1892). In order to unravel the sedimentary processes and sedimentary evolution in these canyons, 27 sediment cores were retrieved in the distal part of each canyon and in the canyon floor/levee complex of the active canyon. Geophysical, sedimentological, geochemical and radiometric dating techniques were applied to analyse these cores. Preliminary data show that only the canyon originating at the current river mouth is active nowadays, while the others remain inactive since engineering works in the watershed occurred, confirming Sastre et al. (2010). However, alternating hemipelagic and turbiditic deposits on the easternmost canyons, evidence underflow processes during the last decades as well. Two canyons, which are located close to the Rhone river mouth, correspond to particularly interesting deeply incised crevasse channels formed when the underwater current broke through the outer bend of a meander in the proximal northern levee. In these canyons, turbidites occur in the sediment record indicating ongoing

  15. Alpha-7 nicotinic acetylcholine receptor agonist treatment in a rat model of Huntington's disease and involvement of heme oxygenase-1

    Directory of Open Access Journals (Sweden)

    Laura Foucault-Fruchard

    2018-01-01

    Full Text Available Neuroinflammation is a common element involved in the pathophysiology of neurodegenerative diseases. We recently reported that repeated alpha-7 nicotinic acetylcholine receptor (α7nAChR activations by a potent agonist such as PHA 543613 in quinolinic acid-injured rats exhibited protective effects on neurons. To further investigate the underlying mechanism, we established rat models of early-stage Huntington's disease by injection of quinolinic acid into the right striatum and then intraperitoneally injected 12 mg/kg PHA 543613 or sterile water, twice a day during 4 days. Western blot assay results showed that the expression of heme oxygenase-1 (HO-1, the key component of the cholinergic anti-inflammatory pathway, in the right striatum of rat models of Huntington's disease subjected to intraperitoneal injection of PHA 543613 for 4 days was significantly increased compared to the control rats receiving intraperitoneal injection of sterile water, and that the increase in HO-1 expression was independent of change in α7nAChR expression. These findings suggest that HO-1 expression is unrelated to α7nAChR density and the increase in HO-1 expression likely contributes to α7nAChR activation-related neuroprotective effect in early-stage Huntington's disease.

  16. Trees as environmental modifier to improve street canyon for pedestrian activities in Muscat

    Science.gov (United States)

    Khudhayer, Wael A.; Shaaban, Awni K.; Sukor, Nur Sabahiah Abdul

    2017-10-01

    Street shading efficiency is a function of orientation and profile proportion of its height to width. Under high sun altitude conditions, minimization of solar irradiance within the urban environment may often be a significant criterion in urban design. This reduction in solar irradiance achieved when the obstruction angle is large (high H/W ratio, H=height, W=width). High H/W values often lessen the solar access to streets. The horizontal sprawl of Muscat region is an example of low H/W ratio represented the remarkable challenge that causes the lack of shading rates in the urban street. This characteristic proliferates the negative impact on the pedestrian activities in the urban street. This research aims to improve the morphology of the street to promote the pedestrian behavior. The amendment based on suggesting different configurations of trees to increase effective shading of the urban street in Muscat. The street canyon abstracted into a virtual elongated channel formed of floor and walls of equal heights on both sides. Four street orientations (E/W, N/S, NE/SW, NW/SE) and three H/W ratio (0.5,1 and 2) are considered sufficient representative of street typologies. A mathematical model developed for calculation of shading efficiency of each street canyon. The trees assumed in this study as canyon's modifier to adjust the low H/W ratio of a street canyon to a higher one. Local trees and other plants in Muscat were studied concerning their morphology. The analysis selected two case study in Muscat to investigate the shading performance of their street canyons subsequently propose the modifications to improve it. The research concluded that the suggested changes of the street canyon by using a particular type of trees could increase the H/W ratio of street canyon significantly.

  17. Estimation of health damage due to emission of air pollutants by cars: the canyon effect

    Energy Technology Data Exchange (ETDEWEB)

    Spadaro, J.V. [Ecole des Mines, Centre d' Energetique, Paris, 75 (France); Rabl, A.

    1999-07-01

    Since current epidemiological evidence suggests that air pollution has harmful effects even at typical ambient concentrations and the dispersion is significant over hundreds to thousands of km, the estimation of total health damage involves consideration of local and regional effects. In recent years, several estimates have been published of health damage due to air pollution from cars, in particular by Delucchi et al of UC Davis and by the ExternE Project of the European Commission. To capture the geographic extent of pollutant dispersion, local and regional models have been used in combination. The present paper addresses a potentially significant contribution of the total damage, not yet taken into account in these studies: the increased concentration of pollutants inside urban street canyons. This canyon effect is appreciable only for primary pollutants, the time constants for the formation of secondary pollutants being long compared to the residence time in the canyon. We assumed linearity of incremental health impact with incremental concentration, in view of the lack of epidemiological evidence for no-effect thresholds or significant deviations from linearity at typical ambient concentrations; therefore, only long term average concentrations matter. We use the FLUENT software to model the dispersion inside a street canyon for a wide range of rectangular geometries and wind velocities. Our results suggest that the canyon effect is of marginal significance for total damages, the contribution of the canyon effect being roughly 10 to 20% of the total. The relative importance of the canyon effect is, of course, highly variable with local conditions; it could be much smaller but it is unlikely to add more than 100% to the flat terrain estimate. (Author)

  18. Effects of building roof greening on air quality in street canyons

    Science.gov (United States)

    Baik, Jong-Jin; Kwak, Kyung-Hwan; Park, Seung-Bu; Ryu, Young-Hee

    2012-12-01

    Building roof greening is a successful strategy for improving urban thermal environment. It is of theoretical interest and practical importance to study the effects of building roof greening on urban air quality in a systematic and quantitative way. In this study, we examine the effects of building roof greening on air quality in street canyons using a computational fluid dynamics (CFD) model that includes the thermodynamic energy equation and the transport equation of passive, non-reactive pollutants. For simplicity, building roof greening is represented by specified cooling. Results for a simple building configuration with a street canyon aspect ratio of one show that the cool air produced due to building roof greening flows into the street canyon, giving rise to strengthened street canyon flow. The strengthened street canyon flow enhances pollutant dispersion near the road, which decreases pollutant concentration there. Thus, building roof greening improves air quality near the road. The degree of air quality improvement near the road increases as the cooling intensity increases. In the middle region of the street canyon, the air quality can worsen when the cooling intensity is not too strong. Results for a real urban morphology also show that building roof greening improves air quality near roads. The degree of air quality improvement near roads due to building roof greening depends on the ambient wind direction. These findings provide a theoretical foundation for constructing green roofs for the purpose of improving air quality near roads or at a pedestrian level as well as urban thermal environment. Further studies using a CFD model coupled with a photochemistry model and a surface energy balance model are required to evaluate the effects of building roof greening on air quality in street canyons in a more realistic framework.

  19. Calcareous nannoplankton and benthic foraminiferal assemblages from the Nazare Canyon (Portuguese continental margin): Preliminary results

    International Nuclear Information System (INIS)

    Guerreiro, C; Oliveira, A; Rodrigues, A; Rosa, F; Cachao, M; Fatela, F

    2009-01-01

    Submarine canyons are assumed to play an important role in oceanic/neritic circulation, marine productivity and sedimentary processes, acting as preferential conduits between the littoral and deep oceanic domain. Here we present first results of a comparative micropalaeontological study on calcareous nannoplankton and benthic foraminifera from surface sediments from the surroundings of the upper Nazare Canyon (Portuguese continental margin) and from the shelf north of the canyon. Regardless of the difficulty to distinguish taphonomical from (palaeo)ecological effects in such a complex and still poorly known marine system, the first results suggest that the canyon's hydro-sedimentary dynamic regime act as a prolongation of the shore/inner shelf hydrodynamic conditions towards west, preventing deposition and/or preservation of the smaller and fragile species of calcareous nannoplankton (e.g. E. huxleyi and G. ericsonii) and enhancing the record of the larger and more opportunistic ones (e.g. G. oceanica); and disturbing benthic foraminiferal productivity and/or diversity, or their preservation in the fossil record. Both calcareous nannoplankton and benthic foraminifera are more abundant off the canyon's domain, suggesting that its highly energetic thalweg conditions are probably filtering the fossil record in the sediment. Still, preliminary results suggest that the occurrence of persistent physical phenomena related with the canyon's morphology and proximity to the coast (e.g. solitary internal waves) may be locally promoting favourable conditions for calcareous nannoplankton, as shown by high values of nannoliths, chlorophyll a and 19' hexanoyloxyfucoxantine (unpublished data) north of the canyon's head. It is our goal to test this hypothesis in the near future by (a) studying multicore and surficial sediments from more recent surveys, and (b) calibrating the sediment results with water column data presently in process at the Institute of Oceanography (IO).

  20. Calcareous nannoplankton and benthic foraminiferal assemblages from the Nazare Canyon (Portuguese continental margin): Preliminary results

    Energy Technology Data Exchange (ETDEWEB)

    Guerreiro, C; Oliveira, A; Rodrigues, A [Division of Marine Geology, Portuguese Hydrographic Institute (IH), Rua das Trinas 49, 1249-093 Lisboa (Portugal); Rosa, F [CIACOMAR, Algarve University, Av. 16 de Julho s/n 8700-311 Olhao (Portugal); Cachao, M; Fatela, F [Geology Center and Geology Department, FCUL, Bloco C6, 3o Piso, sala 6.3.57 Campo Grande 1749-016 Lisboa (Portugal)], E-mail: catarina.guerreiro@hidrografico.pt

    2009-01-01

    Submarine canyons are assumed to play an important role in oceanic/neritic circulation, marine productivity and sedimentary processes, acting as preferential conduits between the littoral and deep oceanic domain. Here we present first results of a comparative micropalaeontological study on calcareous nannoplankton and benthic foraminifera from surface sediments from the surroundings of the upper Nazare Canyon (Portuguese continental margin) and from the shelf north of the canyon. Regardless of the difficulty to distinguish taphonomical from (palaeo)ecological effects in such a complex and still poorly known marine system, the first results suggest that the canyon's hydro-sedimentary dynamic regime act as a prolongation of the shore/inner shelf hydrodynamic conditions towards west, preventing deposition and/or preservation of the smaller and fragile species of calcareous nannoplankton (e.g. E. huxleyi and G. ericsonii) and enhancing the record of the larger and more opportunistic ones (e.g. G. oceanica); and disturbing benthic foraminiferal productivity and/or diversity, or their preservation in the fossil record. Both calcareous nannoplankton and benthic foraminifera are more abundant off the canyon's domain, suggesting that its highly energetic thalweg conditions are probably filtering the fossil record in the sediment. Still, preliminary results suggest that the occurrence of persistent physical phenomena related with the canyon's morphology and proximity to the coast (e.g. solitary internal waves) may be locally promoting favourable conditions for calcareous nannoplankton, as shown by high values of nannoliths, chlorophyll a and 19' hexanoyloxyfucoxantine (unpublished data) north of the canyon's head. It is our goal to test this hypothesis in the near future by (a) studying multicore and surficial sediments from more recent surveys, and (b) calibrating the sediment results with water column data presently in process at the Institute of