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Sample records for huntington beach firm

  1. Huntington beach shoreline contamination investigation, phase III: coastal circulation and transport patterns : the likelihood of OCSD's plume impacting Huntington beach shoreline

    Science.gov (United States)

    Noble, Marlene; Xu, Jingping; Rosenfeld, Leslie; Largier, John; Hamilton, Peter; Jones, Burt; Robertson, George

    2003-01-01

    A consortium of agencies have conducted an extensive investigation of the coastal ocean circulation and transport pathways off Huntington Beach, with the aim of identifying any causal links that may exist between the offshore discharge of wastewater by OCSD and the significant bacterial contamination observed along the Huntington Beach shoreline. This is the third study supported by OCSD to determine possible land-based and coa Although the study identifies several possible coastal ocean pathways by which diluted wastewater may be transported to the beach, including internal tide, sea-breeze and subtidal flow features, there were no direct observations of either the high bacteria concentrations seen in the OCSD plume at the shelf break reaching the shoreline in significant levels or of an association between the existence of a coastal ocean process and beach contamination at or above AB411 levels. It is concluded that the OCSD plume is not a major cause of beach contamination; no causal links could be demonstrated. This conclusion is based on the absence of direct observation of plume-beach links, on analysis of the spatial and temporal patterns of shoreline contamination and coastal ocean processes, and on the observation of higher levels of contamination at the beach than in the plume.

  2. 75 FR 11939 - Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation

    Science.gov (United States)

    2010-03-12

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF LABOR Employment and Training Administration 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...

  3. A temporal and ecological analysis of the Huntington Beach Wetlands through an unmanned aerial system remote sensing perspective

    Science.gov (United States)

    Rafiq, Talha

    Wetland monitoring and preservation efforts have the potential to be enhanced with advanced remote sensing acquisition and digital image analysis approaches. Progress in the development and utilization of Unmanned Aerial Systems (UAS) and Unmanned Aerial Vehicles (UAV) as remote sensing platforms has offered significant spatial and temporal advantages over traditional aerial and orbital remote sensing platforms. Photogrammetric approaches to generate high spatial resolution orthophotos of UAV acquired imagery along with the UAV's low-cost and temporally flexible characteristics are explored. A comparative analysis of different spectral based land cover maps derived from imagery captured using UAV, satellite, and airplane platforms provide an assessment of the Huntington Beach Wetlands. This research presents a UAS remote sensing methodology encompassing data collection, image processing, and analysis in constructing spectral based land cover maps to augment the efforts of the Huntington Beach Wetlands Conservancy by assessing ecological and temporal changes at the Huntington Beach Wetlands.

  4. Effect of metallic additives on in situ combustion of Huntington Beach crude experiments

    Energy Technology Data Exchange (ETDEWEB)

    Baena, C.J.; Castanier, L.M.; Brigham, W.E.

    1990-08-01

    The economics and applicability of an in-situ combustion process for the recovery of crude oil are dictated to a large extent by the nature and the amount of fuel formed during the process. The aim of this work is to use combustion tube studies to determine on a quantitative basis, how the nature and the amount of fuel formed could be changed by the presence of metallic additives. These experiments follow from the qualitative observations on the effect of metallic additives on the in-situ combustion of Huntington Beach crude oil made by De los Rios (1987) at SUPRI. He performed kinetic studies on the oxidation of Huntington Beach crude in porous media and showed that the nature of the fuel formed changed when metallic additives were present. Combustion tube runs were performed using the metallic additives: ferrous chloride (FeCl{sub 2{center dot}}4H{sub 2}O), zinc chloride (ZnCl{sub 2}) and stannic chloride (SnCl{sub 4{center dot}}5H{sub 2}O). Unconsolidated cores were prepared by mixing predetermined amounts of an aqueous solution of the metal salt, Huntington Beach crude oil, Ottawa sand and clay in order to achieve the desired fluid saturations. The mixture was then tamped into the combustion tube. Dry air combustion tube runs were performed keeping the conditions of saturation, air flux and injection pressure approximately the same during each run. The nature of the fuel formed and its impact on the combustion parameters were determined and compared with a control run -- an experiment performed with no metallic additive. 30 refs., 33 figs., 6 tabs.

  5. High resolution renderings and interactive visualization of the 2006 Huntington Beach experiment

    Science.gov (United States)

    Im, T.; Nayak, A.; Keen, C.; Samilo, D.; Matthews, J.

    2006-12-01

    The Visualization Center at the Scripps Institution of Oceanography investigates innovative ways to represent graphically interactive 3D virtual landscapes and to produce high resolution, high quality renderings of Earth sciences data and the sensors and instruments used to collect the data . Among the Visualization Center's most recent work is the visualization of the Huntington Beach experiment, a study launched in July 2006 by the Southern California Ocean Observing System (http://www.sccoos.org/) to record and synthesize data of the Huntington Beach coastal region. Researchers and students at the Visualization Center created visual presentations that combine bathymetric data provided by SCCOOS with USGS aerial photography and with 3D polygonal models of sensors created in Maya into an interactive 3D scene using the Fledermaus suite of visualization tools (http://www.ivs3d.com). In addition, the Visualization Center has produced high definition (HD) animations of SCCOOS sensor instruments (e.g. REMUS, drifters, spray glider, nearshore mooring, OCSD/USGS mooring and CDIP mooring) using the Maya modeling and animation software and rendered over multiple nodes of the OptIPuter Visualization Cluster at Scripps. These visualizations are aimed at providing researchers with a broader context of sensor locations relative to geologic characteristics, to promote their use as an educational resource for informal education settings and increasing public awareness, and also as an aid for researchers' proposals and presentations. These visualizations are available for download on the Visualization Center website at http://siovizcenter.ucsd.edu/sccoos/hb2006.php.

  6. Temporal and spatial variability of fecal indicator bacteria in the surf zone off Huntington Beach, CA

    Science.gov (United States)

    Rosenfeld, L.K.; McGee, C.D.; Robertson, G.L.; Noble, M.A.; Jones, B.H.

    2006-01-01

    Fecal indicator bacteria concentrations measured in the surf zone off Huntington Beach, CA from July 1998-December 2001 were analyzed with respect to their spatial patterns along 23 km of beach, and temporal variability on time scales from hourly to fortnightly. The majority of samples had bacterial concentrations less than, or equal to, the minimum detection limit, but a small percentage exceeded the California recreational water standards. Areas where coliform bacteria exceeded standards were more prevalent north of the Santa Ana River, whereas enterococci exceedances covered a broad area both north and south of the river. Higher concentrations of bacteria were associated with spring tides. No temporal correspondence was found between these bacterial events and either the timing of cold water pulses near shore due to internal tides, or the presence of southerly swell in the surface wave field. All three fecal indicator bacteria exhibited a diel cycle, but enterococci rebounded to high nighttime values almost as soon as the sun went down, whereas coliform levels were highest near the nighttime low tide, which was also the lower low tide. ?? 2006 Elsevier Ltd. All rights reserved.

  7. Internal waves and surf zone water quality at Huntington Beach, California

    Science.gov (United States)

    Wong, H.; Santoro, A.; Nidzieko, N. J.; Hench, J. L.; Boehm, A. B.

    2011-12-01

    This study characterized diurnal, semi-diurnal, and high-frequency internal wave field at Huntington Beach, California, USA and the connection between internal waves and surf zone water quality. An array of oceanographic moorings was deployed in the summer of 2005 and 2006 at 10-20 meter depths offshore of the beach to observe internal waves and cross-shore exchange. Concurrently, surf zone water quality was assessed twice daily at an adjacent station (Huntington State Beach) with measurements of phosphate, dissolved inorganic nitrogen, silicate, chlorophyll a, fecal indicator bacteria, and the human-specific fecal DNA marker in Bacteroidales. Spectral analysis of water temperature shows well-defined spectral peaks at diurnal and semi-diurnal frequencies. Complex Empirical Orthogonal Function analysis of observed currents reveals that the baroclinic component (summation of second to fifth principal components) accounted for 30% of the total variance in the currents in both years, indicating the importance of density-driven flow during the summer when the water column was stratified. The major axis of the first principal component was oriented alongshore, whereas that of the second and third principal components made an angle of 25 to 55 degree with the cross-shore direction. Arrival of cold subthermocline water in the very near shore (within 1 km of the surf zone) was characterized by strong onshore flow near the bottom of the water column. The near bottom, baroclinic, cross-shore current was significantly lag-correlated with the near bottom temperature data along a cross-shore transect towards shore, indicative of shoreward transport of cold subthermocline water. Wavelet analysis of temperature data showed that non-stationary temperature fluctuations were correlated with buoyancy frequency and the near bottom cross-shore baroclinic current. During periods of large temperature fluctuations, the majority of the variance was within the semi-diurnal band; however, the

  8. Antibody engineering and therapeutics conference. The annual meeting of the antibody society, Huntington Beach, CA, December 7-11, 2014.

    Science.gov (United States)

    Larrick, James W; Parren, Paul W H I; Huston, James S; Plückthun, Andreas; Bradbury, Andrew; Tomlinson, Ian M; Chester, Kerry A; Burton, Dennis R; Adams, Gregory P; Weiner, Louis M; Scott, Jamie K; Alfenito, Mark R; Veldman, Trudi; Reichert, Janice M

    2014-01-01

    The 25th anniversary of the Antibody Engineering & Therapeutics Conference, the Annual Meeting of The Antibody Society, will be held in Huntington Beach, CA, December 7-11, 2014. Organized by IBC Life Sciences, the event will celebrate past successes, educate participants on current activities and offer a vision of future progress in the field. Keynote addresses will be given by academic and industry experts Douglas Lauffenburger (Massachusetts Institute of Technology), Ira Pastan (National Cancer Institute), James Wells (University of California, San Francisco), Ian Tomlinson (GlaxoSmithKline) and Anthony Rees (Rees Consulting AB and Emeritus Professor, University of Bath). These speakers will provide updates of their work, placed in the context of the substantial growth of the industry over the past 25 years.

  9. Distribution and sources of surfzone bacteria at Huntington Beach before and after disinfection on an ocean outfall - A frequency-domain analysis

    Science.gov (United States)

    Noble, M.A.; Xu, J. P.; Robertson, G.L.; Rosenfeld, L.K.

    2006-01-01

    Fecal indicator bacteria (FIB) were measured approximately 5 days a week in ankle-depth water at 19 surfzone stations along Huntington Beach and Newport Beach, California, from 1998 to the end of 2003. These sampling periods span the time before and after treated sewage effluent, discharged into the coastal ocean from the local outfall, was disinfected. Bacterial samples were also taken in the vicinity of the outfall during the pre- and post-disinfection periods. Our analysis of the results from both data sets suggest that land-based sources, rather than the local outfall, were the source of the FIB responsible for the frequent closures and postings of local beaches in the summers of 2001 and 2002. Because the annual cycle is the dominant frequency in the fecal and total coliform data sets at most sampling stations, we infer that sources associated with local runoff were responsible for the majority of coliform contamination along wide stretches of the beach. The dominant fortnightly cycle in enterococci at many surfzone sampling stations suggests that the source for these relatively frequent bacteria contamination events in summer is related to the wetting and draining of the land due to the large tidal excursions found during spring tides. Along the most frequently closed section of the beach at stations 3N-15N, the fortnightly cycle is dominant in all FIBs. The strikingly different spatial and spectral patterns found in coliform and in enterococci suggest the presence of different sources, at least for large sections of beach. The presence of a relatively large enterococci fortnightly cycle along the beaches near Newport Harbor indicates that contamination sources similar to those found off Huntington Beach are present, though not at high enough levels to close the Newport beaches. ?? 2006 Elsevier Ltd. All rights reserved.

  10. "EPA'S NATIONAL BEACHES STUDY: HUNTINGTON BEACH, 2003"

    Science.gov (United States)

    The original U. S. Environmental Protection Agency (EPA) recreational water health studies, initiated in 1972 and completed in 1982, were designed to determine the relationship between swimming-associated gastroenteritis and the quality of the bathing water. However, these healt...

  11. Huntington's Disease

    Science.gov (United States)

    Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste ... express emotions. If one of your parents has Huntington's disease, you have a 50 percent chance of ...

  12. 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...

  13. Huntington disease

    Science.gov (United States)

    ... President of the Florida Society of Neurology (FSN). Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team. Huntington's Disease Read more Latest Health News Read more Health ...

  14. Learning about Huntington's Disease

    Science.gov (United States)

    ... Mouse Models Of Huntington's Disease 1998 News Release Learning About Huntington's Disease What do we know about ... and treatment information. Hosted by the Dolan DNA Learning Center at Cold Spring Harbor Laboratory. Huntington's Outreach ...

  15. 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

  16. 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

  17. Huntington's disease phenocopy syndromes.

    Science.gov (United States)

    Wild, Edward J; Tabrizi, Sarah J

    2007-12-01

    Patients presenting with features of Huntington's disease but lacking the causative genetic expansion can be challenging diagnostically. The differential diagnosis of such Huntington's disease phenocopy syndromes has not recently been reviewed. Cohort studies have established the relative frequencies of known Huntington's disease phenocopy syndromes, whereas newly described ones have been characterized genetically, clinically, radiologically and pathologically. About 1% of suspected Huntington's disease cases emerge as phenocopy syndromes. Such syndromes are clinically important in their own right but may also shed light on the pathogenesis of Huntington's disease. Huntington's disease produces a range of clinical phenotypes, and the range of syndromes that may be responsible for Huntington's disease phenocopies is correspondingly wide. Cohort studies have established that, while the majority of phenocopy patients remain undiagnosed, in those patients where a genetic diagnosis is reached the commonest causes are SCA17, Huntington's disease-like syndrome 2 (HDL2), familial prion disease and Friedreich's ataxia. We review the features of the reported genetic causes of Huntington's disease phenocopy syndromes, including HDL1-3, SCA17, familial prion disease, spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, chorea-acanthocytosis and iron-accumulation disorders. We present an evidence-based framework for the genetic testing of Huntington's disease phenocopy cases.

  18. [The Henry E. Huntington Library.

    Science.gov (United States)

    Abraham, Terry

    The biographical sketch of Henry E. Huntington includes a description of the establishment of the Huntington Library and the purpose and scope of its collection. Although this is a free and public library, its use is restricted to qualified scholars having legitimate research needs. Photographic techniques were developed at the Huntington Library…

  19. Biblioteca Publica en Huntington Beach (EE.UU.

    Directory of Open Access Journals (Sweden)

    Historico, Dion

    1977-07-01

    Full Text Available The first stage, which is the only one completed up to the present time, consists of a more extensive architectural complex conceived for housing different cultural functions besides the library: plastic arts, cinematography, scientific and informative activities, etc. The building is organized in two main floors in a rectangular shape, and in several mezzanines, reading rooms, book deposits, projection areas, music and painting departments and technical administration and maintenance offices, occupying a total of approximately 4,400 m2. The architectural design denotes the intention of integration in the treatment of the inside spaces, by the suppression of separate compartments, organizing the necessary distinction of functions in the reading rooms, by means of differences of floor levels and with the use of adequate decorative elements. In the same manner, the outside walls have been substituted by complete glass surfaces which allow full incorporation of the natural surroundings in the architecture.Constituye la primera fase, única realizada hasta el presente, de un complejo arquitectónico más amplio concebido para albergar distintas funciones culturales además de biblioteca: artes plásticas, cinematografía, actividades científicas e informativas, etc. El edificio se organiza en dos plantas principales, de forma rectangular, y en diversas entreplantas, salas de lectura, depósitos de libros, locales de proyección, departamentos de música y pintura, y oficinas técnicas de administración y mantenimiento, ocupando un total aproximado de 4.400 m2. El planteamiento arquitectónico denota un propósito integrador en el tratamiento de los espacios interiores, por la supresión de las compartimentaciones de fábrica, organizándose la necesaria distinción de ambientes en las salas de lectura, mediante diferencias de nivel del suelo y con el empleo de adecuados elementos decorativos. Del mismo modo, los muros de fachada se han sustituido por paramentos íntegramente acristalados, que permiten una plena incorporación del entorno natural en la arquitectura.

  20. Exploring rippled scour depressions offshore Huntington Beach, CA

    Science.gov (United States)

    Phillips, Eleyne L.; Storlazzi, Curt D.; Dartnell, Peter; Edwards, Brian D.

    2007-01-01

    U.S. Geological Survey (USGS) scientists used 1999 multibeam data, and 2002 lidar data collected on the inner shelf off southern California to investigate a field of (<1 m) features, termed "Rippled Scour Depressions" (RSDs). RSDs are elongate, shore-normal, and bathymetrically depressed features; their morphology was determined from multibeam and lidar bathymetry. Wavelengths of ripples seen within RSDs and on the surrounding seafloor were calculated from photography and video collected in 2004 and related to sediment samples collected in the same year. The RSDs were divided into two areas: Region I RSDs contained large (∼80 cm wavelength), straight-crested ripples with coarse-grained lag, and decreased in area between 1999 and 2002; Region II RSDs were smaller, in shallower water, closer to shore, and contained shorter (∼30 cm wavelength) ripples, and increased in area from 1999–2002. The RSDs did not display marked alongshore asymmetry.

  1. 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.

  2. Beach Profile Locations

    Data.gov (United States)

    California Department of Resources — Beaches are commonly characterized by cross-shore surveys. The resulting profiles represent the elevation of the beach surface and nearshore seabed from the back of...

  3. Beach Profile Locations

    Data.gov (United States)

    California Department of Resources — Beaches are commonly characterized by cross-shore surveys. The resulting profiles represent the elevation of the beach surface and nearshore seabed from the back of...

  4. 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.

  5. What is HD - Huntington's Disease?

    Science.gov (United States)

    ... the person less able to work at their customary level and less functional in their regular activities ... not is intensely personal and there is no "right" answer. The Huntington's Disease Society of America recommends ...

  6. Stages of Huntington's Disease (HD)

    Science.gov (United States)

    ... the person less able to work at their customary level and less functional in their regular activities ... not is intensely personal and there is no "right" answer. The Huntington's Disease Society of America recommends ...

  7. Natural history of Huntington disease.

    Science.gov (United States)

    Dorsey, E Ray; Beck, Christopher A; Darwin, Kristin; Nichols, Paige; Brocht, Alicia F D; Biglan, Kevin M; Shoulson, Ira

    2013-12-01

    Understanding the natural history of Huntington disease will inform patients and clinicians on the disease course and researchers on the design of clinical trials. To determine the longitudinal change in clinical features among individuals with Huntington disease compared with controls. Prospective, longitudinal cohort study at 44 research sites in Australia (n = 2), Canada (n =4), and the United States (n = 38). Three hundred thirty-four individuals with clinically manifest Huntington disease who had at least 3 years of annually accrued longitudinal data and 142 controls consisting of caregivers and spouses who had no genetic risk of Huntington disease. Change in movement, cognition, behavior, and function as measured by the Unified Huntington's Disease Rating Scale, the Mini-Mental State Examination, and vital signs. Total motor score worsened by 3.0 points (95% CI, 2.5-3.4) per year and chorea worsened by 0.3 point per year (95% CI, 0.1-0.5). Cognition declined by 0.7 point (95% CI, 0.6-0.8) per year on the Mini-Mental State Examination. Behavior, as measured by the product of frequency and severity score on the Unified Huntington's Disease Rating Scale, worsened by 0.6 point per year (95% CI, 0.0-1.2). Total functional capacity declined by 0.6 point per year (95% CI, 0.5-0.7). Compared with controls, baseline body mass index was lower in those with Huntington disease (25.8 vs 28.8; P Huntington disease all declined in a monotonic manner. These data quantify the natural history of the disease and may inform the design of trials aimed at reducing its burden. clinicaltrials.gov Identifier: NCT00313495.

  8. Huntington's disease in children.

    Science.gov (United States)

    Letort, Derek; Gonzalez-Alegre, Pedro

    2013-01-01

    Huntington's disease (HD) is a dominantly inherited, fatal neurodegenerative disease. This incurable illness is characterized by a triad of a movement disorder, cognitive decline and psychiatric manifestations. Although most patients with HD have disease onset in the adult years, a small but significant proportion present with pediatric HD. It has been long known that patients with early-onset HD commonly exhibit prominent parkinsonism, known as the Westphal variant of HD. However, even among patients with pediatric HD there are differential clinical features depending on the age of onset, with younger patients frequently presenting diagnostic challenges. In his chapter, the characteristics of patients with childhood- and adolescence-onset HD are discussed, focusing on the differential clinical features that can aid the clinical reach a correct diagnosis, the indications and rational use of genetic testing and the currently available options for symptomatic treatment.

  9. ICT, Complementary Firm Strategies and Firm Performance

    OpenAIRE

    Rasel, Fabienne

    2016-01-01

    This doctoral thesis analyses from different perspectives the relationship between information and communication technologies (ICT), complementary firm strategies and firm performance. One chapter examines the role of ICT and decentralized workplace organization for productivity. Two chapters analyse the association between firms' ICT use, international trade activities and productivity. The empirical analyses are based on data from firms located in Germany from the manufacturing or service s...

  10. Strong Firms Lobby, Weak Firms Bribe

    DEFF Research Database (Denmark)

    Bennedsen, Morten; Feldmann, Sven E.; Lassen, David Dreyer

    We use survey responses by firms to examine the firm-level determinants and effects of political influence, their perception of corruption and prevalence of bribe paying. We find that: (a) measures of political influence and corruption/bribes are uncorrelated at the firm level; (b) firms...... that are larger, older, exporting, government-owned, are widely held and/or have fewer competitors, have more political influence, perceive corruption to be less of a problem and pay bribes less often; (c) influence increases sales and government subsidies and in general makes the firm have a more positive view...

  11. Treatment of Huntington's disease.

    Science.gov (United States)

    Frank, Samuel

    2014-01-01

    Huntington's disease (HD) is a dominantly inherited progressive neurological disease characterized by chorea, an involuntary brief movement that tends to flow between body regions. HD is typically diagnosed based on clinical findings in the setting of a family history and may be confirmed with genetic testing. Predictive testing is available to family members at risk, but only experienced clinicians should perform the counseling and testing. Multiple areas of the brain degenerate, mainly involving the neurotransmitters dopamine, glutamate, and γ-aminobutyric acid. Although pharmacotherapies theoretically target these neurotransmitters, few well-conducted trials for symptomatic interventions have yielded positive results and current treatments have focused on the motor aspects of HD. Tetrabenazine is a dopamine-depleting agent that may be one of the more effective agents for reducing chorea, although it has a risk of potentially serious adverse effects. Some newer neuroleptic agents, such as olanzapine and aripiprazole, may have adequate efficacy with a more favorable adverse effect profile than older neuroleptic agents for treating chorea and psychosis. There are no current treatments to change the course of HD, but education and symptomatic therapies can be effective tools for clinicians to use with patients and families affected by HD.

  12. Dopamine and Huntington's disease.

    Science.gov (United States)

    Schwab, Laetitia C; Garas, Shady N; Garas, Shaady N; Drouin-Ouellet, Janelle; Mason, Sarah L; Stott, Simon R; Barker, Roger A

    2015-04-01

    Huntington's disease (HD) is an incurable, inherited, progressive neurodegenerative disorder that is defined by a combination of motor, cognitive and psychiatric features. Pre-clinical and clinical studies have demonstrated an important role for the dopamine (DA) system in HD with dopaminergic dysfunction at the level of both DA release and DA receptors. It is, therefore, not surprising that the drug treatments most commonly used in HD are anti-dopaminergic agents. Their use is based primarily on the belief that the characteristic motor impairments are a result of overactivation of the central dopaminergic pathways. While this is a useful starting place, it is clear that the behavior of the central dopaminergic pathways is not fully understood in this condition and may change as a function of disease stage. In addition, how abnormalities in dopaminergic systems may underlie some of the non-motor features of HD has also been poorly investigated and this is especially important given the greater burden these place on the patients' and families' quality of life. In this review, we discuss what is known about central dopaminergic pathways in HD and how this informs us about the mechanisms of action of the dopaminergic therapies used to treat it. By doing so, we will highlight some of the paradoxes that exist and how solving them may reveal new insights for improved treatment of this currently incurable condition, including the possibility that such drugs may even have effects on disease progression and pathogenesis.

  13. Huntington's Disease and Mitochondria.

    Science.gov (United States)

    Jodeiri Farshbaf, Mohammad; Ghaedi, Kamran

    2017-06-21

    Huntington's disease (HD) as an inherited neurodegenerative disorder leads to neuronal loss in striatum. Progressive motor dysfunction, cognitive decline, and psychiatric disturbance are the main clinical symptoms of the HD. This disease is caused by expansion of the CAG repeats in exon 1 of the huntingtin which encodes Huntingtin protein (Htt). Various cellular and molecular events play role in the pathology of HD. Mitochondria as important organelles play crucial roles in the most of neurodegenerative disorders like HD. Critical roles of the mitochondria in neurons are ATP generation, Ca(2+) buffering, ROS generation, and antioxidant activity. Neurons as high-demand energy cells closely related to function, maintenance, and dynamic of mitochondria. In the most neurological disorders, mitochondrial activities and dynamic are disrupted which associate with high ROS level, low ATP generation, and apoptosis. Accumulation of mutant huntingtin (mHtt) during this disease may evoke mitochondrial dysfunction. Here, we review recent findings to support this hypothesis that mHtt could cause mitochondrial defects. In addition, by focusing normal huntingtin functions in neurons, we purpose mitochondria and Huntingtin association in normal condition. Moreover, mHtt affects various cellular signaling which ends up to mitochondrial biogenesis. So, it could be a potential candidate to decline ATP level in HD. We conclude how mitochondrial biogenesis plays a central role in the neuronal survival and activity and how mHtt affects mitochondrial trafficking, maintenance, integrity, function, dynamics, and hemostasis and makes neurons vulnerable to degeneration in HD.

  14. Neuroimaging in Huntington's disease.

    Science.gov (United States)

    Niccolini, Flavia; Politis, Marios

    2014-06-28

    Huntington's disease (HD) is a progressive and fatal neurodegenerative disorder caused by an expanded trinucleotide CAG sequence in huntingtin gene (HTT) on chromosome 4. HD manifests with chorea, cognitive and psychiatric symptoms. Although advances in genetics allow identification of individuals carrying the HD gene, much is still unknown about the mechanisms underlying the development of overt clinical symptoms and the transitional period between premanifestation and manifestation of the disease. HD has no cure and patients rely only in symptomatic treatment. There is an urgent need to identify biomarkers that are able to monitor disease progression and assess the development and efficacy of novel disease modifying drugs. Over the past years, neuroimaging techniques such as magnetic resonance imaging (MRI) and positron emission tomography (PET) have provided important advances in our understanding of HD. MRI provides information about structural and functional organization of the brain, while PET can detect molecular changes in the brain. MRI and PET are able to detect changes in the brains of HD gene carriers years ahead of the manifestation of the disease and have also proved to be powerful in assessing disease progression. However, no single technique has been validated as an optimal biomarker. An integrative multimodal imaging approach, which combines different MRI and PET techniques, could be recommended for monitoring potential neuroprotective and preventive therapies in HD. In this article we review the current neuroimaging literature in HD.

  15. Offshoring and Firm Overlap

    OpenAIRE

    Schmerer, Hans-Jörg; Capuano, Stella; Egger, Hartmut; Koch, Michael

    2015-01-01

    We set up a model of offshoring with heterogeneous producers that captures two empirical regularities on offshoring firms: larger, more productive firms are more likely to make use of the offshoring opportunity; the fraction of firms that engages in offshoring is positive and smaller than one in any size or revenue category. These patterns generate an overlap of offshoring and non-offshoring firms, which is non-monotonic in the costs of offshoring. In an empirical exercise, we employ firm-lev...

  16. Erosion Negril Beach

    NARCIS (Netherlands)

    Ten Ham, D.; Henrotte, J.; Kraaijeveld, R.; Milosevic, M.; Smit, P.

    2006-01-01

    The ongoing erosion of the Negril Beach has become worse the past decade. In most places along the coast line, the beach will be gone in approximately 10 years. This will result in a major decrease of incomes that are made by the local tourist sector. To prevent the erosion this study has been perfo

  17. Huntington Disease in Asia

    Institute of Scientific and Technical Information of China (English)

    Miao Xu; Zhi-Ying Wu

    2015-01-01

    Objective:The objective was to review the major differences of Huntington disease (HD) in Asian population from those in the Caucasian population.Data Sources:Data cited in this review were obtained from PubMed database and China National Knowledge Infrastructure (CNKI) from 1994 to 2014.All the papers were written in English or Chinese languages,with the terms of Asia/Asian,HD,genotype,epidemiology,phenotype,and treatment used for the literature search.Study Selection:From the PubMed database,we included the articles and reviews which contained the HD patients' data from Asian countries.From the CNKI,we excluded the papers which were not original research.Due to the language's restrictions,those data published in other languages were not included.Results:In total,50 papers were cited in this review,authors of which were from the mainland of China,Japan,India,Thailand,Taiwan (China),Korea,and western countries.Conclusions:The lower epidemiology in Asians can be partly explained by the less cytosine-adenine-guanine repeats,different haplotypes,and CCG polymorphisms.For the physicians,atypical clinical profiles such as the initial symptom of ataxia,movement abnormalities of Parkinsonism,dystonia,or tics need to be paid more attention to and suggest gene testing if necessary.Moreover,some pathogenesis studies may help progress some new advanced treatments.The clinicians in Asian especially in China should promote the usage of genetic testing and put more effects in rehabilitation,palliative care,and offer comfort of patients and their families.The unified HD rating scale also needs to be popularized in Asia to assist in evaluating the progression of HD.

  18. Louisiana's statewide beach cleanup

    Science.gov (United States)

    Lindstedt, Dianne M.; Holmes, Joseph C.

    1989-01-01

    Litter along Lousiana's beaches has become a well-recognized problem. In September 1987, Louisiana's first statewide beach cleanup attracted about 3300 volunteers who filled 16,000 bags with trash collected along 15 beaches. An estimated 800,173 items were gathered. Forty percent of the items were made of plastic and 11% were of polystyrene. Of all the litter collected, 37% was beverage-related. Litter from the oil and gas, commercial fishing, and maritime shipping industries was found, as well as that left by recreational users. Although beach cleanups temporarily rid Louisiana beaches of litter, the real value of the effort is in public participation and education. Civic groups, school children, and individuals have benefited by increasing their awareness of the problems of trash disposal.

  19. Mitochondrial dysfunction and Huntington disease

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Huntington disease (HD) is a chronic autosomal-dominant neurodegenerative disease. The gene coding Huntingtin has been identified, but the pathogenic mechanisms of the disease are still not fully understood. This paper reviews the involvement of mitochondrial dysfunction in pathogenesis of HD.

  20. Is Huntington's disease a tauopathy?

    Science.gov (United States)

    Gratuze, Maud; Cisbani, Giulia; Cicchetti, Francesca; Planel, Emmanuel

    2016-04-01

    Tauopathies are a subclass of neurodegenerative diseases typified by the deposition of abnormal microtubule-associated tau protein within the cerebral tissue. Alzheimer's disease, progressive supranuclear palsy, chronic traumatic encephalopathy and some fronto-temporal dementias are examples of the extended family of tauopathies. In the last decades, intermittent reports of cerebral tau pathology in individuals afflicted with Huntington's disease-an autosomal dominant neurodegenerative disorder that manifests by severe motor, cognitive and psychiatric problems in adulthood-have also begun to surface. These observations remained anecdotal until recently when a series of publications brought forward compelling evidence that this monogenic disorder may, too, be a tauopathy. Collectively, these studies reported that: (i) patients with Huntington's disease present aggregated tau inclusions within various structures of the brain; (ii) tau haplotype influences the cognitive function of Huntington's disease patients; and (iii) that the genetic product of the disease, the mutant huntingtin protein, could alter tau splicing, phosphorylation, oligomerization and subcellular localization. Here, we review the past and current evidence in favour of the postulate that Huntington's disease is a new member of the family of tauopathies. © The Author (2016). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  1. Neurodegenerative disorders: Parkinson's disease and Huntington's disease

    Science.gov (United States)

    Hague, S; Klaffke, S; Bandmann, O

    2005-01-01

    Parkinson's disease and Huntington's disease are both model diseases. Parkinson's disease is the most common of several akinetic-rigid syndromes and Huntington's disease is only one of an ever growing number of trinucleotide repeat disorders. Molecular genetic studies and subsequent molecular biological studies have provided fascinating new insights into the pathogenesis of both disorders and there is now real hope for disease modifying treatment in the not too distant future for patients with Parkinson's disease or Huntington's disease. PMID:16024878

  2. Neurodegenerative disorders: Parkinson's disease and Huntington's disease.

    Science.gov (United States)

    Hague, S M; Klaffke, S; Bandmann, O

    2005-08-01

    Parkinson's disease and Huntington's disease are both model diseases. Parkinson's disease is the most common of several akinetic-rigid syndromes and Huntington's disease is only one of an ever growing number of trinucleotide repeat disorders. Molecular genetic studies and subsequent molecular biological studies have provided fascinating new insights into the pathogenesis of both disorders and there is now real hope for disease modifying treatment in the not too distant future for patients with Parkinson's disease or Huntington's disease.

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

    Science.gov (United States)

    Phukan, Julie; Ali, Elfatih; Pender, Niall P; Molloy, Fiona; Hennessy, Michael; Walsh, Ronan J; Hardiman, Orla

    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.

  4. National List of Beaches

    Data.gov (United States)

    U.S. Environmental Protection Agency — EPA has published a list of coastal recreation waters adjacent to beaches (or similar points of access) used by the public in the U.S. The list, required by the...

  5. Beach Ball Coronagraph Project

    Data.gov (United States)

    National Aeronautics and Space Administration — The “Beach Ball” Coronagraph will be the first steps to simplify and revolutionize the next generation solar coronagraph design.  The solar corona...

  6. Transformation of Manufacturing Firms to Servitisation Firms

    DEFF Research Database (Denmark)

    Lin, Chih-Cheng; Ma, Zheng; Tanev, Stoyan

    2014-01-01

    It is crucial for the manufacturing SMEs to reconsider their business strategy in order to be able to launch customer-centric solutions. This ability is associated with a paradigm shift from a product-orientation to service-orientation. One of the major challenges to success in transforming...... a traditional manufacturing firm to service-oriented firms is the conspicuous lack of publications in this research stream. Applying a case study research approach, this study explores the transformation model for manufacturing SMEs to servitisation firms by adopting a network approach, and reveals...

  7. Transformation of Manufacturing Firms to Servitisation Firms

    DEFF Research Database (Denmark)

    Lin, Chih-Cheng; Ma, Zheng; Tanev, Stoyan

    2014-01-01

    a traditional manufacturing firm to service-oriented firms is the conspicuous lack of publications in this research stream. Applying a case study research approach, this study explores the transformation model for manufacturing SMEs to servitisation firms by adopting a network approach, and reveals......It is crucial for the manufacturing SMEs to reconsider their business strategy in order to be able to launch customer-centric solutions. This ability is associated with a paradigm shift from a product-orientation to service-orientation. One of the major challenges to success in transforming...

  8. Huntington Disease: Molecular Diagnostics Approach.

    Science.gov (United States)

    Bastepe, Murat; Xin, Winnie

    2015-10-06

    Huntington disease (HD) is caused by expansion of a CAG trinucleotide repeat in the first exon of the Huntingtin (HTT) gene. Molecular testing of Huntington disease for diagnostic confirmation and disease prediction requires detection of the CAG repeat expansion. There are three main types of HD genetic testing: (1) diagnostic testing to confirm or rule out disease, (2) presymptomatic testing to determine whether an at-risk individual inherited the expanded allele, and (3) prenatal testing to determine whether the fetus has inherited the expanded allele. This unit includes protocols that describe the complementary use of polymerase chain reactions (PCR) and Southern blot hybridization to accurately measure the CAG trinucleotide repeat size and interpret the test results. In addition, an indirect linkage analysis that does not reveal the unwanted parental HD status in a prenatal testing will also be discussed.

  9. Cortical myoclonus in Huntington's disease.

    Science.gov (United States)

    Thompson, P D; Bhatia, K P; Brown, P; Davis, M B; Pires, M; Quinn, N P; Luthert, P; Honovar, M; O'Brien, M D; Marsden, C D

    1994-11-01

    We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.

  10. Molecular Imaging of Huntington's Disease.

    Science.gov (United States)

    Ciarmiello, Andrea; Giovacchini, Giampiero; Giovannini, Elisabetta; Lazzeri, Patrizia; Borsò, Elisa; Mannironi, Antonio; Mansi, Luigi

    2017-08-01

    The onset and the clinical progression of Huntington Disease (HD) is influenced by several events prompted by a genetic mutation that affects several organs tissues including different regions of the brain. In the last decades years, Positron Emission Tomography (PET) and Magnetic Resonance Imaging (MRI) helped to deepen the knowledge of neurodegenerative mechanisms that guide to clinical symptoms. Brain imaging with PET represents a tool to investigate the physiopathology occurring in the brain and it has been used to predict the age of onset of the disease and to evaluate the therapeutic efficacy of new drugs. This article reviews the contribution of PET and MRI in the research field on Huntington's disease, focusing in particular on some most relevant achievements that have helped recognize the molecular changes, the clinical symptoms and evolution of the disease. J. Cell. Physiol. 232: 1988-1993, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  11. Antipsychotic drugs in Huntington's disease.

    Science.gov (United States)

    Unti, E; Mazzucchi, S; Palermo, G; Bonuccelli, U; Ceravolo, R

    2017-03-01

    The aim of this review is to overview the pharmacological features of neuroleptics experienced in the treatment of Huntington's disease (HD) symptoms. Despite a large number of case reports, randomized controlled trials (RCT) and drug comparison studies are lacking. Areas covered: After evaluating current guidelines and clinical unmet needs we searched PubMed for the term 'Huntington's disease' cross referenced with the terms 'Antipsychotic drugs' 'Neuroleptic drugs' and single drug specific names. Expert commentary: In clinical practice antipsychotics represent the first choice in the management of chorea in the presence of psychiatric symptoms, when poor compliance is suspected or when there is an increased risk of adverse events due to tetrabenazine. Antipsychotics are considered valid strategies, with the second generation preferred to reduce extrapyramidal adverse events, however they may cause more metabolic side effects. In the future 'dopamine stabilizers', such as pridopidine, could replace antipsychotics modulating dopamine transmission.

  12. Performance of Patenting Firms

    DEFF Research Database (Denmark)

    Madsen, Erik Strøjer; Smith, Valdemar; Nielsen, Anders Østergaard

    2000-01-01

    -patenting firms within the manufacturing sector in Denmark. Performance is measured both by growth in employment as well as in the return on equity and profit share in turnover. The results suggest that differences in performance of patenting and non-patenting firms are very small, which questions the political......Most countries focus on industries with high technology and the governments grant subsidies to innovating firms. However, there has been remarkable few studies of the performance of innovative firms or industries. This study examines the performance of patent active firms compared to the non...

  13. Neuropsychiatric Burden in Huntington's Disease.

    Science.gov (United States)

    Paoli, Ricardo Augusto; Botturi, Andrea; Ciammola, Andrea; Silani, Vincenzo; Prunas, Cecilia; Lucchiari, Claudio; Zugno, Elisa; Caletti, Elisabetta

    2017-06-16

    Huntington's disease is a disorder that results in motor, cognitive, and psychiatric problems. The symptoms often take different forms and the presence of disturbances of the psychic sphere reduces patients' autonomy and quality of life, also impacting patients' social life. It is estimated that a prevalence between 33% and 76% of the main psychiatric syndromes may arise in different phases of the disease, often in atypical form, even 20 years before the onset of chorea and dementia. We present a narrative review of the literature describing the main psychopathological patterns that may be found in Huntington's disease, searching for a related article in the main database sources (Medline, ISI Web of Knowledge, Scopus, and Medscape). Psychiatric conditions were classified into two main categories: affective and nonaffective disorders/symptoms; and anxiety and neuropsychiatric features such as apathy and irritability. Though the literature is extensive, it is not always convergent, probably due to the high heterogeneity of methods used. We summarize main papers for pathology and sample size, in order to present a synoptic vision of the argument. Since the association between Huntington's disease and psychiatric symptoms was demonstrated, we argue that the prevalent and more invalidating psychiatric components should be recognized as early as possible during the disease course in order to best address psychopharmacological therapy, improve quality of life, and also reduce burden on caregivers.

  14. Huntington's disease: review and anesthetic case management.

    OpenAIRE

    Cangemi, C. F.; Miller, R. J.

    1998-01-01

    Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. Patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depress...

  15. Drug-induced hyperthermia in Huntington's disease

    NARCIS (Netherlands)

    Gaasbeek, D; Naarding, Paul; Stor, T; Kremer, H P H

    Until now, only three patients with Huntington's disease (HD) and a neuroleptic malignant syndrome (NMS) have been reported in the literature. We describe four cases with advanced stage Huntington's disease who within a period of one year developed drug-induced hyperthermia, either the neuroleptic

  16. Drug-induced hyperthermia in Huntington's disease.

    NARCIS (Netherlands)

    Gaasbeek, D.; Naarding, P.; Stor, T.; Kremer, H.P.H.

    2004-01-01

    Until now, only three patients with Huntington's disease (HD) and a neuroleptic malignant syndrome (NMS) have been reported in the literature. We describe four cases with advanced stage Huntington's disease who within a period of one year developed drug-induced hyperthermia, either the neuroleptic

  17. Drug-induced hyperthermia in Huntington's disease.

    NARCIS (Netherlands)

    Gaasbeek, D.; Naarding, P.; Stor, T.; Kremer, H.P.H.

    2004-01-01

    Until now, only three patients with Huntington's disease (HD) and a neuroleptic malignant syndrome (NMS) have been reported in the literature. We describe four cases with advanced stage Huntington's disease who within a period of one year developed drug-induced hyperthermia, either the neuroleptic m

  18. Apathy is not depression in Huntington's disease

    NARCIS (Netherlands)

    Naarding, Paul; Janzing, Joost G E; Eling, Paul; van der Werf, Sieberen; Kremer, Berry

    2009-01-01

    Apathy and depression are common neuropsychiatric features of Huntington's disease. The authors studied a group of 34 Huntington's disease patients. In addition to the conventional classification according to DSM-IV criteria of depression, emphasis was put on a dimensional approach using scores on

  19. Hawaii Beach Monitoring Program: Beach Profile Data

    Science.gov (United States)

    Gibbs, Ann E.; Richmond, Bruce M.; Fletcher, Charles H.; Hillman, Kindra P.

    2001-01-01

    Coastal erosion is widespread and locally severe in Hawaii and other low-latitude areas. Typical erosion rates in Hawaii are in the range of 15 to 30 cm/yr (0.5 to 1 ft/yr; Hwang, 1981; Sea Engineering, Inc., 1988; Makai Ocean Engineering, Inc. and Sea Engineering, Inc.,1991). Recent studies on Oahu (Fletcher et al., 1997; Coyne et al., 1996) have shown that nearly 24%, or 27.5 km (17.1 mi) of an original 115 km (71.6 mi) of sandy shoreline (1940's) has been either significantly narrowed (17.2 km; 10.7 mi) or lost (10.3 km; 6.4 mi). Nearly one-quarter of the islands' beaches have been significantly degraded over the last half-century and all shorelines have been affected to some degree. Oahu shorelines are by far the most studied, however, beach loss has been identified on the other islands as well, with nearly 13 km (8 mi) of beach likely lost due to shoreline hardening on Maui (Makai Engineering, Inc. and Sea Engineering, Inc., 1991). Causes of coastal erosion and beach loss in Hawaii are numerous but, unfortunately, poorly understood and rarely quantified. Construction of shoreline protection structures limits coastal land loss, but does not alleviate beach loss and may actually accelerate the problem by prohibiting sediment deposition in front of the structures. Other factors contributing to beach loss include: a) reduced sediment supply; b) large storms; and, c) sea-level rise. Reduction in sand supply, either from landward or seaward (primarily reef) sources, can have a myriad of causes. Obvious causes such as beach sand mining and emplacement of structures that interrupt natural sediment transport pathways or prevent access to backbeach sand deposits, remove sediment from the active littoral system. More complex issues of sediment supply can be related to reef health and carbonate production which, in turn, may be linked to changes in water quality. Second, the accumulated effect of large storms is to transport sediment beyond the littoral system. Third

  20. Huntington's disease: clinical characteristics, pathogenesis and therapies.

    Science.gov (United States)

    Nakamura, Ken; Aminoff, Michael J

    2007-02-01

    Huntington's disease is a devastating disorder with no known cure. The disease results from an expanded sequence of CAG repeats in the huntingtin gene and leads to a movement disorder with associated cognitive and systemic deficits. Huntington's disease is diagnosed by genetic testing and disease progression can be followed with a variety of imaging modalities. The accumulation of aggregated huntingtin with associated striatal degeneration is evident at autopsy. The pathophysiology of Huntington's disease remains unknown, although protein aggregation, excitotoxicity, deficits in energy metabolism, transcriptional dysregulation and apoptosis may all be involved. Current pharmacologic therapy for Huntington's disease is limited and exclusively symptomatic. However, the disease is being heavily researched, and a wide range of disease-modifying therapies is currently under development. The efficacy of these therapies is being evaluated in transgenic models of Huntington's disease and in preliminary clinical trials.

  1. Sand supply to beaches

    Science.gov (United States)

    Aagaard, Troels

    2017-04-01

    In most cases, beaches and dunes are built by sand that has been transported onshore from the shoreface. While this has been known for a long time, we are still not able to quantitatively predict onshore sediment transport and sand supply to beaches. Sediment transport processes operating during brief, high-energy stormy conditions - when beaches erode and sand moves offshore - are fairly well known and they can be modelled with a reasonable degree of confidence. However, the slower onshore sand transport leading to beach recovery under low-to-moderate energy conditions - and the reason why beaches and dunes exist in the first place - is not yet well understood. This severely limits our capability to understand and predict coastal behaviour on long time scales, for example in response to changing sea level or wave conditions. This paper will discuss issues and recent developments in sediment transport measurement and prediction on the lower and upper shoreface and into the swash zone. The focus will be on the integration and upscaling of small-scale deterministic process measurements into parametric models that may increase modelling capabilities of coastal behaviour on larger temporal and spatial scales.

  2. Beach rock from Goa Coast

    Digital Repository Service at National Institute of Oceanography (India)

    Setty, M.G.A.P.; Wagle, B.G.

    temperature under the beach and inland. However, the thickness depends upon variabilities of precipitation, hinterland, water table fluctuations, temperature changes, composition and changes in sea level. Since the beach rock is formed in the tidal or spray...

  3. Summary of Annual Beach Notifications

    Data.gov (United States)

    U.S. Environmental Protection Agency — To help beachgoers make informed decisions about swimming at U.S. beaches, EPA gathers state-by-state data about beach closings and advisories. Between 1999 and...

  4. Thermodynamics of firms' growth

    Science.gov (United States)

    Zambrano, Eduardo; Hernando, Alberto; Hernando, Ricardo; Plastino, Angelo

    2015-01-01

    The distribution of firms' growth and firms' sizes is a topic under intense scrutiny. In this paper, we show that a thermodynamic model based on the maximum entropy principle, with dynamical prior information, can be constructed that adequately describes the dynamics and distribution of firms' growth. Our theoretical framework is tested against a comprehensive database of Spanish firms, which covers, to a very large extent, Spain's economic activity, with a total of 1 155 142 firms evolving along a full decade. We show that the empirical exponent of Pareto's law, a rule often observed in the rank distribution of large-size firms, is explained by the capacity of economic system for creating/destroying firms, and that can be used to measure the health of a capitalist-based economy. Indeed, our model predicts that when the exponent is larger than 1, creation of firms is favoured; when it is smaller than 1, destruction of firms is favoured instead; and when it equals 1 (matching Zipf's law), the system is in a full macroeconomic equilibrium, entailing ‘free’ creation and/or destruction of firms. For medium and smaller firm sizes, the dynamical regime changes, the whole distribution can no longer be fitted to a single simple analytical form and numerical prediction is required. Our model constitutes the basis for a full predictive framework regarding the economic evolution of an ensemble of firms. Such a structure can be potentially used to develop simulations and test hypothetical scenarios, such as economic crisis or the response to specific policy measures. PMID:26510828

  5. Immigration and Firm Expansion

    OpenAIRE

    William W. Olney

    2008-01-01

    Research generally focuses on how immigration affects native workers, while the impact of immigration on domestic firms is often overlooked. This paper addresses this important omission by examining whether firms respond to immigration by expanding their production activities within a city in order to utilize the abundant supply of low-skilled workers. Using data on immigration and the universe of establishments in U.S. cities, the results indicate that firms respond to immigration at the ext...

  6. Thermodynamics of firms' growth

    CERN Document Server

    Zambrano, Eduardo; Fernandez-Bariviera, Aurelio; Hernando, Ricardo; Plastino, Angelo

    2015-01-01

    The distribution of firms' growth and firms' sizes is a topic under intense scrutiny. In this paper we show that a thermodynamic model based on the Maximum Entropy Principle, with dynamical prior information, can be constructed that adequately describes the dynamics and distribution of firms' growth. Our theoretical framework is tested against a comprehensive data-base of Spanish firms, which covers to a very large extent Spain's economic activity with a total of 1,155,142 firms evolving along a full decade. We show that the empirical exponent of Pareto's law, a rule often observed in the rank distribution of large-size firms, is explained by the capacity of the economic system for creating/destroying firms, and can be used to measure the health of a capitalist-based economy. Indeed, our model predicts that when the exponent is larger that 1, creation of firms is favored; when it is smaller that 1, destruction of firms is favored instead; and when it equals 1 (matching Zipf's law), the system is in a full mac...

  7. Which firms use measures?

    DEFF Research Database (Denmark)

    Pedersen, Esben Rahbek Gjerdrum; Sudzina, Frantisek

    2012-01-01

    measurement practices. Design/methodology/approach – This paper hypothesises that firms dominated by organic capabilities and operating in unpredictable markets are more likely to adopt comprehensive PM systems. The statistical test of these hypotheses is based on a 2008 survey of 299 Danish firms. Findings....... Originality/value – Much has been said about how changes in the environment and business structure require firms to develop new ways to measure performance. Less has been done to study whether firms adopting comprehensive PM systems actually match the characteristics of the “new economy”. The findings from...

  8. Procurement with specialized firms

    DEFF Research Database (Denmark)

    Boone, Jan; Schottmüller, Christoph

    2016-01-01

    This paper analyzes optimal procurement mechanisms when firms are specialized. The procurement agency has incomplete information concerning the firms’ cost functions and values high quality as well as low price. Lower type firms are cheaper (more expensive) than higher type firms when providing low...... (high) quality. With specialized firms, distortion is limited and a mass of types earns zero profits. The optimal mechanism can be inefficient: types providing lower second best welfare win against types providing higher second best welfare. As standard scoring rule auctions cannot always implement...

  9. Virtual Beach 3: User's Guide

    Science.gov (United States)

    Virtual Beach version 3 (VB3) is a decision support tool that constructs site-specific statistical models to predict fecal indicator bacteria (FIB) concentrations at recreational beaches. VB3 is primarily designed for beach managers responsible for making decisions regarding beac...

  10. Huntington's Disease: An Immune Perspective

    Directory of Open Access Journals (Sweden)

    Annapurna Nayak

    2011-01-01

    Full Text Available Huntington's disease (HD is a progressive neurodegenerative disorder that is caused by abnormal expansion of CAG trinucleotide repeats. Neuroinflammation is a typical feature of most neurodegenerative diseases that leads to an array of pathological changes within the affected areas in the brain. The neurodegeneration in HD is also caused by aberrant immune response in the presence of aggregated mutant huntingtin protein. The effects of immune activation in HD nervous system are a relatively unexplored area of research. This paper summarises immunological features associated with development and progression of HD.

  11. Huntington disease: pathogenesis and treatment.

    Science.gov (United States)

    Dayalu, Praveen; Albin, Roger L

    2015-02-01

    Huntington disease (HD) is an autosomal dominant inherited neurodegenerative disease characterized by progressive motor, behavioral, and cognitive decline, culminating in death. It is caused by an expanded CAG repeat in the huntingtin gene. Even years before symptoms become overt, mutation carriers show subtle but progressive striatal and cerebral white matter atrophy by volumetric MRI. Although there is currently no direct treatment of HD, management options are available for several symptoms. A better understanding of HD pathogenesis, and more sophisticated clinical trials using newer biomarkers, may lead to meaningful treatments. This article reviews the current knowledge of HD pathogenesis and treatment.

  12. 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.

  13. Strong Firms Lobby, Weak Firms Bribe

    DEFF Research Database (Denmark)

    Bennedsen, Morten; Feldmann, Sven E.; Lassen, David Dreyer

    that are larger, older, exporting, government-owned, are widely held and/or have fewer competitors, have more political influence, perceive corruption to be less of a problem and pay bribes less often; (c) influence increases sales and government subsidies and in general makes the firm have a more positive view...

  14. [Molecular therapeutic strategies for Huntington's disease].

    Science.gov (United States)

    Milewski, Michał; Hoffman-Zacharska, Dorota; Ball, Jerzy

    2015-01-01

    Huntington's disease is a progressive neurodegenerative disorder of genetic origin that still lacks an effective treatment. Recently, a number of new attempts have been undertaken to develop a successful molecular therapy for this incurable condition. The novel approaches employ, among others, some new methods to selectively silence the mutated gene or to neutralize its toxic protein product. This paper reviews all major strategies that are currently considered for molecular therapy of Huntington's disease while discussing their potential effectiveness regarding the treatment of both the Huntington's disease and a large group of related neurodegenerative disorders associated with abnormal protein aggregation.

  15. Foreign Firms, Domestic Wages

    DEFF Research Database (Denmark)

    Malchow-Møller, Nikolaj; Schjerning, Bertel; R. Markusen, James

    2013-01-01

    Three types of theories have been used to explain the wage premium in foreign firms: the theories of heterogeneous workers, heterogeneous learning, and heterogeneous firms. We set up a model that explicitly encompasses two of these theories, and that can illustrate the third. This unifying....... In particular, the theory of heterogeneous workers can explain up to 75 percent of the premium....

  16. Foreign Firms, Domestic Wages

    DEFF Research Database (Denmark)

    Malchow-Møller, Nikolaj; Markusen, James R.; Schjerning, Bertel

    large domestic firms. The empirical implications of the model are tested on matched employer-employee data from Denmark. Consistent with the theory, we find considerable evidence of higher wages and wage growth in large and/or foreign-owned firms. These effects survive controlling for individual...

  17. Foreign Firms, Domestic Wages

    DEFF Research Database (Denmark)

    Malchow-Møller, Nikolaj; Schjerning, Bertel; R. Markusen, James

    2013-01-01

    Three types of theories have been used to explain the wage premium in foreign firms: the theories of heterogeneous workers, heterogeneous learning, and heterogeneous firms. We set up a model that explicitly encompasses two of these theories, and that can illustrate the third. This unifying framew...

  18. The market for firms

    DEFF Research Database (Denmark)

    Davis, Jerome Dean; Keiding, Hans

    2010-01-01

    and as a consequence a higher sales value of the firm. Buyers take this signaling into consideration, but irrespective of their countermoves, the equilib- rium result may be a lowering of ex ante product prices, and an ex post market overvaluation of the firm. This model is utilized to suggest possible explanations...

  19. Genetic modifiers of Huntington's disease.

    Science.gov (United States)

    Gusella, James F; MacDonald, Marcy E; Lee, Jong-Min

    2014-09-15

    Huntington's disease (HD) is a devastating neurodegenerative disorder that directly affects more than 1 in 10,000 persons in Western societies but, as a family disorder with a long, costly, debilitating course, it has an indirect impact on a far greater proportion of the population. Although some palliative treatments are used, no effective treatment exists for preventing clinical onset of the disorder or for delaying its inevitable progression toward premature death, approximately 15 years after diagnosis. Huntington's disease involves a movement disorder characterized by chorea, as well as a variety of psychiatric disturbances and intellectual decline, with a gradual loss of independence. A dire need exists for effective HD therapies to alleviate the suffering and costs to the individual, family, and health care system. In past decades, genetics, the study of DNA sequence variation and its consequences, provided the tools to map the HD gene to chromosome 4 and ultimately to identify its mutation as an expanded CAG trinucleotide repeat in the coding sequence of a large protein, dubbed huntingtin. Now, advances in genetic technology offer an unbiased route to the identification of genetic factors that are disease-modifying agents in human patients. Such genetic modifiers are expected to highlight processes capable of altering the course of HD and therefore to provide new, human-validated targets for traditional drug development, with the goal of developing rational treatments to delay or prevent onset of HD clinical signs.

  20. Corruption and firm performance: Evidence from Greek firms

    OpenAIRE

    2012-01-01

    This article investigates the relationship between corruption and firm performance in Greece using firm level data. Corruption is overall negatively associated with firm size and growth at the firm level. We focus on the effect of ‘administrative corruption’, whereby firms engage in corrupt practices and bribery of government officials. We contrast the firm experience of corruption and the contextual experience of corruption at the sectoral level and find that the latter, contextual corruptio...

  1. Huntington's disease: review and anesthetic case management.

    Science.gov (United States)

    Cangemi, C F; Miller, R J

    1998-01-01

    Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. Patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depression, and apathy leads to cachexia. Factors of considerable concern to the anesthesiologist who treats patients with Huntington's disease may include how to treat frail elderly people incapable of cooperation, how to treat patients suffering from malnourishment, and how to treat patients with an increased risk for aspiration or exaggerated responses to sodium thiopental and succinylcholine. The successful anesthetic management of a 65-yr-old woman with Huntington's disease who presented for full-mouth extractions is described.

  2. Huntington's Disease: Speech, Language and Swallowing

    Science.gov (United States)

    ... Disease Society of America Huntington's Disease Youth Organization Movement Disorder Society National Institute of Neurological Disorders and Stroke Typical Speech and Language Development Learning More Than One Language Adult Speech and Language Child Speech and Language Swallowing ...

  3. Impaired mitochondrial trafficking in Huntington's disease

    OpenAIRE

    Li, Xiao-Jiang; Orr, Adam L.; Li, Shihua

    2009-01-01

    Abstract Impaired mitochondrial function has been well documented in Huntington?s disease. Mutant huntingtin is found to affect mitochondria via various mechanisms including the dysregulation of gene transcription and impairment of mitochondrial function or trafficking. The lengthy and highly branched neuronal processes constitute complex neural networks in which there is a large demand for mitochondria-generated energy. Thus, the impaired mitochondria trafficking in neuronal cells...

  4. 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.

  5. Global Sourcing and Firm Selection

    DEFF Research Database (Denmark)

    Kohler, Wilhelm; Smolka, Marcel

    2014-01-01

    We analyze the sourcing strategies of firms active in the Spanish manufacturing sector. We show that firms that select strategies of vertical integration and of foreign sourcing ex post tend to have been more productive, ex ante, than other firms.......We analyze the sourcing strategies of firms active in the Spanish manufacturing sector. We show that firms that select strategies of vertical integration and of foreign sourcing ex post tend to have been more productive, ex ante, than other firms....

  6. Ethical issues and Huntington's disease.

    Science.gov (United States)

    Kromberg, Jennifer G R; Wessels, Tina-Marié

    2013-10-11

    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 twin sought predictive testing for this dominantly inherited disease, created several ethical dilemmas. Another case where predictive testing was carried out on two young children, at high risk, by a laboratory at the request of an adoption agency and a doctor, with a view to giving information to the foster parents, also posed many ethical conundrums for the counsellor. The ethical issues that arose in these cases are discussed in this paper. 

  7. Cholesterol metabolism in Huntington disease.

    Science.gov (United States)

    Karasinska, Joanna M; Hayden, Michael R

    2011-09-06

    The CNS is rich in cholesterol, which is essential for neuronal development and survival, synapse maturation, and optimal synaptic activity. Alterations in brain cholesterol homeostasis are linked to neurodegeneration. Studies have demonstrated that Huntington disease (HD), a progressive and fatal neurodegenerative disorder resulting from polyglutamine expansion in the huntingtin protein, is associated with changes in cellular cholesterol metabolism. Emerging evidence from human and animal studies indicates that attenuated brain sterol synthesis and accumulation of cholesterol in neuronal membranes represent two distinct mechanisms occurring in the presence of mutant huntingtin that influence neuronal survival. Increased knowledge of how changes in intraneuronal cholesterol metabolism influence the pathogenesis of HD will provide insights into the potential application of brain cholesterol regulation as a therapeutic strategy for this devastating disease.

  8. Juvenile Huntington disease in Argentina.

    Science.gov (United States)

    Gatto, Emilia Mabel; Parisi, Virginia; Etcheverry, José Luis; Sanguinetti, Ana; Cordi, Lorena; Binelli, Adrian; Persi, Gabriel; Squitieri, Ferdinando

    2016-01-01

    We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international series, however, in this context we identified the highest JHD frequency reported so far (19.72%; 14/71). Age at onset of JHD is challenging and still under discussion. Our findings reinforce the hypothesis that clinical manifestations, other than the typical movement disorder, may anticipate age at onset of even many years. Analyses of JHD cohorts are required to explore it frequency in populations with different backgrounds to avoid an underestimation of this rare phenotype. Moreover, data from selected populations may open new pathways in therapeutic approaches and may explain new potential correlations between HD presentations and environmental or biological factors.

  9. Language impairment in Huntington's disease.

    Science.gov (United States)

    Azambuja, Mariana Jardim; Radanovic, Marcia; Haddad, Mônica Santoro; Adda, Carla Cristina; Barbosa, Egberto Reis; Mansur, Letícia Lessa

    2012-06-01

    Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (poral comprehension (preading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

  10. Firms, crowds, and innovation.

    Science.gov (United States)

    Felin, Teppo; Lakhani, Karim R; Tushman, Michael L

    2017-05-01

    The purpose of this article is to suggest a (preliminary) taxonomy and research agenda for the topic of "firms, crowds, and innovation" and to provide an introduction to the associated special issue. We specifically discuss how various crowd-related phenomena and practices-for example, crowdsourcing, crowdfunding, user innovation, and peer production-relate to theories of the firm, with particular attention on "sociality" in firms and markets. We first briefly review extant theories of the firm and then discuss three theoretical aspects of sociality related to crowds in the context of strategy, organizations, and innovation: (1) the functions of sociality (sociality as extension of rationality, sociality as sensing and signaling, sociality as matching and identity), (2) the forms of sociality (independent/aggregate and interacting/emergent forms of sociality), and (3) the failures of sociality (misattribution and misapplication). We conclude with an outline of future research directions and introduce the special issue papers and essays.

  11. 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

  12. The Multinational Firm

    DEFF Research Database (Denmark)

    Morgan, Glenn; Hull Kristensen, Peer; Whitley, Richard

    In contrast to the traditional view of multinational firms as cohesive rational actors maximizing the use of resources across national boundaries, the contributors to this volume argue that they are complex social arenas where competing groups draw on resources from their own socially......-embedded locations in developing new transnational social relationships. As firms seek to manage across national and institutional boundaries, they stretch their existing capacities and routines and develop new sets of transnational social relationships through different groups competing and cooperating...

  13. 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...

  14. Sand hazards on tourist beaches.

    Science.gov (United States)

    Heggie, Travis W

    2013-01-01

    Visiting the beach is a popular tourist activity worldwide. Unfortunately, the beach environment is abundant with hazards and potential danger to the unsuspecting tourist. While the traditional focus of beach safety has been water safety oriented, there is growing concern about the risks posed by the sand environment on beaches. This study reports on the death and near death experience of eight tourists in the collapse of sand holes, sand dunes, and sand tunnels. Each incident occurred suddenly and the complete burial in sand directly contributed to the victims injury or death in each case report.

  15. Silencing Huntington's chorea: Is RNA Interference a Potential Cure?

    OpenAIRE

    Metz, Gerlinde A.; Whishaw, Ian Q.; Afra Foroud; Nafisa M Jadavji

    2006-01-01

    In 1872, George Huntington described Huntington's disease as characterized by motor, cognitive and psychiatric impairments. Huntington's disease is a dominant and autosomal mutation on chromosome 4 featuring the insertion of numerous CAG repeats. CAG codes for the amino acid, glutmanine that forms part of the Huntingtin protein (htt). Excess glutamine attachments make htt prone to accumulate in neurons. Three genes can be considered when developing therapies for Huntington's disease. They inc...

  16. Executive compensation and firm performance: Evidence from Indian firms

    Directory of Open Access Journals (Sweden)

    Mehul Raithatha

    2016-09-01

    Full Text Available The study examines the relationship between executive compensation and firm performance among Indian firms. The evidence suggests that firm performance measured by accounting, as well as market-based measures, significantly affects executive compensation. We also test for the presence of persistence in executive compensation by employing the system-generalised methods of moments (GMM estimator. We find significant persistence in executive compensation among the sample firms. Further, we report the absence of pay–performance relationship among the smaller sample firms and business group affiliated firms. Thus, our findings cast doubts over the performance-based executive compensation practices of Indian business group affiliated firms.

  17. Exercise effects in Huntington disease.

    Science.gov (United States)

    Frese, Sebastian; Petersen, Jens A; Ligon-Auer, Maria; Mueller, Sandro Manuel; Mihaylova, Violeta; Gehrig, Saskia M; Kana, Veronika; Rushing, Elisabeth J; Unterburger, Evelyn; Kägi, Georg; Burgunder, Jean-Marc; Toigo, Marco; Jung, Hans H

    2017-01-01

    Huntington disease (HD) is a relentlessly progressive neurodegenerative disorder with symptoms across a wide range of neurological domains, including cognitive and motor dysfunction. There is still no causative treatment for HD but environmental factors such as passive lifestyle may modulate disease onset and progression. In humans, multidisciplinary rehabilitation has a positive impact on cognitive functions. However, a specific role for exercise as a component of an environmental enrichment effect has been difficult to demonstrate. We aimed at investigating whether endurance training (ET) stabilizes the progression of motor and cognitive dysfunction and ameliorates cardiovascular function in HD patients. Twelve male HD patients (mean ± SD, 54.8 ± 7.1 years) and twelve male controls (49.1 ± 6.8 years) completed 26 weeks of endurance training. Before and after the training intervention, clinical assessments, exercise physiological tests, and a body composition measurement were conducted and a muscle biopsy was taken from M. vastus lateralis. To examine the natural course of the disease, HD patients were additionally assessed 6 months prior to ET. During the ET period, there was a motor deficit stabilization as indicated by the Unified Huntington's Disease Rating Scale motor section score in HD patients (baseline: 18.6 ± 9.2, pre-training: 26.0 ± 13.7, post-training: 26.8 ± 16.4). Peak oxygen uptake ([Formula: see text]) significantly increased in HD patients (∆[Formula: see text] = +0.33 ± 0.28 l) and controls (∆[Formula: see text] = +0.29 ± 0.41 l). No adverse effects of the training intervention were reported. Our results confirm that HD patients are amenable to a specific exercise-induced therapeutic strategy indicated by an increased cardiovascular function and a stabilization of motor function.

  18. Modern Competitive Strategy Firm

    Directory of Open Access Journals (Sweden)

    František Bartes

    2010-01-01

    Full Text Available The article deals with problems of an ever increasing number of cases of successful application of old combat strategies in business practice. Nevertheless, the author refuses the so called “direct conflict“ of firms, when it is really fought for victory on the market. His approach is based on the philosophy of “victory without fight“, in other words – of achieving the aim without direct conflict with the rival firm.In the end he comes to the conclusion that combat philosophies are more and more often incorporated into classical approach of strategic firm control and become the integral part of philosophy of management. At the close happens to opinion that the one from basic factors absolutely necessary to creation such strategy at company, is necessary present so-called „creative intelligence“.

  19. Foreign Firms, Domestic Wages

    DEFF Research Database (Denmark)

    Malchow-Møller, Nikolaj; Markusen, James R.; Schjerning, Bertel

    by developing a model that allows for two "pure" explanations for the wage premium. The first is a heterogenous-worker explanation along the lines of Yeaple (2005), where firms that select more scaleintensive technologies select ex-ante more productive workers. In this case, the wage premium is a pure selection...... phenomenon. The second explanation builds on the heterogeneous-firm model of Melitz (2003) combined with on-the-job learning as in Markusen (2001). Productivity differences between firms are internalized by ex-ante homogeneous workers, so the wage premium is a pure learning phenomenon due to ex-post higher...... by selection are in the neighborhood of 30-65% of the total premium, with the remainder consistent with learning. There is also considerable support for a number of other predictions specific to the worker-learning explanation....

  20. Innovation in Family Firms

    DEFF Research Database (Denmark)

    Filser, Matthias; Brem, Alexander; Gast, Johanna

    2016-01-01

    Over the past decade, research on innovation in family firms has received growing attention by scholars and practitioners around the globe with a wide range of aspects explored within the current body of literature. Despite the constantly growing number of scientific publications, research lacks...... a comprehensive and critical review of past and present research achievements. First, conducting a bibliometric analysis with a focus on innovation in family firms, we identify five topical clusters that help to understand the foundations of recent findings: namely ownership and governance, structural settings......, organizational culture and behaviour, resources, and innovation and strategy. Second, based on a thorough literature review the major research avenues are reflected. The comparison of the results of both analyses showed the following areas for future research on family firm innovation: members‘ individual human...

  1. Collaborative Communities of Firms

    DEFF Research Database (Denmark)

    2011-01-01

    Both small and medium-size entrepreneurial firms face liabilities such as resource scarcity and scale diseconomies, making it difficult for them to innovate on a continuous basis. In response, experimentation with new ways of organizing for innovation has increased. One successful result is an or......Both small and medium-size entrepreneurial firms face liabilities such as resource scarcity and scale diseconomies, making it difficult for them to innovate on a continuous basis. In response, experimentation with new ways of organizing for innovation has increased. One successful result...

  2. Biochemical aspects of Huntington's chorea.

    Science.gov (United States)

    Caraceni, T; Calderini, G; Consolazione, A; Riva, E; Algeri, S; Girotti, F; Spreafico, R; Branciforti, A; Dall'olio, A; Morselli, P L

    1977-01-01

    Fifteen patients affected by Huntington's chorea were divided into two groups, 'slow' and 'fast', according to IQ scores on the Wechsler-Bellevue scale, and scores on some motor performance tests. A possible correlation was looked for between some biochemical data (cerebrospinal fluid (CSF), homovanillic acid (HVA), and 5-hydroxyindolacetic acid (5HIAA) levels, plasma dopamine-beta-hydroxylase (DBH), dopamine (DA) uptake by platelets), and clinical data (duration of illness, severity of symptoms, age of patients, IQ scores, 'slow' and 'fast' groups). The CSF, HVA, and 5HIAA levels were found to be significantly lowered in comparison with normal controls. DBH activity and DA uptake by platelets did not differ significantly from normal subjects. Treatment with haloperidol in all patients and with dipropylacetic acid in three patients did not appear to modify the CSF, HVA, and 5HIAA concentrations, the plasma DBH activity, or the DA uptake. There were no significant differences in the CSF, HVA, and 5HIAA contents between the two groups of patients, and there was no correlation between biochemical data and clinical features. PMID:143508

  3. Protein oxidation in Huntington disease.

    Science.gov (United States)

    Sorolla, M Alba; Rodríguez-Colman, María José; Vall-llaura, Núria; Tamarit, Jordi; Ros, Joaquim; Cabiscol, Elisa

    2012-01-01

    Huntington disease (HD) is an inherited neurodegenerative disorder caused by expansion of CAG repeats in the huntingtin gene, affecting initially the striatum and progressively the cortex. Oxidative stress, and consequent protein oxidation, has been described as important to disease progression. This review focuses on recent advances in the field, with a particular emphasis on the identified target proteins and the role that their oxidation has or might have in the pathophysiology of HD. Oxidation and the resulting inactivation and/or degradation of important proteins can explain the impairment of several metabolic pathways in HD. Oxidation of enzymes involved in ATP synthesis can account for the energy deficiency observed. Impairment of protein folding and degradation can be due to oxidation of several heat shock proteins and Valosin-containing protein. Oxidation of two enzymes involved in the vitamin B6 metabolism could result in decreased availability of pyridoxal phosphate, which is a necessary cofactor in transaminations, the kynurenine pathway and the synthesis of glutathione, GABA, dopamine and serotonin, all of which have a key role in HD pathology. In addition, protein oxidation often contributes to oxidative stress, aggravating the molecular damage inside the cell. Copyright © 2012 International Union of Biochemistry and Molecular Biology, Inc.

  4. Error processing in Huntington's disease.

    Directory of Open Access Journals (Sweden)

    Christian Beste

    Full Text Available BACKGROUND: Huntington's disease (HD is a genetic disorder expressed by a degeneration of the basal ganglia inter alia accompanied with dopaminergic alterations. These dopaminergic alterations are related to genetic factors i.e., CAG-repeat expansion. The error (related negativity (Ne/ERN, a cognitive event-related potential related to performance monitoring, is generated in the anterior cingulate cortex (ACC and supposed to depend on the dopaminergic system. The Ne is reduced in Parkinson's Disease (PD. Due to a dopaminergic deficit in HD, a reduction of the Ne is also likely. Furthermore it is assumed that movement dysfunction emerges as a consequence of dysfunctional error-feedback processing. Since dopaminergic alterations are related to the CAG-repeat, a Ne reduction may furthermore also be related to the genetic disease load. METHODOLOGY/PRINCIPLE FINDINGS: We assessed the error negativity (Ne in a speeded reaction task under consideration of the underlying genetic abnormalities. HD patients showed a specific reduction in the Ne, which suggests impaired error processing in these patients. Furthermore, the Ne was closely related to CAG-repeat expansion. CONCLUSIONS/SIGNIFICANCE: The reduction of the Ne is likely to be an effect of the dopaminergic pathology. The result resembles findings in Parkinson's Disease. As such the Ne might be a measure for the integrity of striatal dopaminergic output function. The relation to the CAG-repeat expansion indicates that the Ne could serve as a gene-associated "cognitive" biomarker in HD.

  5. Predictive testing for Huntington's disease.

    Science.gov (United States)

    Tibben, Aad

    2007-04-30

    Worldwide, predictive testing for Huntington's disease has become an accepted clinical application that has allowed many individuals from HD-families to proceed with their life without the uncertainty of being at risk. International guidelines have extensively contributed to establishing counselling programmes of high quality, and have served as a model for other genetic disorders. Psychological follow-up studies have increased the insight into the far-reaching impact of test results for all individuals involved. Although the guidelines have served as a useful frame of reference, clinical experience has shown the importance of a case-by-case approach to do justice to the specific needs of the individual test candidate. Issues such as ambiguous test results, lack of awareness in a test candidate of early signs of the disease, non-compliance to the test protocol, or the test candidate's need for information on the relationship between age at onset and CAG-repeat require careful consideration. Receiving a test result is only one of the transition points in the life of an individual at risk; such result needs to be valued from a life-cycle perspective.

  6. Huntington's Disease and Striatal Signaling.

    Science.gov (United States)

    Roze, Emmanuel; Cahill, Emma; Martin, Elodie; Bonnet, Cecilia; Vanhoutte, Peter; Betuing, Sandrine; Caboche, Jocelyne

    2011-01-01

    Huntington's Disease (HD) is the most frequent neurodegenerative disease caused by an expansion of polyglutamines (CAG). The main clinical manifestations of HD are chorea, cognitive impairment, and psychiatric disorders. The transmission of HD is autosomal dominant with a complete penetrance. HD has a single genetic cause, a well-defined neuropathology, and informative pre-manifest genetic testing of the disease is available. Striatal atrophy begins as early as 15 years before disease onset and continues throughout the period of manifest illness. Therefore, patients could theoretically benefit from therapy at early stages of the disease. One important characteristic of HD is the striatal vulnerability to neurodegeneration, despite similar expression of the protein in other brain areas. Aggregation of the mutated Huntingtin (HTT), impaired axonal transport, excitotoxicity, transcriptional dysregulation as well as mitochondrial dysfunction, and energy deficits, are all part of the cellular events that underlie neuronal dysfunction and striatal death. Among these non-exclusive mechanisms, an alteration of striatal signaling is thought to orchestrate the downstream events involved in the cascade of striatal dysfunction.

  7. Therapeutic advances in Huntington's Disease.

    Science.gov (United States)

    Shannon, Kathleen M; Fraint, Avram

    2015-09-15

    Huntington's disease is a rare hereditary degenerative disease with a wide variety of symptoms that encompass movement, cognition, and behavior. The genetic mutation that causes the disease has been known for more than 20 y, and animal models have illuminated a host of intracellular derangements that occur downstream of protein translation. A number of clinical trials targeting these metabolic consequences have failed to produce a single effective therapy, although clinical trials continue. New strategies targeting the protein at the level of transcription, translation, and posttranslational modification and aggregation engender new hope that a successful strategy will emerge, but there is much work ahead. Some of the clinical manifestations of the illness, particularly chorea, affective symptoms, and irritability, are amenable to palliative strategies, but physicians have a poor evidence base on which to select the best agents. Clinical trials since 2013 have dashed hopes that coenzyme Q10 or creatine might have disease-modifying properties but suggested other agents were safe or hinted at efficacy (cysteamine, selisistat, hydroxyquinoline) and could proceed into later-stage disease modification trials. The hunt for effective symptom relief suggested that pridopidine might be shown effective given the right outcome measure. This review summarizes recent progress in HD and highlights promising new strategies for slowing disease progression and relieving suffering in HD. © 2015 International Parkinson and Movement Disorder Society.

  8. Huntington's disease: a clinical review.

    Science.gov (United States)

    McColgan, Peter; Tabrizi, Sarah J

    2017-08-17

    Huntington's disease (HD) is a fully penetrant neurodegenerative disease caused by a dominantly inherited CAG trinucleotide repeat expansion in the huntingtin gene on chromosome 4. In Western populations HD has a prevalence of 10.6-13.7 individuals per 100,000. It is characterised by cognitive, motor and psychiatric disturbance. At the cellular level mutant huntingtin results in neuronal dysfunction and death through a number of mechanisms, including disruption of proteostasis, transcription and mitochondrial function and direct toxicity of the mutant protein. Early macroscopic changes are seen in the striatum with involvement of the cortex as the disease progresses. There are currently no disease modifying treatments therefore supportive and symptomatic management is the mainstay of treatment. In recent years there have been significant advances in understanding both the cellular pathology and the macroscopic structural brain changes that occur as the disease progresses. In the last decade there has been a large growth in potential therapeutic targets and clinical trials. Perhaps the most promising of these are the emerging therapies aimed at lowering levels of mutant huntingtin. Antisense oligonucleotide therapy is one such approach with clinical trials currently underway. This may bring us one step closer to treating and potentially preventing this devastating condition. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  9. Movement sequencing in Huntington disease.

    Science.gov (United States)

    Georgiou-Karistianis, Nellie; Long, Jeffrey D; Lourens, Spencer G; Stout, Julie C; Mills, James A; Paulsen, Jane S

    2014-08-01

    To examine longitudinal changes in movement sequencing in prodromal Huntington's disease (HD) participants (795 prodromal HD; 225 controls) from the PREDICT-HD study. Prodromal HD participants were tested over seven annual visits and were stratified into three groups (low, medium, high) based on their CAG-Age Product (CAP) score, which indicates likely increasing proximity to diagnosis. A cued movement sequence task assessed the impact of advance cueing on response initiation and execution via three levels of advance information. Compared to controls, all CAP groups showed longer initiation and movement times across all conditions at baseline, demonstrating a disease gradient for the majority of outcomes. Across all conditions, the high CAP group had the highest mean for baseline testing, but also demonstrated an increase in movement time across the study. For initiation time, the high CAP group showed the highest mean baseline time across all conditions, but also faster decreasing rates of change over time. With progress to diagnosis, participants may increasingly use compensatory strategies, as evidenced by faster initiation. However, this occurred in conjunction with slowed execution times, suggesting a decline in effectively accessing control processes required to translate movement into effective execution.

  10. Language impairment in Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Jardim Azambuja

    2012-06-01

    Full Text Available Language alterations in Huntington's disease (HD are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT. HD patients performed poorer in verbal fluency (p<0.0001, oral comprehension (p<0.0001, repetition (p<0.0001, oral agility (p<0.0001, reading comprehension (p=0.034 and narrative writing (p<0.0001. There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively. Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

  11. 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.

  12. Unusual early-onset Huntingtons disease.

    Science.gov (United States)

    Vargas, Antonio P; Carod-Artal, Francisco J; Bomfim, Denise; Vázquez-Cabrera, Carolina; Dantas-Barbosa, Carmela

    2003-06-01

    Huntington's disease is an autosomal dominant progressive neurodegenerative disorder characterized by involuntary movements, cognitive decline, and behavioral disorders leading to functional disability. In contrast to patients with adult onset, in which chorea is the major motor abnormality, children often present with spasticity, rigidity, and significant intellectual decline associated with a more rapidly progressive course. An unusual early-onset Huntington's disease case of an 11-year-old boy with severe hypokinetic/rigid syndrome appearing at the age of 2.5 years is presented. Clinical diagnosis was confirmed by polymerase chain reaction study of the expanded IT-15 allele with a compatible size of 102 cytosine-adenosine-guanosine repeats L-Dopa mildly ameliorated rigidity, bradykinesia, and dystonia. We conclude that Huntington's disease should be included in the differential diagnoses of regressive syndromes of early childhood.

  13. Development of biomarkers for Huntington's disease.

    Science.gov (United States)

    Weir, David W; Sturrock, Aaron; Leavitt, Blair R

    2011-06-01

    Huntington's disease is an autosomal dominant, progressive neurodegenerative disorder, for which there is no disease-modifying treatment. By use of predictive genetic testing, it is possible to identify individuals who carry the gene defect before the onset of symptoms, providing a window of opportunity for intervention aimed at preventing or delaying disease onset. However, without robust and practical measures of disease progression (ie, biomarkers), the efficacy of therapeutic interventions in this premanifest Huntington's disease population cannot be readily assessed. Current progress in the development of biomarkers might enable evaluation of disease progression in individuals at the premanifest stage of the disease; these biomarkers could be useful in defining endpoints in clinical trials in this population. Clinical, cognitive, neuroimaging, and biochemical biomarkers are being investigated for their potential in clinical use and their value in the development of future treatments for patients with Huntington's disease.

  14. Monkey hybrid stem cells develop cellular features of Huntington's disease

    Directory of Open Access Journals (Sweden)

    Lorthongpanich Chanchao

    2010-02-01

    Full Text Available Abstract Background Pluripotent stem cells that are capable of differentiating into different cell types and develop robust hallmark cellular features are useful tools for clarifying the impact of developmental events on neurodegenerative diseases such as Huntington's disease. Additionally, a Huntington's cell model that develops robust pathological features of Huntington's disease would be valuable for drug discovery research. Results To test this hypothesis, a pluripotent Huntington's disease monkey hybrid cell line (TrES1 was established from a tetraploid Huntington's disease monkey blastocyst generated by the fusion of transgenic Huntington's monkey skin fibroblast and a wild-type non-transgenic monkey oocyte. The TrES1 developed key Huntington's disease cellular pathological features that paralleled neural development. It expressed mutant huntingtin and stem cell markers, was capable of differentiating to neural cells, and developed teratoma in severely compromised immune deficient (SCID mice. Interestingly, the expression of mutant htt, the accumulation of oligomeric mutant htt and the formation of intranuclear inclusions paralleled neural development in vitro , and even mutant htt was ubiquitously expressed. This suggests the development of Huntington's disease cellular features is influenced by neural developmental events. Conclusions Huntington's disease cellular features is influenced by neural developmental events. These results are the first to demonstrate that a pluripotent stem cell line is able to mimic Huntington's disease progression that parallels neural development, which could be a useful cell model for investigating the developmental impact on Huntington's disease pathogenesis.

  15. Maternal transmission in sporadic Huntington's disease.

    OpenAIRE

    Sánchez, A; Milà, M.; Castellví-Bel, S; Rosich, M; Jiménez, D; Badenas, C.; ESTIVILL, X.

    1997-01-01

    Huntington's disease is an autosomal dominant neurodegenerative disorder caused by the expansion of a (CAG)n repeat in the IT15 gene. Three per cent of cases are sporadic and in those in which family studies have been performed, the origin of the mutation was always paternal. The first sporadic case of Huntington's disease is presented in which a premutated maternal allele of 37 CAG repeats was transmitted expanded to the proband (43 CAG repeats). Molecular analysis of the IT15 gene is extrem...

  16. Foreign Firms, Domestic Wages

    DEFF Research Database (Denmark)

    Malchow-Møller, Nikolaj; Markusen, James R.; Schjerning, Bertel

    Many papers have documented a wage premium in foreign-owned and large firms. However, there is very little formal theory in the literature and empirical analyses are typically not based on hypotheses which are rigorously derived from theory. This paper contributes to the theory-empirics gap by de...

  17. Firms, crowds, and innovation

    Science.gov (United States)

    Felin, Teppo; Lakhani, Karim R; Tushman, Michael L

    2017-01-01

    The purpose of this article is to suggest a (preliminary) taxonomy and research agenda for the topic of “firms, crowds, and innovation” and to provide an introduction to the associated special issue. We specifically discuss how various crowd-related phenomena and practices—for example, crowdsourcing, crowdfunding, user innovation, and peer production—relate to theories of the firm, with particular attention on “sociality” in firms and markets. We first briefly review extant theories of the firm and then discuss three theoretical aspects of sociality related to crowds in the context of strategy, organizations, and innovation: (1) the functions of sociality (sociality as extension of rationality, sociality as sensing and signaling, sociality as matching and identity), (2) the forms of sociality (independent/aggregate and interacting/emergent forms of sociality), and (3) the failures of sociality (misattribution and misapplication). We conclude with an outline of future research directions and introduce the special issue papers and essays. PMID:28690428

  18. Whom do new firms hire?

    DEFF Research Database (Denmark)

    2015-01-01

    ’s productivity is based on its pre-entry experience and persistent shocks, and over time firms learn about their productivity. The model predicts that more productive firms are larger and hire more talented employees, which gives rise to various predictions about how pre-entry experience, firm growth rates......Using the matched employer-employee data set for Denmark and information on the founders of new firms, we analyze the hiring choices of all new firms that entered from 2003 to 2010. We develop a theoretical model in which the quality of a firm’s employees determines its average cost, a firm...

  19. Governance in a beach seine fishery

    NARCIS (Netherlands)

    Medard, Modesta; Dijk, Van Han; Hebinck, Paul; Geheb, Kim

    2016-01-01

    Beach seine gear is one of the prominent fishing gears in Nile Perch fishery. Before Nile Perch was introduced to the lake, beach seines the species targeted with beach seine were Tilapia, Bagrus, Haplochromis, Protopterus and Labeo. In 1994, beach seines were banned in Tanzania and by 2004, this

  20. Are Private Firms Really More Tax Aggressive Than Public Firms ?

    OpenAIRE

    Pierk, Jochen

    2016-01-01

    This paper tests the notion that private firms are more tax aggressive than public firms. Tax avoidance measures, e.g. effective tax rates, cannot be used to compare private and public firms when private and public firms have different levels of importance on financial accounting earnings (Hanlon and Heitzman 2010). To disentangle financial reporting incentives from tax aggressiveness, I use the fact that European groups must prepare two sets of financial statements: first, gro...

  1. Pathogenic insights from Huntington's disease-like 2 and other Huntington's disease genocopies.

    Science.gov (United States)

    Margolis, Russell L; Rudnicki, Dobrila D

    2016-12-01

    Huntington's disease-like 2 (HDL2) is a rare, progressive, autosomal dominant neurodegenerative disorder that genetically, clinically, and pathologically closely resembles Huntington's disease. We review HDL2 pathogenic mechanisms and examine the implications of these mechanisms for Huntington's disease and related diseases. HDL2 is caused by a CTG/CAG repeat expansion in junctophilin-3. Available data from cell and animal models and human brain suggest that HDL2 is a complex disease in which transcripts and proteins expressed bidirectionally from the junctophilin-3 locus contribute to pathogenesis through both gain-and loss-of-function mechanisms. Recent advances indicate that the pathogenesis of Huntington's disease is equally complex, despite the emphasis on toxic gain-of-function properties of the mutant huntingtin protein. Studies examining in parallel the genetic, clinical, neuropathological, and mechanistic similarities between Huntington's disease and HDL2 have begun to identify points of convergence between the pathogenic pathways of the two diseases. Comparisons to other diseases that are phenotypically or genetically related to Huntington's disease and HDL2 will likely reveal additional common pathways. The ultimate goal is to identify shared therapeutic targets and eventually develop therapies that may, at least in part, be effective across multiple similar rare diseases, an essential approach given the scarcity of resources for basic and translational research.

  2. Firm-Specific Learning and the Nature of the Firm

    DEFF Research Database (Denmark)

    Knudsen, Thorbjørn

    2007-01-01

    This article discusses the limits of a purely transaction cost explanation of the existence of the firm by pointing to additional factors. A simple heuristic model shows that firm-specific learning effects can overcome the costs of monitoring employees, and explain the existence of the firm even ...

  3. Beach ridges and prograded beach deposits as palaeoenvironment records

    Science.gov (United States)

    Tamura, Toru

    2012-09-01

    Beach ridges are landforms commonly developed on prograded coasts with beach shorelines. A sequence of beach ridges, coupled with their subsurface deposits, can be regarded as a time series of coastal evolution. Methodological advances in field surveying and chronology applicable to beach ridges have led to detailed palaeoenvironmental reconstructions to be derived from such sequences. This paper reconsiders the basic aspects of beach ridges and deposits, which need to be properly understood for their comprehensive interpretation in a palaeo-environmental context. It also reviews case studies in which beach-ridge sequences have been used to unveil past sea-level history, catastrophic events, and climate changes. Proposed formative processes of beach ridges include: 1) progradation of sandy beach and berm formations in relation to fairweather waves, coupled with aeolian foredune accumulation; 2) building of gravel ridges by storm waves; 3) welding of longshore bars. Beach-ridge formation through sea-level oscillation is thought to be questionable and caution is suggested for this process when undertaking palaeoenvironmental reconstruction. Beach deposit stratification is known to dip either landwards or seawards, but landward dips are uncommon. Seaward dipping stratification is formed in relation to beachface progradation, and is usually dissected in places by erosion surfaces resulting from episodic beach retreat. The boundary between the foreshore and the underlying shoreface is well defined only in the case that longshore bars lead to complex bedding structure relative to that of the foreshore. Reliable chronology of beach ridges can be determined by radiocarbon and optically-stimulated luminescence (OSL) dating. Radiocarbon dating of articulated shells, which are considered not to be extensively reworked, provides robust results, but OSL dating is more useful as it enables direct dating of sediment grains. It is noted that there are restrictions in chronological

  4. Beach Volume Change Using Uav Photogrammetry Songjung Beach, Korea

    Science.gov (United States)

    Yoo, C. I.; Oh, T. S.

    2016-06-01

    Natural beach is controlled by many factors related to wave and tidal forces, wind, sediment, and initial topography. For this reason, if numerous topographic data of beach is accurately collected, coastal erosion/acceleration is able to be assessed and clarified. Generally, however, many studies on coastal erosion have limitation to analyse the whole beach, carried out of partial area as like shoreline (horizontal 2D) and beach profile (vertical 2D) on account of limitation of numerical simulation. This is an important application for prevention of coastal erosion, and UAV photogrammetry is also used to 3D topographic data. This paper analyses the use of unmanned aerial vehicles (UAV) to 3D map and beach volume change. UAV (Quadcopter) equipped with a non-metric camera was used to acquire images in Songjung beach which is located south-east Korea peninsula. The dynamics of beach topography, its geometric properties and estimates of eroded and deposited sand volumes were determined by combining elevation data with quarterly RTK-VRS measurements. To explore the new possibilities for assessment of coastal change we have developed a methodology for 3D analysis of coastal topography evolution based on existing high resolution elevation data combined with low coast, UAV and on-ground RTK-VRS surveys. DSMs were obtained by stereo-matching using Agisoft Photoscan. Using GCPs the vertical accuracy of the DSMs was found to be 10 cm or better. The resulting datasets were integrated in a local coordinates and the method proved to be a very useful fool for the detection of areas where coastal erosion occurs and for the quantification of beach change. The value of such analysis is illustrated by applications to coastal of South Korea sites that face significant management challenges.

  5. BEACH VOLUME CHANGE USING UAV PHOTOGRAMMETRY SONGJUNG BEACH, KOREA

    Directory of Open Access Journals (Sweden)

    C. I. Yoo

    2016-06-01

    Full Text Available Natural beach is controlled by many factors related to wave and tidal forces, wind, sediment, and initial topography. For this reason, if numerous topographic data of beach is accurately collected, coastal erosion/acceleration is able to be assessed and clarified. Generally, however, many studies on coastal erosion have limitation to analyse the whole beach, carried out of partial area as like shoreline (horizontal 2D and beach profile (vertical 2D on account of limitation of numerical simulation. This is an important application for prevention of coastal erosion, and UAV photogrammetry is also used to 3D topographic data. This paper analyses the use of unmanned aerial vehicles (UAV to 3D map and beach volume change. UAV (Quadcopter equipped with a non-metric camera was used to acquire images in Songjung beach which is located south-east Korea peninsula. The dynamics of beach topography, its geometric properties and estimates of eroded and deposited sand volumes were determined by combining elevation data with quarterly RTK-VRS measurements. To explore the new possibilities for assessment of coastal change we have developed a methodology for 3D analysis of coastal topography evolution based on existing high resolution elevation data combined with low coast, UAV and on-ground RTK-VRS surveys. DSMs were obtained by stereo-matching using Agisoft Photoscan. Using GCPs the vertical accuracy of the DSMs was found to be 10 cm or better. The resulting datasets were integrated in a local coordinates and the method proved to be a very useful fool for the detection of areas where coastal erosion occurs and for the quantification of beach change. The value of such analysis is illustrated by applications to coastal of South Korea sites that face significant management challenges.

  6. Basic Remote Sensing Investigations for Beach Reconnaissance.

    Science.gov (United States)

    Progress is reported on three tasks designed to develop remote sensing beach reconnaissance techniques applicable to the benthic, beach intertidal...and beach upland zones. Task 1 is designed to develop remote sensing indicators of important beach composition and physical parameters which will...ultimately prove useful in models to predict beach conditions. Task 2 is designed to develop remote sensing techniques for survey of bottom features in

  7. The Innovative Capacity of Firms

    Directory of Open Access Journals (Sweden)

    Neely Andy

    2014-11-01

    Full Text Available Innovation is widely accepted as a crucial competitive weapon in today's global market place. Yet the levels of innovation achieved by different firms, even within the same industry, can vary widely. The key question raised by this observation is why. Why are some firms more innovative than others? What are the factors that determine a firm's capacity to innovate and how can these be managed to enhance the firm's innovative potential? This paper sets out to address these and related issues. It reports the results of a study of competitiveness and innovation of firms in the East of England. In the paper it is argued that the innovative capacity of a firm is a function of the firm's culture, resources, competences and networks. Justification for this framework is provided by a review of the relevant literature and a series of case studies examining the capacity to innovate of a sample of firms in the East of England.

  8. Impaired motor speech performance in Huntington's disease.

    Science.gov (United States)

    Skodda, Sabine; Schlegel, Uwe; Hoffmann, Rainer; Saft, Carsten

    2014-04-01

    Dysarthria is a common symptom of Huntington's disease and has been reported, besides other features, to be characterized by alterations of speech rate and regularity. However, data on the specific pattern of motor speech impairment and their relationship to other motor and neuropsychological symptoms are sparse. Therefore, the aim of the present study was to describe and objectively analyse different speech parameters with special emphasis on the aspect of speech timing of connected speech and non-speech verbal utterances. 21 patients with manifest Huntington's disease and 21 age- and gender-matched healthy controls had to perform a reading task and several syllable repetition tasks. Computerized acoustic analysis of different variables for the measurement of speech rate and regularity generated a typical pattern of impaired motor speech performance with a reduction of speech rate, an increase of pauses and a marked disability to steadily repeat single syllables. Abnormalities of speech parameters were more pronounced in the subgroup of patients with Huntington's disease receiving antidopaminergic medication, but were also present in the drug-naïve patients. Speech rate related to connected speech and parameters of syllable repetition showed correlations to overall motor impairment, capacity of tapping in a quantitative motor assessment and some score of cognitive function. After these preliminary data, further investigations on patients in different stages of disease are warranted to survey if the analysis of speech and non-speech verbal utterances might be a helpful additional tool for the monitoring of functional disability in Huntington's disease.

  9. 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

  10. 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

  11. Wearable Sensors in Huntington Disease: A Pilot Study.

    Science.gov (United States)

    Andrzejewski, Kelly L; Dowling, Ariel V; Stamler, David; Felong, Timothy J; Harris, Denzil A; Wong, Cynthia; Cai, Hang; Reilmann, Ralf; Little, Max A; Gwin, Joseph T; Biglan, Kevin M; Dorsey, E Ray

    2016-06-18

    The Unified Huntington's Disease Rating Scale (UHDRS) is the principal means of assessing motor impairment in Huntington disease but is subjective and generally limited to in-clinic assessments. To evaluate the feasibility and ability of wearable sensors to measure motor impairment in individuals with Huntington disease in the clinic and at home. Participants with Huntington disease and controls were asked to wear five accelerometer-based sensors attached to the chest and each limb for standardized, in-clinic assessments and for one day at home. A second chest sensor was worn for six additional days at home. Gait measures were compared between controls, participants with Huntington disease, and participants with Huntington disease grouped by UHDRS total motor score using Cohen's d values. Fifteen individuals with Huntington disease and five controls completed the study. Sensor data were successfully captured from 18 of the 20 participants at home. In the clinic, the standard deviation of step time (time between consecutive steps) was increased in Huntington disease (p Huntington disease, and participants with Huntington disease grouped by motor impairment.

  12. Silencing Huntington's chorea: Is RNA Interference a Potential Cure?

    Directory of Open Access Journals (Sweden)

    Gerlinde A. Metz

    2006-01-01

    Full Text Available In 1872, George Huntington described Huntington's disease as characterized by motor, cognitive and psychiatric impairments. Huntington's disease is a dominant and autosomal mutation on chromosome 4 featuring the insertion of numerous CAG repeats. CAG codes for the amino acid, glutmanine that forms part of the Huntingtin protein (htt. Excess glutamine attachments make htt prone to accumulate in neurons. Three genes can be considered when developing therapies for Huntington's disease. They include targeting the symptoms of the disease, the progression of the disease and the cause of the disease. By using RNA interference (RNAi, the cause of the disease can be targeted. RNAi is a method that could potentially silence the formation of abnormal htt. This paper will discuss how RNAi could potentially cure Huntington's disease, by describing the genetic and proteinomic basis of Huntington's disease, the function of RNAi in Huntington's disease and the problems of benefits of RNAi. Preliminary work using RNAi in transgenic mice has shown a decrease in the behavioural expression of the mutant Huntington gene. There are several limitations associated with using RNAi as a gene therapy. For example, the effects of RNAi are short lived. A transposition system such as Sleeping Beauty can be used to increase the integration of the gene, however, for patients who currently have Huntington's disease, RNAi may potentially be used in combination with drugs or other treatments to target both symptoms and the underlying cause of Huntington's disease. This combination could eventually alleviate many painful symptoms associated with Huntington's disease and could even stop the progressive neurodegeneration of Huntington's disease. This review concludes that a substantial amount of new research is still necessary before RNAi is directly applicable to human patients with Huntington's disease.

  13. Belgian firms visiting CERN

    CERN Multimedia

    FP Department

    2009-01-01

    25 – 26 MAY 2009 09.00 to 17.00 Monday 25 May 09.00 to 17.00 Tuesday 26 May Individual interviews will take place in technicians’ offices. The firms will contact relevant users/technicians but any user wishing to make contact with a particular firm is welcome to use the contact details which are available from each departmental secretariat or from the GS Department web pages here. List of Companies:Automation Services and Consulting BVBA Burrick NV, (PLC) Cissoid DB Engineering Design, Drafting & Services BVBA Entelec Control Systems GILLAM-Fei S.A. HPC ICSENSE IWT – Enterprise Europe Flanders Jema SA Mecasoft SA SA Polmans Rapid-Torc Resarm Engineering Plastics SA Sentera Europa NV SLC BVBA Stocker Industrie SA Technord Tecnubel Winlock BVBA For further information please contact Caroline Laignel (GS-DI 73722) or Karine Robert (GS-SEM-LS 74407).

  14. Entrepreneurship and the Firm

    DEFF Research Database (Denmark)

    Foss, Nicolai Juul; Klein, Peter G.

    While characteristically "Austrian" themes such as entrepreneurship, economic calculation, tacit knowledge and the temporal structure of capital are clearly relevant to the business firm, Austrian economists have said relatively little about management, organization and strategy. This work features...... topics. These include knowledge management, authority and hierarchy, modularity, corporate restructuring, telecommunications regulation and competitive advantage. They clearly show how Austrian ideas can usefully engage, challenge and extend more mainstream perspectives on economic organization....

  15. Foreign Firms Partner Up

    Institute of Scientific and Technical Information of China (English)

    DAVID HENDRICKSON

    2006-01-01

    @@ There is an important strategic adjustment occurring on the part of foreign dairy companies seeking to capitalize on the China market. Rather than going it alone,with local partnerships little more than an entry mechanism,global giants such as Danone,Arla, and Fonterra are taking advantage of the country's more relaxed foreign investment rules by upping their stakes in established local firms and expanding the role they play in business and industrial operations.

  16. 1933 Long Beach, USA Images

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — 5 kilometers southwest of Newport Beach. Seriously affected area: 1,200 square kilometers. Damage: $40 million. Schools were among the buildings most severely...

  17. Human Health at the Beach

    Science.gov (United States)

    ... day at the beach are from swimming. Food poisoning from improperly refrigerated picnic lunches may also have ... simple steps to protect against overexposure to UV radiation as well as how to check the UV ...

  18. Horry County Beach Management Plan

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — Horry County has coordinated with DHEC OCRM to fully inventory, analyze, and documenteach of the ten required elements for an approvable local comprehensive beach...

  19. 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

  20. Landing Techniques in Beach Volleyball

    Science.gov (United States)

    Tilp, Markus; Rindler, Michael

    2013-01-01

    The aims of the present study were to establish a detailed and representative record of landing techniques (two-, left-, and right-footed landings) in professional beach volleyball and compare the data with those of indoor volleyball. Beach volleyball data was retrieved from videos taken at FIVB World Tour tournaments. Landing techniques were compared in the different beach and indoor volleyball skills serve, set, attack, and block with regard to sex, playing technique, and court position. Significant differences were observed between men and women in landings following block actions (χ2(2) = 18.19, p volleyball revealed overall differences both in men (χ2(2) = 161.4, p volleyball players land more often on both feet than indoor volleyball players. Besides the softer surface in beach volleyball, and therefore resulting lower loads, these results might be another reason for fewer injuries and overuse conditions compared to indoor volleyball. Key Points About 1/3 of all jumping actions in beach volleyball result in a landing on one foot. Especially following block situations men land on one foot more often than women. Landing techniques are related to different techniques and positions. Landings on one foot are less common in beach volleyball than indoor volleyball. This could be a reason for fewer injuries and overuse conditions. PMID:24149150

  1. Molecular diagnostic analysis for Huntington's disease: a prospective evaluation.

    OpenAIRE

    MacMillan, J C; Davies, P.; Harper, P S

    1995-01-01

    The availability of mutation analysis for the CAG repeat expansion associated with Huntington's disease has prompted clinicians in various specialties to request testing of samples from patients displaying clinical features that might be attributable to Huntington's disease. A series of 38 cases presenting with clinical features thought possibly to be due to Huntington's disease were analysed prospectively. In 53% of such cases presenting initially with chorea and 62.5% with psychiatric sympt...

  2. Normal CAG and CCG repeats in the Huntington`s disease genes of Parkinson`s disease patients

    Energy Technology Data Exchange (ETDEWEB)

    Rubinsztein, D.C.; Leggo, J.; Barton, D.E. [Cambridge Univ. (United Kingdom)] [and others

    1995-04-24

    The clinical features of Parkinson`s disease, particularly rigidity and bradykinesia and occasionally tremor, are seen in juvenile-onset Huntington`s disease. Therefore, the CAG and CCG repeats in the Huntington`s disease gene were investigated in 45 Parkinson`s disease patients and compared to 40 control individuals. All of the Parkinson`s disease chromosomes fell within the normal size ranges. In addition, the distributions of the two repeats in the Parkinson`s disease patients did not differ significantly from those of the control population. Therefore, abnormalities of these trinucleotide repeats in the Huntington`s disease gene are not likely to contribute to the pathogenesis of Parkinson`s disease. 12 refs., 2 figs.

  3. Measuring Firm Performance

    DEFF Research Database (Denmark)

    Assaf, A. George; Josiassen, Alexander; Gillen, David

    2014-01-01

    Set in the airport industry, this paper measures firm performance using both desirable and bad outputs (i.e. airport delays). We first estimate a model that does not include the bad outputs and then a model that includes bad outputs. The results show important differences in the efficiency...... and productivity results depending on whether bad outputs are or are not included in the model. The differences reflect the resource cost to society and the potential cost to an airport if such externalities were internalized. Finally, the paper provides measures of shadow prices for the bad output, in our case...

  4. The Identity of Ownership on Firm Internationalization

    DEFF Research Database (Denmark)

    Strandskov, Jesper; Madsen, Tage Koed; Pedersen, Bent

    2016-01-01

    The paper develops an integrative ownership-internationalization model that explores the influences of various owner types (i.e. investor-owned firms, family-owned firms, employee-owned firms, cooperative–owned firms, and state-owned firms) on firm internationalization. Based on a comparative...

  5. Danish firms visit CERN

    CERN Document Server

    FP Department

    2011-01-01

    30 – 31 JANUARY 2012 09:00 to 17:00 Monday 30 January 09:00 to 17:00 Tuesday 31 January Individual interviews will take place in technicians’ offices. The firms will contact relevant users/technicians but any user wishing to make contact with a particular firm is welcome to use the contact details available from the secretariat of their department or from the GS Department web page. List of Companies: · Axcon APS · BB Electronics A/S · B.Rustfrit Stal A/S · CIM Industrial Systems A/S · Danfysik A/S · Develco A/S · Eletronic A/S · GPV Group · Innoware A/S · JLI Vision A/S · NECAS A/S· NKT Cables A/S · Noliac A/S · Prodan A/S · Röttger’s Vaerktoj A/S · Vengcon APS For further information please contact Claudia Bruggmann Furlan  GS-IS-LS 73312 or Caroline Laignel GS-DI 73722.

  6. LANDING TECHNIQUES IN BEACH VOLLEYBALL

    Directory of Open Access Journals (Sweden)

    Markus Tilp

    2013-09-01

    Full Text Available The aims of the present study were to establish a detailed and representative record of landing techniques (two-, left-, and right-footed landings in professional beach volleyball and compare the data with those of indoor volleyball. Beach volleyball data was retrieved from videos taken at FIVB World Tour tournaments. Landing techniques were compared in the different beach and indoor volleyball skills serve, set, attack, and block with regard to sex, playing technique, and court position. Significant differences were observed between men and women in landings following block actions (χ²(2 = 18.19, p < 0.01 but not following serve, set, and attack actions. Following blocking, men landed more often on one foot than women. Further differences in landings following serve and attack with regard to playing technique and position were mainly observed in men. The comparison with landing techniques in indoor volleyball revealed overall differences both in men (χ²(2 = 161.4, p < 0.01 and women (χ²(2 = 84.91, p < 0.01. Beach volleyball players land more often on both feet than indoor volleyball players. Besides the softer surface in beach volleyball, and therefore resulting lower loads, these results might be another reason for fewer injuries and overuse conditions compared to indoor volleyball

  7. Large genetic animal models of Huntington's Disease.

    Science.gov (United States)

    Morton, A Jennifer; Howland, David S

    2013-01-01

    The dominant nature of the Huntington's disease gene mutation has allowed genetic models to be developed in multiple species, with the mutation causing an abnormal neurological phenotype in all animals in which it is expressed. Many different rodent models have been generated. The most widely used of these, the transgenic R6/2 mouse, carries the mutation in a fragment of the human huntingtin gene and has a rapidly progressive and fatal neurological phenotype with many relevant pathological changes. Nevertheless, their rapid decline has been frequently questioned in the context of a disease that takes years to manifest in humans, and strenuous efforts have been made to make rodent models that are genetically more 'relevant' to the human condition, including full length huntingtin gene transgenic and knock-in mice. While there is no doubt that we have learned, and continue to learn much from rodent models, their usefulness is limited by two species constraints. First, the brains of rodents differ significantly from humans in both their small size and their neuroanatomical organization. Second, rodents have much shorter lifespans than humans. Here, we review new approaches taken to these challenges in the development of models of Huntington's disease in large brained, long-lived animals. We discuss the need for such models, and how they might be used to fill specific niches in preclinical Huntington's disease research, particularly in testing gene-based therapeutics. We discuss the advantages and disadvantages of animals in which the prodromal period of disease extends over a long time span. We suggest that there is considerable 'value added' for large animal models in preclinical Huntington's disease research.

  8. Revisiting the neuropsychiatry of Huntington's disease

    Directory of Open Access Journals (Sweden)

    Antonio Lucio Teixeira

    Full Text Available ABSTRACT Huntington's disease (HD is an autosomal dominant neurodegenerative disease classified under the choreas. Besides motor symptoms, HD is marked by cognitive and behavioral symptoms, impacting patients' functional capacity. The progression of cognitive impairment and neuropsychiatric symptoms occur in parallel with neurodegeneration. The nature of these symptoms is very dynamic, and the major clinical challenges include executive dysfunction, apathy, depression and irritability. Herein, we provide a focused updated review on the cognitive and psychiatric features of HD.

  9. [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.

  10. Firm Search for External Knowledge

    DEFF Research Database (Denmark)

    Sofka, Wolfgang; Grimpe, Christoph

    2012-01-01

    The innovation performance of modern firms is increasingly determined by their ability to search and absorb external knowledge. However, after a certain threshold firms "oversearch" their environment and innovation performance declines. In this paper, we argue that prior literature has largely...... ignored the institutional context that provides or denies access to external knowledge at the country level. Combining institutional and knowledge search theory, we suggest that the market orientation of the institutional environment and the magnitude of institutional change influence when firms begin...... to experience the negative performance effects of oversearch. Based on a comprehensive sample of almost 8,000 firms from ten European countries, we find that institutions matter considerably for firms' search activity. Higher market orientation of institutions increases the effectiveness of firms' search...

  11. 78 FR 35596 - Special Local Regulation; Long Beach Regatta, Powerboat Race, Atlantic Ocean, Long Beach, NY

    Science.gov (United States)

    2013-06-13

    ... SECURITY Coast Guard 33 CFR Part 100 RIN 1625-AA08 Special Local Regulation; Long Beach Regatta, Powerboat Race, Atlantic Ocean, Long Beach, NY AGENCY: Coast Guard, DHS. ACTION: Notice of Proposed Rulemaking... Atlantic Ocean off Long Beach, NY during the Long Beach Regatta Powerboat Race scheduled for August...

  12. 33 CFR 100.736 - Annual Fort Myers Beach air show; Fort Myers Beach, FL.

    Science.gov (United States)

    2010-07-01

    ... 33 Navigation and Navigable Waters 1 2010-07-01 2010-07-01 false Annual Fort Myers Beach air show; Fort Myers Beach, FL. 100.736 Section 100.736 Navigation and Navigable Waters COAST GUARD, DEPARTMENT... Fort Myers Beach air show; Fort Myers Beach, FL. (a)(1) Regulated Area. The regulated area is formed...

  13. 77 FR 50019 - Safety Zone; Cocoa Beach Air Show, Atlantic Ocean, Cocoa Beach, FL

    Science.gov (United States)

    2012-08-20

    ... SECURITY Coast Guard 33 CFR Part 165 RIN 1625-AA00 Safety Zone; Cocoa Beach Air Show, Atlantic Ocean, Cocoa... establishing a temporary safety zone on the waters of the Atlantic Ocean located east of Cocoa Beach, Florida during the Cocoa Beach Air Show. The Cocoa Beach Air Show will include aircraft engaging in...

  14. Improving Oasis Beach: Creating a sustainable and attractive beach around hotel Oasis in Varadero Cuba

    NARCIS (Netherlands)

    Vrolijk, E.F.; Poelhekke, L.; Schlepers, M.H.; De Boer, G.G.

    2014-01-01

    In the North of Cuba, the Oasis beach area is situated. The beach suffers from structural erosion and earlier measures to deal with this have not succeeded. In this project, a solution is offered to reach two goals: foremost, a beach improvement to the Oasis beach sector and second, a halt to the s

  15. 76 FR 54703 - Safety Zone; Myrtle Beach Triathlon, Atlantic Intracoastal Waterway, Myrtle Beach, SC

    Science.gov (United States)

    2011-09-02

    ... SECURITY Coast Guard 33 CFR Part 165 RIN 1625-AA00 Safety Zone; Myrtle Beach Triathlon, Atlantic... Beach, South Carolina during the Myrtle Beach Triathlon. The Myrtle Beach Triathlon, which is comprised of a series of triathlon races, is scheduled to take place on Saturday, October 8, 2011 and...

  16. 76 FR 37700 - Safety Zone; Myrtle Beach Triathlon, Atlantic Intracoastal Waterway, Myrtle Beach, SC

    Science.gov (United States)

    2011-06-28

    ... SECURITY Coast Guard 33 CFR Part 165 RIN 1625-AA00 Safety Zone; Myrtle Beach Triathlon, Atlantic... Waterway in Myrtle Beach, South Carolina during the Myrtle Beach Triathlon. The Myrtle Beach Triathlon, which is comprised of a series of triathlon races, is scheduled to take place on Saturday, October...

  17. 77 FR 14321 - Safety Zone; Myrtle Beach Triathlon, Atlantic Intracoastal Waterway, Myrtle Beach, SC

    Science.gov (United States)

    2012-03-09

    ... SECURITY Coast Guard 33 CFR Part 165 RIN 1625-AA00 Safety Zone; Myrtle Beach Triathlon, Atlantic... Waterway in Myrtle Beach, South Carolina during the Myrtle Beach Triathlon. The Myrtle Beach Triathlon, which is comprised of a series of triathlon races, is scheduled to take place on Saturday, October...

  18. Lake Beach Monitoring Locations in Iowa

    Data.gov (United States)

    Iowa State University GIS Support and Research Facility — Monitored state lake beach locations in Iowa. The Watershed Monitoring & Assessment Section of the Iowa DNR takes regular water samples at these listed beaches...

  19. Santa Barbara Littoral Cell CRSMP Beaches 2009

    Data.gov (United States)

    California Department of Resources — Several criteria were used for beach selection. BEACON 's Coastal Regional Sediment Management Plan included all of the most popular beaches in the two counties...

  20. Amchitka beach surveys, 1978-1980

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — Surveys of 16 beaches on Amchitka Island began 28 October 1978 as part of Alaska Beached Bird Survey. The purpose of the surveys is to provide baseline data on...

  1. Santa Barbara Littoral Cell CRSMP Beaches 2009

    Data.gov (United States)

    California Department of Resources — Several criteria were used for beach selection. BEACON 's Coastal Regional Sediment Management Plan included all of the most popular beaches in the two counties...

  2. Heterotrophic bacterial populations in tropical sandy beaches

    Digital Repository Service at National Institute of Oceanography (India)

    Nair, S.; LokaBharathi, P.A.

    Distribution pattern of heterotrophic bacterial flora of three sandy beaches of the west coast of India was studied. The population in these beaches was microbiologically different. Population peaks of halotolerant and limnotolerant forms were...

  3. The financing of innovative firms

    OpenAIRE

    Hall, Bronwyn H.

    2009-01-01

    To what extent are new and/or innovative firms fundamentally different from established firms, and therefore require a different form of financing? The theoretical background for this proposition is presented, and the empirical evidence on its importance is reviewed. Owing to the intangible nature of their investment, asymmetric-information and moral-hazard, these firms are more likely to be financed by equity than debt and behave in some cases as though they are cash-constrained, especially ...

  4. TAXATION AND FINANCE CONSTRAINED FIRMS

    OpenAIRE

    Iris Claus

    2006-01-01

    This paper develops an open economy model to assess the long-run effects of taxation where firms are finance constrained. Finance constraints arise because of imperfect information between borrowers and lenders. Only borowers (firms) can costlessly observe actual returns from production. Imperfect information and finance constraints magnify the effects of taxation. A reduction (rise) in income taxation increases (lowers) firms' internal funds and their ability to assess external finance to ex...

  5. Inventory and Evaluation of Cultural Resources, Bolsa Chica Mesa and Huntington Beach Mesa, Orange County, California

    Science.gov (United States)

    1989-09-30

    1615 Relacl6n de la Jornada que Hizo el General Sevastian Vizcayno al Descubrimiento de las Californias el Aho de 1602 por Mandado del Segor...California Press, Berkeley. Prosch, R. R. 1978 Catalog of Fossil Hominids of North America . Fischer. New York and Stu’tf-ar t.7 Ricketts, E. F., J

  6. H10331: NOS Hydrographic Survey , Vicinity of Huntington Beach, Calfornia, 1990-02-26

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The National Oceanic and Atmospheric Administration (NOAA) has the statutory mandate to collect hydrographic data in support of nautical chart compilation for safe...

  7. Credit rationing and firm size

    Directory of Open Access Journals (Sweden)

    G. CALCAGNINI

    2013-10-01

    Full Text Available This paper examines the likelihood of credit rationing faced by firms of different size. Contrary to common thought, several recent contributions on this topic argue that, when rationing credit, size alone is not a sufficient condition for discriminating between firms. We show that this result can be predicted using a framework based on the Stiglitz-Weiss model. In particular, in an environment of asymmetric information, we highlight how the likelihood of credit rationing depends upon the shape of the distribution function of project returns, especially its asymmetry and Kurtosis. Our empirical results do not support the hypothesis that small firms face more credit rationing than larger firms.

  8. Experiences of Emerging Economy Firms

    DEFF Research Database (Denmark)

    Experiences of Emerging Economy Firms investigates the different elements of the experiences of emerging economy firms and sheds essential light on a large variety of aspects associated with their functioning in both home and host contexts. For example, firms must be able to overcome the liability...... of foreign and emerging issues when they expand their activities in various contexts, enter, exit, and re-enter overseas markets; they have to overcome institutional barriers, adapt the cultural challenges in foreign markets, undergo the impact of large multinational firms from developed economies...

  9. Experiences of Emerging Economy Firms

    DEFF Research Database (Denmark)

    Vissak, Tiia; Zhang, Xiaotian

    2015-01-01

    Experiences of Emerging Economy Firms investigates the different elements of the experiences of emerging economy firms and sheds essential light on a large variety of aspects associated with their functioning in both home and host contexts. For example, firms must be able to overcome the liability...... of foreign and emerging issues when they expand their activities in various contexts, enter, exit, and re-enter overseas markets; they have to overcome institutional barriers, adapt the cultural challenges in foreign markets, undergo the impact of large multinational firms from developed economies...

  10. Experiences of Emerging Economy Firms

    DEFF Research Database (Denmark)

    Vissak, Tiia; Zhang, Xiaotian

    2015-01-01

    of foreign and emerging issues when they expand their activities in various contexts, enter, exit, and re-enter overseas markets; they have to overcome institutional barriers, adapt the cultural challenges in foreign markets, undergo the impact of large multinational firms from developed economies......Experiences of Emerging Economy Firms investigates the different elements of the experiences of emerging economy firms and sheds essential light on a large variety of aspects associated with their functioning in both home and host contexts. For example, firms must be able to overcome the liability...

  11. Sequential Divestiture and Firm Asymmetry

    Directory of Open Access Journals (Sweden)

    Wen Zhou

    2013-01-01

    Full Text Available Simple Cournot models of divestiture tend to generate incentives to divest which are too strong, predicting that firms will break up into an infinite number of divisions resulting in perfect competition. This paper shows that if the order of divestitures is endogenized, firms will always choose sequential, and hence very limited, divestitures. Divestitures favor the larger firm and the follower in a sequential game. Divestitures in which the larger firm is the follower generate greater industry profit and social welfare, but a smaller consumer surplus.

  12. Belgian Firms Visit CERN

    CERN Document Server

    2001-01-01

    Fifteen Belgian firms visited CERN last 2 and 3 April to present their know-how. Industrial sectors ranging from precision machining to electrical engineering and electronics were represented. And for the first time, companies from the Flemish and Brussels regions of the country joined their Walloon compatriots, who have come to CERN before. The visit was organised by Mr J.-M. Warêgne, economic and commercial attaché at the Belgian permanent mission for the French-speaking region, Mr J. Van de Vondel, his opposite number for the Flemish region, and Mrs E. Solowianiuk, economic and commercial counsellor at the Belgian permanent mission for the Brussels-Capital region.

  13. Scientific Auditing Firms

    CERN Document Server

    Sarma, Gopal P

    2016-01-01

    The "crisis of reproducibility" has been a significant source of controversy, heated debate, and calls for reform to institutional science in recent years. As a long-term solution to address both the present crisis and future obstacles, I propose the creation of a new form of research organization whose purpose would be to conduct random audits of the scientific literature. I suggest that data analytics of a digitized scientific corpus may play a critical role in allowing broadly educated scientists to identify linchpin results to investigate in further detail across all disciplines. I argue that a simple "mock" trial run of a simplified auditing firm consisting of several researchers over a short time period would provide valuable insight into the feasibility of this proposal.

  14. Inside the Family Firm

    DEFF Research Database (Denmark)

    Bennedsen, Morten; Nielsen, Kasper; Pérez-González, Francisco

    2005-01-01

    This paper uses a unique dataset from Denmark to investigate (1) the role of family characteristics in corporate decision making, and (2) the consequences of these decisions on firm performance. We focus on the decision to appoint either a family or an external chief executive officer (CEO). We...... show that a departing CEO's family characteristics have a strong predictive power in explaining CEO succession decisions: family CEOs are more frequently selected the larger the size of the family, the higher the ratio of male children and when the departing CEOs had only had one spouse. We...... then analyze the impact of family successions on performance. We overcome endogeneity and omitted variables problems of previous papers in the literature by using the gender of a departing CEO's first-born child as an instrumental variable (IV) for family successions. This is a plausible IV as male first...

  15. Inside the Family Firm

    DEFF Research Database (Denmark)

    Bennedsen, Morten; Nielsen, Kasper; Pérez-González, Francisco

    2005-01-01

    This paper uses a unique dataset from Denmark to investigate (1) the role of family characteristics in corporate decision making, and (2) the consequences of these decisions on firm performance. We focus on the decision to appoint either a family or an external chief executive officer (CEO). We...... show that a departing CEO's family characteristics have a strong predictive power in explaining CEO succession decisions: family CEOs are more frequently selected the larger the size of the family, the higher the ratio of male children and when the departing CEOs had only had one spouse. We...... then analyze the impact of family successions on performance. We overcome endogeneity and omitted variables problems of previous papers in the literature by using the gender of a departing CEO's first-born child as an instrumental variable (IV) for family successions. This is a plausible IV as male first...

  16. Patenting activities and firm performance : Does firm size matter?

    NARCIS (Netherlands)

    Andries, P.; Faems, D.L.M.

    2013-01-01

    Whereas prior research has provided valuable insights into the willingness of small and medium-sized enterprises (SMEs) and large firms to engage in patenting, a comparison of the performance implications of patenting activities across small and large firms is still lacking. This gap is important be

  17. Differentiating Experts' Anticipatory Skills in Beach Volleyball

    Science.gov (United States)

    Canal-Bruland, Rouwen; Mooren, Merel; Savelsbergh, Geert J. P.

    2011-01-01

    In this study, we examined how perceptual-motor expertise and watching experience contribute to anticipating the outcome of opponents' attacking actions in beach volleyball. To this end, we invited 8 expert beach volleyball players, 8 expert coaches, 8 expert referees, and 8 control participants with no beach volleyball experience to watch videos…

  18. Differentiating Experts' Anticipatory Skills in Beach Volleyball

    Science.gov (United States)

    Canal-Bruland, Rouwen; Mooren, Merel; Savelsbergh, Geert J. P.

    2011-01-01

    In this study, we examined how perceptual-motor expertise and watching experience contribute to anticipating the outcome of opponents' attacking actions in beach volleyball. To this end, we invited 8 expert beach volleyball players, 8 expert coaches, 8 expert referees, and 8 control participants with no beach volleyball experience to watch videos…

  19. Altered cholesterol and fatty acid metabolism in Huntington disease.

    Science.gov (United States)

    Block, Robert C; Dorsey, E Ray; Beck, Christopher A; Brenna, J Thomas; Shoulson, Ira

    2010-01-01

    Huntington disease is an autosomal dominant neurodegenerative disorder characterized by behavioral abnormalities, cognitive decline, and involuntary movements that lead to a progressive decline in functional capacity, independence, and ultimately death. The pathophysiology of Huntington disease is linked to an expanded trinucleotide repeat of cytosine-adenine-guanine (CAG) in the IT-15 gene on chromosome 4. There is no disease-modifying treatment for Huntington disease, and novel pathophysiological insights and therapeutic strategies are needed. Lipids are vital to the health of the central nervous system, and research in animals and humans has revealed that cholesterol metabolism is disrupted in Huntington disease. This lipid dysregulation has been linked to specific actions of the mutant huntingtin on sterol regulatory element binding proteins. This results in lower cholesterol levels in affected areas of the brain with evidence that this depletion is pathologic. Huntington disease is also associated with a pattern of insulin resistance characterized by a catabolic state resulting in weight loss and a lower body mass index than individuals without Huntington disease. Insulin resistance appears to act as a metabolic stressor attending disease progression. The fish-derived omega-3 fatty acids, eicosapentaenoic acid and docosahexaenoic acid, have been examined in clinical trials of Huntington disease patients. Drugs that combat the dysregulated lipid milieu in Huntington disease may help treat this perplexing and catastrophic genetic disease.

  20. Arithmetic Word-Problem-Solving in Huntington's Disease

    Science.gov (United States)

    Allain, P.; Verny, C.; Aubin, G.; Pinon, K.; Bonneau, D.; Dubas, F.; Gall, D.L.

    2005-01-01

    The purpose of this study was to examine executive functioning in patients with Huntington's disease using an arithmetic word-problem-solving task including eight solvable problems of increasing complexity and four aberrant problems. Ten patients with Huntington's disease and 12 normal control subjects matched by age and education were tested.…

  1. Comprehension of Complex Discourse in Different Stages of Huntington's Disease

    Science.gov (United States)

    Saldert, Charlotta; Fors, Angelika; Stroberg, Sofia; Hartelius, Lena

    2010-01-01

    Background: Huntington's disease not only affects motor speech control, but also may have an impact on the ability to produce and understand language in communication. Aims: The ability to comprehend basic and complex discourse was investigated in three different stages of Huntington's disease. Methods & Procedures: In this experimental group…

  2. Firm Traits and Web Based Disclosures in Top Nigerian Firms

    Directory of Open Access Journals (Sweden)

    Bello Ayuba

    2016-02-01

    Full Text Available The use of the internet as a medium of dissemination of information to stakeholders is increasingly gaining grounds. This study extends existing literature on web disclosures by investigating the characteristics that predict the extent of web-based disclosures. In this study, corporate websites of top Nigerian firms are used as sources of data, while a regression analysis is employed to examine the extent of prediction. Results indicate that the firm size and industry type are significant determinants of web disclosures. However, other firm traits such as ownership dispersion and financial performance do not significantly explain the extent of internet disclosures. The study recommends that a regulatory template for corporate web disclosures be put in place by government regardless of the size or industry classification of the firm. This is with a view to considerably reduce agency conflicts arising from information asymmetry in publicly listed firms in Nigeria.

  3. Testing and Refining the Ohio Nowcast at Two Lake Erie Beaches-2008

    Science.gov (United States)

    Francy, Donna S.; Bertke, Erin E.; Darner, Robert A.

    2009-01-01

    The Ohio Nowcast has been providing real-time beach advisories to the public on the basis of predictive models since 2006. In support of the nowcast, data were collected during the recreational season of 2008 to validate and refine predictive models at two Lake Erie beaches. Predictive models yield data on the probability that the single-sample bathing-water standard for E. coli will be exceeded. Field personnel collected or compiled data on Escherichia coli (E. coli) concentrations as well as variables expected to affect these concentrations, including manual and automated measurements of turbidity, wave height, and water temperature; lake level; and radar and airport rainfall amounts. Two new variables were measured during 2008 - photosynthetically-active radiation at Huntington (Bay Village) and foreshore head at Edgewater (Cleveland). (The foreshore is a strip of land along a body of water between low and high water marks.) The performance of the nowcast was monitored during 2008. The Huntington nowcast yielded a greater percentage of correct responses (84.9 percent) than did the previous day's E. coli concentration (75.2 percent). In contrast, at Edgewater, the nowcast yielded a slightly higher percentage of correct responses (61.0 percent) as compared to the previous day's E. coli concentration (56.5 percent), but both percentages were relatively low. Lake levels in 2008 were significantly higher than levels in the data used to develop the Edgewater models (2004-7), confounding their abilities to provide correct responses. At Edgewater during 2008, the strongest relation (as measured by Pearson's correlation) was between E. coli concentrations and the difference in foreshore head over the past 24 hours (r=0.48), a variable not included in the models. At Huntington, photosynthetically-active radiation on the previous day showed a significant negative relation to E. coli concentrations (r=-0.33) during 2008. Refined models were developed for Huntington and

  4. Examination of Huntington's disease in a Chinese family.

    Science.gov (United States)

    Yu, Mingxia; Li, Xiaogai; Wu, Sanyun; Shen, Ji; Tu, Jiancheng

    2014-02-15

    We report brain imaging and genetic diagnosis in a family from Wuhan, China, with a history of Huntington's disease. Among 17 family members across three generations, four patients (II2, II6, III5, and III9) show typical Huntington's disease, involuntary dance-like movements. Magnetic resonance imaging found lateral ventricular atrophy in three members (II2, II6, and III5). Moreover, genetic analysis identified abnormally amplified CAG sequence repeats (> 40) in two members (III5 and III9). Among borderline cases, with clinical symptoms and brain imaging features of Huntington's disease, two cases were identified (II2 and II6), but shown by mutation analysis for CAG expansions in the important transcript 15 gene, to be non-Huntington's disease. Our findings suggest that clinical diagnosis of Huntington's disease requires a combination of clinical symptoms, radiological changes, and genetic diagnosis.

  5. Communication and Huntington's Disease: Qualitative Interviews and Focus Groups with Persons with Huntington's Disease, Family Members, and Carers

    Science.gov (United States)

    Hartelius, Lena; Jonsson, Maria; Rickeberg, Anneli; Laakso, Katja

    2010-01-01

    Background: As an effect of the cognitive, emotional and motor symptoms associated with Huntington's disease, communicative interaction is often dramatically changed. No study has previously included the subjective reports on this subject from individuals with Huntington's disease. Aims: To explore the qualitative aspects of how communication is…

  6. Does internationalization foster firm performance?

    NARCIS (Netherlands)

    van den Berg, Marcel

    2014-01-01

    The research in this dissertation concerns the impact of internationalization of business activities on several dimensions of firm performance. We show that the productivity ranking by trade status of Dutch manufacturing firms in increasing order of productivity is: non-traders, importers, exporters

  7. Competitive Advantage of Interconnected Firms

    DEFF Research Database (Denmark)

    Nell, Phillip Christopher; Andersson, Ulf

    We model the complex interplay between firm-level variables, notably capabilities and performance, and relationship-level variables: relationship-specific assets and network context. We test how the network context of individual exchange relationships as well as firm-level capabilities jointly af...

  8. The Internationalisation of Service Firms

    DEFF Research Database (Denmark)

    Blagoeva, Denitsa Hazarbassanova

    The question the thesis aims at resolving is: How do the value creation logics of firms impact their internationalisation? The overall aim of this PhD project is to explore and test an approach to understanding the internationalisation of service firms, based not on opposing them to manufacturing...

  9. Does internationalization foster firm performance?

    NARCIS (Netherlands)

    van den Berg, Marcel

    2014-01-01

    The research in this dissertation concerns the impact of internationalization of business activities on several dimensions of firm performance. We show that the productivity ranking by trade status of Dutch manufacturing firms in increasing order of productivity is: non-traders, importers, exporters

  10. Intra-Firm Adoption Decisions

    NARCIS (Netherlands)

    Y.M. van Everdingen (Yvonne); B. Wierenga (Berend)

    2001-01-01

    textabstractThe subject of this paper is intra-firm adoption decisions, a relatively unexplored research area in the marketing literature. In particular, we investigate which factors influence the intra-firm adoption decisions regarding the common European currency of the treasury, purchasing and sa

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

    NARCIS (Netherlands)

    Vis, Johanna Catharina

    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 sele

  12. Can beaches survive climate change?

    Science.gov (United States)

    Vitousek, Sean; Barnard, Patrick L.; Limber, Patrick

    2017-04-01

    Anthropogenic climate change is driving sea level rise, leading to numerous impacts on the coastal zone, such as increased coastal flooding, beach erosion, cliff failure, saltwater intrusion in aquifers, and groundwater inundation. Many beaches around the world are currently experiencing chronic erosion as a result of gradual, present-day rates of sea level rise (about 3 mm/year) and human-driven restrictions in sand supply (e.g., harbor dredging and river damming). Accelerated sea level rise threatens to worsen coastal erosion and challenge the very existence of natural beaches throughout the world. Understanding and predicting the rates of sea level rise and coastal erosion depends on integrating data on natural systems with computer simulations. Although many computer modeling approaches are available to simulate shoreline change, few are capable of making reliable long-term predictions needed for full adaption or to enhance resilience. Recent advancements have allowed convincing decadal to centennial-scale predictions of shoreline evolution. For example, along 500 km of the Southern California coast, a new model featuring data assimilation predicts that up to 67% of beaches may completely erode by 2100 without large-scale human interventions. In spite of recent advancements, coastal evolution models must continue to improve in their theoretical framework, quantification of accuracy and uncertainty, computational efficiency, predictive capability, and integration with observed data, in order to meet the scientific and engineering challenges produced by a changing climate.

  13. Can beaches survive climate change?

    Science.gov (United States)

    Vitousek, Sean; Barnard, Patrick L.; Limber, Patrick W.

    2017-01-01

    Anthropogenic climate change is driving sea level rise, leading to numerous impacts on the coastal zone, such as increased coastal flooding, beach erosion, cliff failure, saltwater intrusion in aquifers, and groundwater inundation. Many beaches around the world are currently experiencing chronic erosion as a result of gradual, present-day rates of sea level rise (about 3 mm/year) and human-driven restrictions in sand supply (e.g., harbor dredging and river damming). Accelerated sea level rise threatens to worsen coastal erosion and challenge the very existence of natural beaches throughout the world. Understanding and predicting the rates of sea level rise and coastal erosion depends on integrating data on natural systems with computer simulations. Although many computer modeling approaches are available to simulate shoreline change, few are capable of making reliable long-term predictions needed for full adaption or to enhance resilience. Recent advancements have allowed convincing decadal to centennial-scale predictions of shoreline evolution. For example, along 500 km of the Southern California coast, a new model featuring data assimilation predicts that up to 67% of beaches may completely erode by 2100 without large-scale human interventions. In spite of recent advancements, coastal evolution models must continue to improve in their theoretical framework, quantification of accuracy and uncertainty, computational efficiency, predictive capability, and integration with observed data, in order to meet the scientific and engineering challenges produced by a changing climate.

  14. Characterization of conservative somatic instability of the CAG repeat region in Huntington`s disease

    Energy Technology Data Exchange (ETDEWEB)

    Schaefer, F.V.; Calikoglu, A.S.; Whetsell, L.H. [H.A. Chapman Research Institute of Medical Genetics, Tulsa, OK (United States)

    1994-09-01

    Instability and enlargement of a CAG repeat region at the beginning of the huntingtin gene (IT-15) has been linked with Huntington`s disease. The CAG repeat size shows a highly significant correlation with age-of-onset of clinicial features in individuals with 40 or more repeats who have Huntington disease. The clinical status of nonsymptomatic individuals with 30 to 39 CAG repeats is considered ambiguous. In order to define more carefully the nature of the HD expansion instability, we examined patients in our HD population using a discriminating fluorescence-based PCR approach. The degree of somatic mutation increases with both earlier age of onset and the size of the inherited allele. A single prominent band one repeat larger than the index peak was typical in individuals with 40-41 CAG repeats. Three to four larger bands are typically discerned in individuals with 50 or more repeats. In an extreme example, an individual with approximately 95 repeats had at least 8 prominent bands. Plotting the degree of somatic mutation relative to the size of the HD allele shows somatic mutation activity increases with size. By this approach 40-60% of the alleles in a 40-41 CAG repeat HD loci is represented in the primary allele. In contrast, the primary allele represents a relatively minor proportion of the total alleles for expansions greater than 50 CAG repeats (10-20%). The limited range of somatic mutation suggest that the instability is restricted to very early stages of embryogenesis before tissue development diverges or that persistent somatic instability occurs at a slow rate. Therefore, the properties of somatic instability in Huntington`s disease have aspects that are both in common but also different from that found in other trinucleotide repeat expanding diseases such as myotonic muscular dystrophy and fragile X syndrome.

  15. Virtual Beach 3: user's guide

    Science.gov (United States)

    Cyterski, Mike; Brooks, Wesley; Galvin, Mike; Wolfe, Kurt; Carvin, Rebecca; Roddick, Tonia; Fienen, Mike; Corsi, Steve

    2014-01-01

    Virtual Beach version 3 (VB3) is a decision support tool that constructs site-specific statistical models to predict fecal indicator bacteria (FIB) concentrations at recreational beaches. VB3 is primarily designed for beach managers responsible for making decisions regarding beach closures or the issuance of swimming advisories due to pathogen contamination. However, researchers, scientists, engineers, and students interested in studying relationships between water quality indicators and ambient environmental conditions will find VB3 useful. VB3 reads input data from a text file or Excel document, assists the user in preparing the data for analysis, enables automated model selection using a wide array of possible model evaluation criteria, and provides predictions using a chosen model parameterized with new data. With an integrated mapping component to determine the geographic orientation of the beach, the software can automatically decompose wind/current/wave speed and magnitude information into along-shore and onshore/offshore components for use in subsequent analyses. Data can be examined using simple scatter plots to evaluate relationships between the response and independent variables (IVs). VB3 can produce interaction terms between the primary IVs, and it can also test an array of transformations to maximize the linearity of the relationship The software includes search routines for finding the "best" models from an array of possible choices. Automated censoring of statistical models with highly correlated IVs occurs during the selection process. Models can be constructed either using previously collected data or forecasted environmental information. VB3 has residual diagnostics for regression models, including automated outlier identification and removal using DFFITs or Cook's Distances.

  16. Foreign Entry and Heterogeneous Growth of Firms

    DEFF Research Database (Denmark)

    Deng, Paul Duo; Jefferson, Gary H.

    distance from foreign firms. Domestic firms with smaller technological distance from their foreign counterparts tend to experience faster productivity growth, while firms with larger technological distance tend to lag further behind. We test this hypothesis using a unique firm-level data of Chinese...... manufacturing. Our empirical results confirm that foreign entry indeed generates strong heterogeneous growth patterns among domestic firms....

  17. Altered Fractional Anisotropy in Early Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Silky Singh

    2013-02-01

    Full Text Available Huntington's disease (HD is a dominantly inherited neurodegenerative disease best known for chorea. The disorder includes numerous other clinical features including mood disorder, eye movement abnormalities, cognitive disturbance, pendular knee reflexes, motor impersistence, and postural instability. We describe a mild case of HD early in the disease course with depression and subtle neurological manifestations. In addition, we review MRI and diffusion tensor imaging features in this patient. The bicaudate ratio, a measure of caudate atrophy, was increased. Fractional anisotropy values of the bilateral caudate and putamen were increased, signifying neurodegeneration of these structures in HD.

  18. Huntingtin processing in pathogenesis of Huntington disease

    Institute of Scientific and Technical Information of China (English)

    Zhenghong QIN; Zhenlun GU

    2004-01-01

    Huntington's disease (HD) is caused by an expansion of the polyglutamine tract in the protein named huntingtin.The expansion of polyglutamine tract induces selective degeneration of striatal projection neurons and cortical pyramidal neurons. The bio-hallmark of HD is the formation of intranuclear inclusions and cytoplasmic aggregates in association with other cellular proteins in vulnerable neurons. Accumulation of N-terminal mutant huntingtin in HD brains is prominent. These pathological features are related to protein misfolding and impairments in protein processing and degradation in neurons. This review focused on the role of proteases in huntingtin cleavage and degradation and the contribution of altered processing of mutant huntingtin to HD pathogenesis.

  19. Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington's disease.

    Science.gov (United States)

    Paul, Bindu D; Sbodio, Juan I; Xu, Risheng; Vandiver, M Scott; Cha, Jiyoung Y; Snowman, Adele M; Snyder, Solomon H

    2014-05-01

    Huntington's disease is an autosomal dominant disease associated with a mutation in the gene encoding huntingtin (Htt) leading to expanded polyglutamine repeats of mutant Htt (mHtt) that elicit oxidative stress, neurotoxicity, and motor and behavioural changes. Huntington's disease is characterized by highly selective and profound damage to the corpus striatum, which regulates motor function. Striatal selectivity of Huntington's disease may reflect the striatally selective small G protein Rhes binding to mHtt and enhancing its neurotoxicity. Specific molecular mechanisms by which mHtt elicits neurodegeneration have been hard to determine. Here we show a major depletion of cystathionine γ-lyase (CSE), the biosynthetic enzyme for cysteine, in Huntington's disease tissues, which may mediate Huntington's disease pathophysiology. The defect occurs at the transcriptional level and seems to reflect influences of mHtt on specificity protein 1, a transcriptional activator for CSE. Consistent with the notion of loss of CSE as a pathogenic mechanism, supplementation with cysteine reverses abnormalities in cultures of Huntington's disease tissues and in intact mouse models of Huntington's disease, suggesting therapeutic potential.

  20. Extracting Firm Information from Administrative Records: The ASSD Firm Panel

    OpenAIRE

    Fink, Martina; Segalla, Esther; Weber, Andrea; Zulehner, Christine

    2010-01-01

    This paper demonstrates how firm information can be extracted from administrative social security records. We use the Austrian Social Security Database (ASSD) and derive firms from employer identifiers in the universe of private sector workers. To correctly pin down entry end exits we use a worker flow approach which follows clusters of workers as they move across administrative entities. This procedure enables us to define different types of entry and exit such as start-ups, spinoffs, closur...

  1. The Role of Firm Ownership on Internationalization

    DEFF Research Database (Denmark)

    Hobdari, Bersant; Gregoric, Aleksandra; Sinani, Evis

    2011-01-01

    This paper investigates how different types of owners influence the extent of firm internationalization, measured by the share of firm exports in total sales. The results of the analysis carried out using firm level data of Estonian and Slovenian firms, show that the firms under the control...... in internationalization process. Finally, high market share also leads to increased internationalization through exports as firms seek to expand in foreign markets after having dominated the domestic ones....

  2. Time-frequency analysis of beach bacteria variations and its implication for recreational water quality modeling.

    Science.gov (United States)

    Ge, Zhongfu; Frick, Walter E

    2009-02-15

    This paper exploited the potential of the wavelet analysis in resolving beach bacteria concentration and candidate explanatory variables across multiple time scales with temporal information preserved. The wavelet transform of E. coli concentration and its explanatory variables observed at Huntington Beach, Ohio in 2006 exhibited well-defined patterns of different time scales, phases, and durations, which cannot be clearly shown in conventional time-domain analyses. If linear regression modeling is to be used for the ease of implementation and interpretation,the wavelet-transformed regression model reveals that low model residual can be realized through matching major patterns and their phase angles between E. coli concentration and its explanatory variables. The property of pattern matching for linear regression models can be adopted as a criterion for choosing useful predictors, while phase matching further explains why intuitively good variables such as wave height and onshore wind speed were excluded from the optimal models by model selection processes in Frick et al. (Environ. Sci. Technol. 2008, 42,4818-4824). The phase angles defined by the wavelet analysis in the time-frequency domain can help identify the physical processes and interactions occurring between bacteria concentration and its explanatory variables. It was deduced, for this particular case, that wind events resulted in elevated E. coli concentration, wave height, and turbidity at the beach with a periodicity of 7-8 days. Wind events also brought about increased beach bacteria concentrations through large-scale current circulations in the lake with a period of 21 days. The time length for linear regression models with statistical robustness can also be deduced from the periods of the major patterns in bacteria concentration and explanatory variables, which explains and supplements the modeling efforts performed in (1).

  3. Unawareness of motor phenoconversion in Huntington disease.

    Science.gov (United States)

    McCusker, Elizabeth A; Gunn, David G; Epping, Eric A; Loy, Clement T; Radford, Kylie; Griffith, Jane; Mills, James A; Long, Jeffrey D; Paulsen, Jane S

    2013-09-24

    To determine whether Huntington disease (HD) mutation carriers have motor symptoms (complaints) when definite motor onset (motor phenoconversion) is diagnosed and document differences between the groups with and without unawareness of motor signs. We analyzed data from 550 HD mutation carriers participating in the multicenter PREDICT-HD Study followed through the HD prodrome. Data analysis included demographics, the Unified Huntington's Disease Rating Scale (UHDRS) and the Participant HD History of symptoms, self-report of progression, and cognitive, behavioral, and imaging measures. Unawareness was identified when no motor symptoms were self-reported but when definite motor HD was diagnosed. Of 38 (6.91%) with onset of motor HD, almost half (18/38 = 47.36%) had no motor symptoms despite signs of disease on the UHDRS motor rating and consistent with unawareness. A group with motor symptoms and signs was similar on a range of measures to the unaware group. Those with unawareness of HD signs reported less depression. Patients with symptoms had more striatal atrophy on imaging measures. Only half of the patients with newly diagnosed motor HD had motor symptoms. Unaware patients were less likely to be depressed. Self-report of symptoms may be inaccurate in HD at the earliest stage.

  4. 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.

  5. Web traffic and firm performance

    DEFF Research Database (Denmark)

    Farooq, Omar; Aguenaou, Samir

    2013-01-01

    Does the traffic generated by websites of firms signal anything to stock market participants? Does higher web-traffic translate into availability of more information and therefore lower agency problems? And if answers to above questions are in affirmative, does higher web-traffic traffic translate...... into better firm performance? This paper aims to answer these questions by documenting a positive relationship between the extent of web-traffic and firm performance in the MENA region during the 2010. We argue that higher web-traffic lowers the agency problems in firms by disseminating more information...... to stock market participants. Consequently, lower agency problems translate into better performance. Furthermore, we also show that agency reducing role of web-traffic is more pronounced in regimes where information environment is already bad. For example, our results show stronger impact of web-traffic...

  6. Government Control of Privatized Firms

    OpenAIRE

    Bortolotti, Bernardo; Faccio, Mara

    2007-01-01

    We study the change in government control of privatized firms in OECD countries. Results indicate that governments typically transfer ownership rights without relinquishing proportional control. Control is commonly retained by leveraging state investments through pyramids, dual-class shares, and golden shares. Indeed, at the end of 2000, after the largest privatization wave in history, governments retain control of 62.4% of privatized firms. In civil law countries, governments tend to retain ...

  7. Consumer Networks and Firm Reputation

    DEFF Research Database (Denmark)

    Huck, Steffen; Lünser, Gabriele K.; Tyran, Jean-Robert

    Arguing that consumers are the carriers of firms' reputations, we examine the role of consumer networks for trust in markets that suffer from moral hazard. When consumers are embedded in a network, they can exchange information with their neighbours about their private experiences with different...... sellers. We find that such information exchange fosters firms' incentives for reputation building and, thus, enhances trust and efficiency in markets. This efficiency-enhancing effect is already achieved with a rather low level of network density...

  8. PROFITABILITY AND SIZE OF FIRM.

    Science.gov (United States)

    Contents: The profit ratio as a measure of the relative ability of firms to expand; the profit dratio as a measure of efficiency; other uses of the... profit ratio; earlier studies using the profit ratio; valid conclusions of earlier studies; assets vs. net worth; methodology of this study...distribution of post-tax profit ratex; effect of taxes on the relative ability to expand; relative profitability of income firms; variability of profit rates

  9. Gross job flows and firms

    OpenAIRE

    Scott Schuh; Robert K. Triest

    1999-01-01

    This paper extends the work of Dunne, Roberts, and Samuelson [3] and Davis, Haltiwanger, and Schuh [2] on gross job flows among manufacturing plants. Gross job creation, destruction, and reallocation have been shown to be important in understanding the birth, growth, and death of plants, and the relation of plant life cycles to the business cycle. However, little is known about job flows between firms or how job flows among plants occur within firms (corporate restructuring). We use informati...

  10. Consumer Networks and Firm Reputation

    DEFF Research Database (Denmark)

    Huck, Steffen; Lünser, Gabriele K.; Tyran, Jean-Robert

    Arguing that consumers are the carriers of firms' reputations, we examine the role of consumer networks for trust in markets that suffer from moral hazard. When consumers are embedded in a network, they can exchange information with their neighbours about their private experiences with different ...... sellers. We find that such information exchange fosters firms' incentives for reputation building and, thus, enhances trust and efficiency in markets. This efficiency-enhancing effect is already achieved with a rather low level of network density...

  11. Association of Huntington's disease and schizophrenia-like psychosis in a Huntington's disease pedigree

    OpenAIRE

    Guimarães João; Xavier Miguel; Corrêa Bernardo

    2006-01-01

    Abstract Background Huntington's disease (HD) is a dominantly inherited, neurodegenerative disorder due to expansion of a polymorphic trinucleotide repeat in the short arm of chromosome 4. Clinical manifestations consist of a triad of choreic movements, cognitive decline and psychiatric syndromes starting in the fourth to fifth decade. Psychiatric manifestations vary and may precede motor and cognitive changes. Personality changes and depression occur most commonly. Paranoid schizophrenia-lik...

  12. 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.

  13. 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.

  14. 1H magnetic resonance spectroscopy in preclinical Huntington disease

    NARCIS (Netherlands)

    van Oostrom, Joost C. H.; Sijens, Paul E.; Roos, Raymund A. C.; Leenders, Klaus L.

    2007-01-01

    Huntington disease (HD) is a hereditary brain disease, causing progressive deterioration after a preclinical phase. The pathophysiology of early brain abnormalities around disease onset is largely unknown. Some preclinical mutation carriers (PMC) show structural or metabolic changes on brain imaging

  15. Juvenile Huntington's disease: a case report and literature review.

    Science.gov (United States)

    Reyes Molón, L; Yáñez Sáez, R M; López-Ibor Alcocer, M I

    2010-01-01

    Huntington's disease is the most frequent neurodegenerative disease with a prevalence of fewer than 10 cases per 10,000 inhabitants; the juvenile form is responsible for less than 10% of all cases. Huntington's disease belongs to the group known as "triad syndromes," which evolve with cognitive, motor and neuropsychiatric manifestations. Around 30% of patients debut with behavioral symptoms, which are a major challenge for management by patients, families, and caregivers. Huntington's disease (HD) is reviewed and a case of juvenile onset is reported in this article. The characteristics of juvenile-onset Huntington's disease (HD) differ from those of adult-onset HD, as chorea does not occur, although bradykinesia, dystonia, and signs of cerebellar disorder, such as rigidity, are present, frequently in association with convulsive episodes and psychotic manifestations.

  16. Genetics Home Reference: Huntington disease-like syndrome

    Science.gov (United States)

    ... 21915. Citation on PubMed Wild EJ, Tabrizi SJ. Huntington's disease phenocopy syndromes. Curr Opin Neurol. 2007 Dec;20(6):681-7. Review. Citation on PubMed Reviewed : August 2008 Published : August ...

  17. Episodic Memory Decline in Huntington's Disease, A Binding Deficit?

    NARCIS (Netherlands)

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

    2013-01-01

    Background: Huntington's disease (HD) is characterized by episodic memory deterioration. Objective: Our paper investigates the cognitive mechanisms that might underlie this decline. To this aim, we tested two executive hypotheses, the binding and the inhibition hypotheses. Methods: Fifteen HD patien

  18. Parcels and Land Ownership, Published in 2011, Huntington County Government.

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This Parcels and Land Ownership dataset as of 2011. The extent of these data is generally Huntington County, IN. This metadata was auto-generated through the Ramona...

  19. O paradigma de Huntington e o realismo político Huntington's paradigm and political realism

    Directory of Open Access Journals (Sweden)

    José R. Novaes Chiappin

    1994-12-01

    Full Text Available Examina-se a proposta de Huntington de um novo paradigma da política internacional (centrado na idéia de "civilizações" em substituição ao paradigma do realismo. Demonstra-se que se trata, na realidade, de um subparadigma do realismo e, portanto, a ele subordinado. Aplica-se isso à mudança da concepção estratégica de "contenção", que passa a aplicar-se às civilizações não-ocidentais e não mais ao expansionismo soviético.Huntington's proposal of a new paradigm for international politics (focused on the idea of "civilizations", meant to replace the paradigm of realism, is examined. It is shown that the proposed new paradigm should in fact be viewed as as sub-paradigm of the realist one. In particular, it is pointed out that Huntington's proposal, in a realist vein, draws on the idea of "containment", which is now directed (instead of its former target, the soviet expansionism to non-Western civilizations.

  20. Case study Piçarras Beach: Erosion and nourishment of a headland bay beach

    NARCIS (Netherlands)

    Van den Heuvel, S.; Hoekstra, R.; De Zeeuw, R.; Zoon, A.

    2008-01-01

    Master project report. Piçarras is one of the touristic beaches of Santa Catarina state in Brazil. Piçarras beach is a headland bay beach. In the bay irregular features like an island, rocky outcrops and shoals are present influencing wave propagation. In the south Piçarras is bounded by Piçarras ri

  1. 77 FR 27120 - Safety Zone; Virginia Beach Oceanfront Air Show, Atlantic Ocean, Virginia Beach, VA

    Science.gov (United States)

    2012-05-09

    ..., Virginia Beach, VA in the Federal Register (76 FR 13519). We received one comment on the proposed rule. No... SECURITY Coast Guard 33 CFR Part 165 RIN 1625-AA00 Safety Zone; Virginia Beach Oceanfront Air Show, Atlantic Ocean, Virginia Beach, VA AGENCY: Coast Guard, DHS. ACTION: Temporary final rule. SUMMARY:...

  2. Beach Nourishment Techniques. Report 3. Typical U.S. Beach Nourishment Projects Using Offshore Sand Deposits.

    Science.gov (United States)

    1981-05-01

    Cooperative Beach Erosion Control Project at Presque Isle Peninsula, Erie , Pennsylvania ," Buffalo, N. Y. U. S. Army Engineer District, Charleston. 1963 (Mar...104 Presque Isle , Pa .. .. ..................... 109 REFERENCES .. ............................ 115 2A BEACH NOURISHMENT...RIVER COUNTY, FL T PRESQUE ISLE . PA Figure 1. Beach fill projects location map ...../ ...... studies have been authorized, or which are publicly owned

  3. Studies on Thiobacilli spp. isolated from sandy beaches of Kerala

    Digital Repository Service at National Institute of Oceanography (India)

    Gore, P.S.; Raveendran, O.; Unnithan, R.V.

    Occurrence, isolation and oxidative activity of Thiobacilli spp. from some sandy beaches of Kerala are reported. These organisms were encountered in polluted beaches and were dominant during monsoon in all the beaches...

  4. Subtle changes among presymptomatic carriers of the Huntington's disease gene

    OpenAIRE

    S. Kirkwood; Siemers, E.; Hodes, M; Conneally, P; Christian, J.; Foroud, T

    2000-01-01

    OBJECTIVES—To compare the neurological and psychometric characteristics of presymptomatic gene carriers and non-gene carriers who are at risk for developing Huntington's disease so as to characterise early signs of disease and to identify markers of neurological function that could be used to assess the impact of experimental therapies on the progression of disease, even among those who are clinically presymptomatic.
METHODS—A sample of people at risk for Huntington's dis...

  5. Samuel Huntington, Clash of Civilizations: A Book Review

    OpenAIRE

    Yrd. Doç. Dr. Cengiz Kartýn

    2015-01-01

    Samuel Huntington's The Clash of Civilizations was written in 1993 by him. Study is a work containing the article and the responses to this article. Work is composed of two main parts. Makes the important point of this study is the process that began with the September 11 attacks by some strategists that is exactly the way towards a world where it is hidden in Huntington's fictionalized articulate.

  6. Linking SNPs to CAG repeat length in Huntington's disease patients.

    Science.gov (United States)

    Liu, Wanzhao; Kennington, Lori A; Rosas, H Diana; Hersch, Steven; Cha, Jang-Ho; Zamore, Phillip D; Aronin, Neil

    2008-11-01

    Allele-specific silencing using small interfering RNAs targeting heterozygous single-nucleotide polymorphisms (SNPs) is a promising therapy for human trinucleotide repeat diseases such as Huntington's disease. Linking SNP identities to the two HTT alleles, normal and disease-causing, is a prerequisite for allele-specific RNA interference. Here we describe a method, SNP linkage by circularization (SLiC), to identify linkage between CAG repeat length and nucleotide identity of heterozygous SNPs using Huntington's disease patient peripheral blood samples.

  7. Neuropathological diagnosis and CAG repeat expansion in Huntington's disease.

    OpenAIRE

    Xuereb, J H; MacMillan, J C; Snell, R; Davies, P.; Harper, P S

    1996-01-01

    OBJECTIVE--To correlate the degree of CAG repeat expansion with neuropathological findings in Huntington's disease. METHODS--The CAG repeat polymorphism was analysed in a large series of brain samples from 268 patients with a clinical diagnosis of Huntington's disease in which full neuropathological data was available. RESULTS--Analysis by polymerase chain reaction was successful in 63% of samples (169 of 268). Repeat expansions were detected in 152 of 153 (99%) samples with a neuropathologic...

  8. Levodopa responsive parkinsonism in an adult with Huntington's disease

    OpenAIRE

    Racette, B.; Perlmutter, J

    1998-01-01

    A patient is reported on with Huntington's disease who, as an adult, first developed severe parkinsonism with bradykinesia, rigidity, postural instability and festinating gait. His clinical signs were similar to those of the Westphal variant of Huntington's disease except that he also had resting tremor and a supranuclear gaze palsy. Magnetic resonance imaging showed caudate and putamen atrophy. Genetic analysis disclosed 49 triple CAG repeats in allele 1 and 17 in allele 2 ...

  9. 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...... eminences grown as free-floating roller-tube cultures can be successfully grafted in a rat Huntington model and that a clinical MR scanner offers a useful noninvasive tool for studying striatal graft development....

  10. [Olanzapine improves chorea in patients with Huntington's disease].

    Science.gov (United States)

    Jiménez-Jiménez, F J; de Toledo, M; Puertas, I; Barón, M; Zurdo, M; Barcenilla, B

    The main treatment for choreatic movements associated to Huntington s disease are the neuroleptic drugs, however, its use causes long term troubles. We describe two patients with a predominantly choreic Huntington s disease, who experience improvement of choreatic movements after introduction of olanzapine to their treatment, being this drug well tolerated. The improvement of chorea suggests that olanzapine has a dopaminergic D2 receptors blocking action.

  11. Merger incentives and the failing firm defense

    NARCIS (Netherlands)

    Bouckaert, J.M.C.; Kort, P.M.

    2014-01-01

    The merger incentives between profitable firms differ fundamentally from the incentives of a profitable firm to merge with a failing firm. We investigate these incentives under different modes of price competition and Cournot behavior. Our main finding is that firms strictly prefer exit of the faili

  12. Merger incentives and the failing firm defense

    NARCIS (Netherlands)

    Bouckaert, J.M.C.; Kort, P.M.

    2014-01-01

    The merger incentives between profitable firms differ fundamentally from the incentives of a profitable firm to merge with a failing firm. We investigate these incentives under different modes of price competition and Cournot behavior. Our main finding is that firms strictly prefer exit of the

  13. Why do Worker-Firm Matches Dissolve?

    NARCIS (Netherlands)

    Gielen, A. C.; van Ours, J.C.

    2006-01-01

    In a dynamic labor market worker-firm matches dissolve frequently causing workers to separate and firms to look for replacements.A separation may be initiated by the worker (a quit) or the firm (a layoff), or may result from a joint decision.A dissolution of a worker-firm match may be ineffcient if

  14. Family firm research – A review

    Directory of Open Access Journals (Sweden)

    Qiang Cheng

    2014-09-01

    Part I of the article discusses the fundaments of family firms: the prevalence of and the agency conflicts within family firms. Part II summarizes the findings of recent U.S. family firm studies. It reviews the evidence on the family firm premium (how, which, and when family firms are associated with a valuation premium, the manifestation of the agency conflict between majority and minority shareholders in family firms, earnings quality and corporate disclosure, and the determinants of family ownership and control. Part III discusses the prevalence and characteristics of Chinese family firms and reviews the findings of related studies. The article concludes with some suggestions for future research.

  15. The Internationalisation of Service Firms

    DEFF Research Database (Denmark)

    Blagoeva, Denitsa Hazarbassanova

    The question the thesis aims at resolving is: How do the value creation logics of firms impact their internationalisation? The overall aim of this PhD project is to explore and test an approach to understanding the internationalisation of service firms, based not on opposing them to manufacturing...... Monetary Fund, the World Bank and OECD, 2014). The attention to internationalisation of services has significantly increased and yet, many questions remain open (e.g. Pla-Barber and Ghauri, 2012).......The question the thesis aims at resolving is: How do the value creation logics of firms impact their internationalisation? The overall aim of this PhD project is to explore and test an approach to understanding the internationalisation of service firms, based not on opposing them to manufacturing...... ones, looking at descriptive service characteristics nor industry effects, but on the way they create value. Why and how are service firms different? and What are the drivers behind their internationalisation? are the questions motivating this PhD project. It explores if the value creation...

  16. Process Ambidexterity for Entrepreneurial Firms

    Directory of Open Access Journals (Sweden)

    Sonia D. Bot

    2012-04-01

    Full Text Available Technology-based entrepreneurial firms must effectively support both mainstream exploitation and new-stream exploration in order to remain competitive for the long term. The processes that support exploitation and exploration initiatives are different in terms of logistics, payoff horizons, and capabilities. Few firms are able to strike a balance between the two, where mainstream exploitation usually trumps new-stream exploration. The ultimate goal is for the firm to operate effectively in a repeatable, scalable, and systematic manner, rather than relying on good luck and hoping either to come up with the next innovation or for the product to function according to its requirements. This article builds on the author’s years of experience in building businesses and transforming medium and large-sized, entrepreneurial technology firms, leading large-scale breakthrough and sustained performance improvements by using and evolving Lean Six Sigma methodologies, and reviews of technology innovation management and entrepreneurship literature. This article provides a process-based perspective to understanding and addressing the issues on balancing mainstream exploitation and new-stream exploration in medium and large-sized entrepreneurial firms and extending it to startups. The resulting capability is known as process ambidexterity and requires disciplined, agile, and lean business management.

  17. A case report of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Anita Choudhary

    2017-09-01

    Full Text Available Huntington disease (HD is a progressive neurodegenerative disorder, characterized by autosomal dominant inheritance, movement disorder, dementia, and behavioural disturbances. It is caused by a mutation in IT15 gene on chromosome 4p16.3, which leads to unstable CAG trinucleotide repeat expansion. The onset of juvenile HD occurs before the 2nd decade of life and comprises approximately 10% of total HD patients. Juvenile HD differs in symptomatology and is usually transmitted from paternal side with genetic anticipation phenomenon. Magnetic resonance imaging (MRI of the brain shows specific changes of early affection of caudate nucleus and putamen. Multidisciplinary approach with symptomatic treatment of specific symptoms is the current available management. Gene editing and gene silencing treatment are under trial. Hereby, we introduce a case of an 8-year-old boy, who presented with typical symptoms of juvenile HD, positive family history with genetic anticipation phenomenon and characteristic MRI findings.

  18. Plants and phytochemicals for Huntington's disease.

    Science.gov (United States)

    Choudhary, Sunayna; Kumar, Puneet; Malik, Jai

    2013-07-01

    Huntington's disease (HD) is a neurodegenerative disorder characterized by progressive motor dysfunction, including chorea and dystonia, emotional disturbances, memory, and weight loss. The medium spiny neurons of striatum and cortex are mainly effected in HD. Various hypotheses, including molecular genetics, oxidative stress, excitotoxicity, metabolic dysfunction, and mitochondrial impairment have been proposed to explain the pathogenesis of neuronal dysfunction and cell death. Despite no treatment is available to fully stop the progression of the disease, there are treatments available to help control the chorea. The present review deals with brief pathophysiology of the disease, plants and phytochemicals that have shown beneficial effects against HD like symptoms. The literature for the current review was collected using various databases such as Science direct, Pubmed, Scopus, Sci-finder, Google Scholar, and Cochrane database with a defined search strategy.

  19. The choreography of neuroinflammation in Huntington's disease.

    Science.gov (United States)

    Crotti, Andrea; Glass, Christopher K

    2015-06-01

    Currently, the concept of 'neuroinflammation' includes inflammation associated with neurodegenerative diseases, in which there is little or no infiltration of blood-derived immune cells into the brain. The roles of brain-resident and peripheral immune cells in these inflammatory settings are poorly understood, and it is unclear whether neuroinflammation results from immune reaction to neuronal dysfunction/degeneration, and/or represents cell-autonomous phenotypes of dysfunctional immune cells. Here, we review recent studies examining these questions in the context of Huntington's disease (HD), where mutant Huntingtin (HTT) is expressed in both neurons and glia. Insights into the cellular and molecular mechanisms underlying neuroinflammation in HD may provide a better understanding of inflammation in more complex neurodegenerative disorders, and of the contribution of the neuroinflammatory component to neurodegenerative disease pathogenesis.

  20. Huntington disease: DNA analysis in brazilian population

    Directory of Open Access Journals (Sweden)

    RASKIN SALMO

    2000-01-01

    Full Text Available Huntington disease (HD is associated with expansions of a CAG trinucleotide repeat in the HD gene. Accurate measurement of a specific CAG repeat sequence in the HD gene in 92 Brazilian controls without HD, 44 Brazilian subjects with clinical findings suggestive of HD and 40 individuals from 6 putative HD families, showed a range from 7 to 33 repeats in normal subjects and 39 to 88 repeats in affected subjects. A trend between early age at onset of first symptoms and increasing number of repeats was seen. Major increase of repeat size through paternal inheritance than through maternal inheritance was observed. Data generated from this study may have significant implications for the etiology, knowledge of the incidence, diagnosis, prognosis, genetic counseling and treatment of HD Brazilian patients.

  1. Huntington's Disease: Pathogenic Mechanisms and Therapeutic Targets.

    Science.gov (United States)

    Wright, Dean J; Renoir, Thibault; Gray, Laura J; Hannan, Anthony J

    2017-01-01

    Huntington's disease (HD) is a tandem repeat disorder involving neurodegeneration and a complex combination of symptoms. These include psychiatric symptoms, cognitive deficits culminating in dementia, and the movement disorder epitomised by motor signs such as chorea. HD is caused by a CAG repeat expansion encoding an extended tract of the amino acid glutamine in the huntingtin protein. This polyglutamine expansion appears to induce a 'change of function', possibly a 'gain of function', in the huntingtin protein, which leads to various molecular and cellular cascades of pathogenesis. In the current review, we will briefly describe these broader aspects of HD pathogenesis, but will then focus on specific aspects where there are substantial bodies of experimental evidence, including oxidative stress, mitochondrial dysfunction, glutamatergic dysfunction and neuroinflammation. Furthermore, we will review recent preclinical therapeutic approaches targeting some of these pathogenic pathways, their clinical implications and future directions.

  2. Huntington's Disease: Relationship Between Phenotype and Genotype.

    Science.gov (United States)

    Sun, Yi-Min; Zhang, Yan-Bin; Wu, Zhi-Ying

    2017-01-01

    Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disease with the typical manifestations of involuntary movements, psychiatric and behavior disorders, and cognitive impairment. It is caused by the dynamic mutation in CAG triplet repeat number in exon 1 of huntingtin (HTT) gene. The symptoms of HD especially the age at onset are related to the genetic characteristics, both the CAG triplet repeat and the modified factors. Here, we reviewed the recent advancement on the genotype-phenotype relationship of HD, mainly focus on the characteristics of different expanded CAG repeat number, genetic modifiers, and CCG repeat number in the 3' end of CAG triplet repeat and their effects on the phenotype. We also reviewed the special forms of HD (juvenile HD, atypical onset HD, and homozygous HD) and their phenotype-genotype correlations. The review will aid clinicians to predict the onset age and disease course of HD, give the genetic counseling, and accelerate research into the HD mechanism.

  3. Huntington's Disease: Calcium Dyshomeostasis and Pathology Models.

    Science.gov (United States)

    Kolobkova, Y A; Vigont, V A; Shalygin, A V; Kaznacheyeva, E V

    2017-01-01

    Huntington's disease (HD) is a severe inherited neurodegenerative disorder characterized by motor dysfunction, cognitive decline, and mental impairment. At the molecular level, HD is caused by a mutation in the first exon of the gene encoding the huntingtin protein. The mutation results in an expanded polyglutamine tract at the N-terminus of the huntingtin protein, causing the neurodegenerative pathology. Calcium dyshomeostasis is believed to be one of the main causes of the disease, which underlies the great interest in the problem among experts in molecular physiology. Recent studies have focused on the development of animal and insect HD models, as well as patient-specific induced pluripotent stem cells (HD-iPSCs), to simulate the disease's progression. Despite a sesquicentennial history of HD studies, the issues of diagnosis and manifestation of the disease have remained topical. The present review addresses these issues.

  4. Cerebrospinal Fluid Biomarkers for Huntington's Disease.

    Science.gov (United States)

    Byrne, Lauren M; Wild, Edward J

    2016-01-01

    Cerebrospinal fluid (CSF) is enriched in brain-derived components and represents an accessible and appealing means of interrogating the CNS milieu to study neurodegenerative diseases and identify biomarkers to facilitate the development of novel therapeutics. Many such CSF biomarkers have been proposed for Huntington's disease (HD) but none has been validated for clinical trial use. Across many studies proposing dozens of biomarker candidates, there is a notable lack of statistical power, consistency, rigor and validation. Here we review proposed CSF biomarkers including neurotransmitters, transglutaminase activity, kynurenine pathway metabolites, oxidative stress markers, inflammatory markers, neuroendocrine markers, protein markers of neuronal death, proteomic approaches and mutant huntingtin protein itself. We reflect on the need for large-scale, standardized CSF collections with detailed phenotypic data to validate and qualify much-needed CSF biomarkers for clinical trial use in HD.

  5. 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.

  6. Neuronal Ca(2+) dyshomeostasis in Huntington disease.

    Science.gov (United States)

    Giacomello, Marta; Oliveros, Juan C; Naranjo, Jose R; Carafoli, Ernesto

    2013-01-01

    The expansion of the N-terminal poly-glutamine tract of the huntingtin (Htt) protein is responsible for Huntington disease (HD). A large number of studies have explored the neuronal phenotype of HD, but the molecular aethiology of the disease is still very poorly understood. This has hampered the development of an appropriate therapeutical strategy to at least alleviate its symptoms. In this short review, we have focused our attention on the alteration of a specific cellular mechanism common to all HD models, either genetic or induced by treatment with 3-NPA, i.e. the cellular dyshomeostasis of Ca(2+). We have highlighted the direct and indirect (i.e. transcriptionally mediated) effects of mutated Htt on the maintenance of the intracellular Ca(2+) balance, the correct modulation of which is fundamental to cell survival and the disturbance of which plays a key role in the death of the cell.

  7. Who benefits from firm-sponsored training?

    OpenAIRE

    Dostie, Benoit

    2015-01-01

    Workers participating in firm-sponsored training receive higher wages as a result. But given that firms pay the majority of costs for training, shouldn't they also benefit? Empirical evidence shows that this is in fact the case. Firm-sponsored training leads to higher productivity levels and increased innovation, both of which benefit the firm. Training can also be complementary to, and enhance, other types of firm investment, particularly in physical capital, such as information and communic...

  8. Earnings Management Priorities of Private Family Firms

    OpenAIRE

    Abdolmohammadi, Mohammad; Kvaal, Erlend; Langli, John Christian

    2010-01-01

    We compare earnings management priorities of private family and private non-family firms. Our study is made possible by the availability of a new and unique database on family relationships between CEOs, board members and owners of private Norwegian firms. We hypothesize and find that compared with private non-family firms, private family firms are likely to manage earnings downward. However, we also find that highly leveraged private family firms make more income increasing accounting choice...

  9. Knowledge management in the firm

    DEFF Research Database (Denmark)

    Rasmussen, Palle; Nielsen, Peter

    2011-01-01

    /methodology/approach – The dimensions of knowledge management are identified and related to learning, organizational configurations, human resources management and institutional environments in order to identify and percent the most important approaches to knowledge management and the development over time. Findings – Creating...... to innovation and illustrates how combinations of these might benefit firm performance. It also stresses the preconditions of employee involvement and participation to knowledge management and not least the importance of interaction with environmental resources. To improve performance firms should be aware...... the theoretical arguments should also be valid for the public sector. Originality/value – The paper relates knowledge management to theoretical approaches on learning, organization and innovation and shows the growing importance of these constructs in firm performance....

  10. How Firms Make Boundary Decisions

    DEFF Research Database (Denmark)

    Dobrajska, Magdalena; Billinger, Stephan; Becker, Markus

    2014-01-01

    We report findings from an analysis of 234 firm boundary decisions that a manufacturing firm has made during a 10 year period. Extensive interviews with all major decision makers located both at the headquarters and subsidiaries allow us to examine (a) who was involved in each boundary decision......, and (b) how the firm arrived at a particular transactional choice in each decision. We find that decision makers extensively adapt decision structures in order to effectively make governance mode choices. They adapt hierarchy span, i.e. the number of hierarchical levels involved, and expertise span, i.......e. the number of same-level decision makers with dissimilar knowledge basis. We observe that decision makers heavily rely on varying hierarchy and expertise span in order to improve the quality of the decision outcome. Central to the adaption of decision structures is that decision makers, over time...

  11. Firm Culture and Leadership as Firm Performance Predictors : a Resource-Based Perspective

    NARCIS (Netherlands)

    Wilderom, C.P.M.; van den Berg, P.

    2000-01-01

    In this study, we tested part of the resource-based view of the firm by examining two 'soft' resources, firm culture and top leadership, as predictors of 'hard' or bottom-line firm performance.Transformational top leadership was found to predict firm performance directly while the link between firm

  12. Firm Culture and Leadership as Firm Performance Predictors : a Resource-Based Perspective

    NARCIS (Netherlands)

    Wilderom, C.P.M.; van den Berg, P.

    2000-01-01

    In this study, we tested part of the resource-based view of the firm by examining two 'soft' resources, firm culture and top leadership, as predictors of 'hard' or bottom-line firm performance.Transformational top leadership was found to predict firm performance directly while the link between firm

  13. The Relationship between Offshoring Strategies and Firm Performance: Impact of innovation, absorptive capacity and firm size

    NARCIS (Netherlands)

    M.W. Roza-van Vuren (Marja)

    2011-01-01

    textabstractHow do offshoring strategies impact firm performance? And how are innovation, absorptive capacity and firm size influencing this relationship? This research investigates how firms of varying size, well-established firms and growing firms may profit from relocating business activities to

  14. A demographic approach to firm dynamics: formation of new firms and survival of old ones

    NARCIS (Netherlands)

    Nijkamp, P.; Geenhuizen, van M.

    1995-01-01

    This paper introduces a new approach to firm dynamics, named demography of firms. It is the study of demographic events in a population of firms. Thepaper argues that withina demographic approach, attention should be paid to a broad range of firm dynamics, including both new firm formation and survi

  15. The Relationship between Offshoring Strategies and Firm Performance: Impact of innovation, absorptive capacity and firm size

    NARCIS (Netherlands)

    M.W. Roza-van Vuren (Marja)

    2011-01-01

    textabstractHow do offshoring strategies impact firm performance? And how are innovation, absorptive capacity and firm size influencing this relationship? This research investigates how firms of varying size, well-established firms and growing firms may profit from relocating business activities to

  16. Five years of beach drainage survey on a macrotidal beach (Quend-Plage, northern France)

    Science.gov (United States)

    Bain, Olivier; Toulec, Renaud; Combaud, Anne; Villemagne, Guillaume; Barrier, Pascal

    2016-07-01

    A drainage system was installed in 2008 on the macrotidal beach of Quend-Plage, close to Abbeville (Somme, northern France), following a period of significant erosion of recreational areas. The "Direction départementale des territoires et de la mer" (French Coastal Department Authority) has requested a biannual survey in order to validate the beach drainage setup and its efficiency. This paper presents the methodology used for this survey, and the response of the coastal system to this soft engineering method for preventing erosion. These five years of drainage operation have strongly modified the morphology of the beach. Three main modifications occurred: (i) accretion of the upper beach and foredune, (ii) erosion of the lower and middle beach and (iii) a slight shift in directions of the beach bars and troughs. These morphological changes finally led to the stabilization of the beach.

  17. Firm-Specific Learning and the Nature of the Firm

    DEFF Research Database (Denmark)

    Knudsen, Thorbjørn

    2007-01-01

    in the absence of transaction costs related to the market mode of organisation. Comparing the relative importance of transaction cost and capabilities explanations for the existence of the firm, there is no a priori reason to assume the supremacy of one type of explanation over the other. We argue...... that a conjoint research program, encompassing both transaction costs and capabilities, must be developed and tested empirically. Cet article discute les limites d'une explication de l'existence de la firme fondée exclusivement sur les coûts de transaction en indiquant quelques facteurs additionnels susceptibles...... d'intervenir. Un modèle heuristique simple montre que les effets d'apprentissage spécifiques à la firme peuvent surmonter les coûts de surveillance des employés et expliquer l'existence de la firme même en l'absence de coûts de transaction liés au mode d'organisation de marché. Une comparaison de l'importance...

  18. Audit firm rotation, audit firm tenure and earnings conservatism

    NARCIS (Netherlands)

    Kramer, S.T.; Georgakopoulos, G.; Sotiropoulos, I.; Vasileiou, K.Z.

    2011-01-01

    This study aims to contribute to the debate around the possibility of mandating audit firm rotation. Specifically, it examines conservatism as an attribute of earnings quality, which has not attracted particular attention in the auditor rotation research. Applying regression analyses on a sample,

  19. Beach Nourishment and Artificial Surf Reef

    NARCIS (Netherlands)

    Klein, A.H.; De Menezes, J.T.; Sperb, R.S.; Siegle, E.; Fontura, R.; Van de Graaff, J.; Stive, M.J.F.; Van der Schrieck, G.L.M.; Verhagen, H.J.

    2004-01-01

    Balneário Camboriú is a very touristy city in southern Brazil, situated in the five kilometer wide Camboriú Bay. Its main tourist attraction is the beach, which is 5800 m long and rather narrow with a dry width of 10 to 20 m. The city is facing several problems regarding the beach that have a

  20. Beach Nourishment and Artificial Surf Reef

    NARCIS (Netherlands)

    Klein, A.H.; De Menezes, J.T.; Sperb, R.S.; Siegle, E.; Fontura, R.; Van de Graaff, J.; Stive, M.J.F.; Van der Schrieck, G.L.M.; Verhagen, H.J.

    2004-01-01

    Balneário Camboriú is a very touristy city in southern Brazil, situated in the five kilometer wide Camboriú Bay. Its main tourist attraction is the beach, which is 5800 m long and rather narrow with a dry width of 10 to 20 m. The city is facing several problems regarding the beach that have a negati

  1. Beach Nourishment and Artificial Surf Reef

    NARCIS (Netherlands)

    Klein, A.H.; De Menezes, J.T.; Sperb, R.S.; Siegle, E.; Fontura, R.; Van de Graaff, J.; Stive, M.J.F.; Van der Schrieck, G.L.M.; Verhagen, H.J.

    2004-01-01

    Balneário Camboriú is a very touristy city in southern Brazil, situated in the five kilometer wide Camboriú Bay. Its main tourist attraction is the beach, which is 5800 m long and rather narrow with a dry width of 10 to 20 m. The city is facing several problems regarding the beach that have a negati

  2. Stability and safety of Anjuna beach, Goa

    Digital Repository Service at National Institute of Oceanography (India)

    Swamy, G.N.; Varadachari, V.V.R.

    Studies on the Anjuna Beach, Goa, India, from March to December, 1975, show that it is fairly stable though it undergoes seasonal changes and a series of short-term cuts and fills. The beach appears to be quite safe as the longshore currents...

  3. Long Beach's Pivotal Turn around RTI

    Science.gov (United States)

    Elliott, Judy

    2008-01-01

    This article briefly describes the tiered approach to intervention adopted by the Long Beach Unified School District. Long Beach Unified School District is the state's third largest urban school district with more than 90,000 students, 84 percent of whom are minority and 68 percent of whom qualify for free and reduced price lunch, and where over…

  4. Social security and firm performance

    DEFF Research Database (Denmark)

    Torm, Nina; Lee, Sangheon

    2015-01-01

    The relationship between job quality and firm performance has been much debated with mixed evidence, and the evidence is particularly limited in the case of small and medium enterprises (SMEs) in emerging economies. This paper investigates how social security provision as a key determining factor......) in that the benefits may not realize in the immediate term. Thus, specific policy measures such as subsidising social insurance contributions for small firms during an initial period until the business becomes viable could potentially encourage active participation in mandatory schemes. A series of robustness check...

  5. Monitoring beach changes using GPS surveying techniques

    Science.gov (United States)

    Morton, Robert; Leach, Mark P.; Paine, Jeffrey G.; Cardoza, Michael A.

    1993-01-01

    A need exists for frequent and prompt updating of shoreline positions, rates of shoreline movement, and volumetric nearshore changes. To effectively monitor and predict these beach changes, accurate measurements of beach morphology incorporating both shore-parallel and shore-normal transects are required. Although it is possible to monitor beach dynamics using land-based surveying methods, it is generally not practical to collect data of sufficient density and resolution to satisfy a three-dimensional beach-change model of long segments of the coast. The challenge to coastal scientists is to devise new beach monitoring methods that address these needs and are rapid, reliable, relatively inexpensive, and maintain or improve measurement accuracy.

  6. Quantitative 7T phase imaging in premanifest Huntington disease.

    Science.gov (United States)

    Apple, A C; Possin, K L; Satris, G; Johnson, E; Lupo, J M; Jakary, A; Wong, K; Kelley, D A C; Kang, G A; Sha, S J; Kramer, J H; Geschwind, M D; Nelson, S J; Hess, C P

    2014-09-01

    In vivo MR imaging and postmortem neuropathologic studies have demonstrated elevated iron concentration and atrophy within the striatum of patients with Huntington disease, implicating neuronal loss and iron accumulation in the pathogenesis of this neurodegenerative disorder. We used 7T MR imaging to determine whether quantitative phase, a measurement that reflects both iron content and tissue microstructure, is altered in subjects with premanifest Huntington disease. Local field shift, calculated from 7T MR phase images, was quantified in 13 subjects with premanifest Huntington disease and 13 age- and sex-matched controls. All participants underwent 3T and 7T MR imaging, including volumetric T1 and 7T gradient recalled-echo sequences. Local field shift maps were created from 7T phase data and registered to caudate ROIs automatically parcellated from the 3T T1 images. Huntington disease-specific disease burden and neurocognitive and motor evaluations were also performed and compared with local field shift. Subjects with premanifest Huntington disease had smaller caudate volume and higher local field shift than controls. A significant correlation between these measurements was not detected, and prediction accuracy for disease state improved with inclusion of both variables. A positive correlation between local field shift and genetic disease burden was also found, and there was a trend toward significant correlations between local field shift and neurocognitive tests of working memory and executive function. Subjects with premanifest Huntington disease exhibit differences in 7T MR imaging phase within the caudate nuclei that correlate with genetic disease burden and trend with neurocognitive assessments. Ultra-high-field MR imaging of quantitative phase may be a useful approach for monitoring neurodegeneration in premanifest Huntington disease. © 2014 by American Journal of Neuroradiology.

  7. Dynamics of Shengjini beach (Albania)

    Science.gov (United States)

    Gashi, Ferim; Nikolli, Pal

    2015-04-01

    Dynamics of Shengjini beach (Albania) Pal Nikolli , Ferim GASHI Through archaeological and historical data, presentations of ancient topographic, cartographic materials (topographic maps obtained at different periods from 1870 to 1990), aerial photographs (2007), satellite images (2014) and direct measurements, paper defines and analyzes the position of the coastline of Shengjini beach (Lezha) from century XVI until today. The coastline of the Shengjini city (port) to Drin River estuary is oriented north-south direction and is approximately 10.5 km long. This part of the coast is sandy and sediment comes mainly from the River Drin and distributed by currents along the coast. In this paper are make provision for the position of the coastline in the future and analyzed the possibilities of human intervention in the coastal environment , etc. This work forms the basis for the issuance of necessary data required for various projections at the coastal environment Shëngjini. Results of this study will have a significant impact on state policies for integrated management of the coastal zone in the study and development of tourism. Key words: GIS, Remonte Sennsing, cartography, management of coastal zone, tourism, environment.

  8. Drivers of Discretionary Firm Donations in Brazil

    Directory of Open Access Journals (Sweden)

    Rodrigo Bandeira-de-Mello

    2008-10-01

    Full Text Available Discretionary firm donation is usually related to the stakeholder theory and corporate social performance. Although theoretical explanations for this social behavior are pervasive in related literature, empirical modeling remains underdeveloped. We developed an explanatory structural model of discretionary firm donation using firm and industry level indicators. Unlike previous research, we estimated the explanatory power of the construct we called stakeholder orientation. Our tentative model was tested on a Brazilian sample of 101 publicly traded donor firms, using data on firm donations to social projects and to political candidates in electoral campaigns. The main results suggest that discretionary donation seems to be a strategy for managing conflicting claims in highly stakeholder oriented firms; the characteristics of the firm are more important than industry effects in explaining firm donations; and large firms, showing slack resources, and with a less concentrated ownership structure tend to engage in discretionary donation more intensively.

  9. Placebo effect characteristics observed in a single, international, longitudinal study in Huntington's disease.

    NARCIS (Netherlands)

    Cubo, E.; Gonzalez, M.; Puerto, I. del; Yebenes, J.G. de; Arconada, O.F.; Gabriel y Galan, J.M.; Kremer, H.P.H.; Warrenburg, B.P.C. van de

    2012-01-01

    BACKGROUND: Classically, clinical trials are based on the placebo-control design. Our aim was to analyze the placebo effect in Huntington's disease. METHODS: Placebo data were obtained from an international, longitudinal, placebo-controlled trial for Huntington's disease (European Huntington's Disea

  10. 75 FR 33617 - Notice of Proposed Settlement Agreement and Opportunity for Public Comment: West Huntington Spill...

    Science.gov (United States)

    2010-06-14

    ... AGENCY Notice of Proposed Settlement Agreement and Opportunity for Public Comment: West Huntington Spill... United States Department of Justice on behalf of EPA, in connection with the West Huntington Spill Site, Huntington, West Virginia (``Site''). DATES: Written comments on the proposed settlement agreement must...

  11. Placebo effect characteristics observed in a single, international, longitudinal study in Huntington's disease.

    NARCIS (Netherlands)

    Cubo, E.; Gonzalez, M.; Puerto, I. del; Yebenes, J.G. de; Arconada, O.F.; Gabriel y Galan, J.M.; Kremer, H.P.H.; Warrenburg, B.P.C. van de

    2012-01-01

    BACKGROUND: Classically, clinical trials are based on the placebo-control design. Our aim was to analyze the placebo effect in Huntington's disease. METHODS: Placebo data were obtained from an international, longitudinal, placebo-controlled trial for Huntington's disease (European Huntington's

  12. 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...

  13. Why Do Distressed Firms Acquire?

    NARCIS (Netherlands)

    Q. Zhang (Quxian)

    2016-01-01

    textabstractAcquisitions made by distressed firms in recent years are economically important. This paper explores the rationale behind such acquisitions using a natural experiment. Exploiting a recent tax change which reduces debt restructuring costs for certain creditors and decreases bankruptcy

  14. Improving the firm's environmental conduct

    DEFF Research Database (Denmark)

    Knudsen, Thorbjørn; Koed Madsen, Tage

    2001-01-01

    lead to strategic advantage and, thus, economic gains at the firm level. In view of the great importance of this claim, the purpose of the present article was to apply resource-based insights in order to develop this reasoning further and provide an empirical test of three hypotheses related...

  15. Firm default and aggregate fluctuations

    NARCIS (Netherlands)

    Jacobson, Tor; Linde, Jesper; Roszbach, Kasper

    2013-01-01

    This paper studies the relationship between macroeconomic fluctuations and corporate defaults while conditioning on industry affiliation and an extensive set of firm-specific factors. By using a panel data set for virtually all incorporated Swedish businesses over 1990-2009, a period which includes

  16. Small firm transformation through IS

    NARCIS (Netherlands)

    Levy, Margi; Powell, Philip

    2008-01-01

    Globally, Small and Medium-sized Enterprises (SMEs) are encouraged, particularly by governments, to embrace c-business. Fully adopting e-business involves substantial change in firms, both internally and externally. However, there is little understanding of the mechanisms by which such business

  17. Small firm transformation through IS

    NARCIS (Netherlands)

    Levy, Margi; Powell, Philip

    2008-01-01

    Globally, Small and Medium-sized Enterprises (SMEs) are encouraged, particularly by governments, to embrace c-business. Fully adopting e-business involves substantial change in firms, both internally and externally. However, there is little understanding of the mechanisms by which such business tran

  18. The Identity of Ownership on Firm Internationalization

    DEFF Research Database (Denmark)

    Strandskov, Jesper; Madsen, Tage Koed; Pedersen, Bent

    2016-01-01

    The paper develops an integrative ownership-internationalization model that explores the influences of various owner types (i.e. investor-owned firms, family-owned firms, employee-owned firms, cooperative–owned firms, and state-owned firms) on firm internationalization. Based on a comparative...... analysis of the various owner types, we discuss how each owner group’s main objectives, risk behavior and provision of resources influence their internationalization strategies and decisions (i.e. scale, scope and speed decisions)....

  19. Persistent Patterns in Trading Firms' Actions

    Science.gov (United States)

    Zamani, Neda; Doyne Farmer, J.

    2007-03-01

    To understand the dynamics of price formation in financial markets, we take the approach of investigating the actions of market participants. We examine the impact of the participating firms on the price and find that different firms have different patterns of impact. Each firm can be representative of many traders with different trading habits and strategies but nevertheless we observe statistically significant differences in the firms' market impacts. We also investigate a method of clustering the firms based on a simplified conception of strategies. We find consistent clusters and patterns in firm strategies and show that these relations are statistically significant.

  20. Entrepreneurship and the Economics of the Firm

    DEFF Research Database (Denmark)

    Foss, Nicolai Juul

    The study of entrepreneurship and the study of economic organizing lack contact. In fact, the modern theory of the firm virtually ignores entrepreneurship, while the literature on entrepreneurship often sees little value in the economic theory of the firm. In contrast, we argue in this chapter...... that entrepreneurship theory and the theory of the firm can be usefully integrated, and that doing so would improve both bodies of theory. Adding the entrepreneur to the theory of the firm provides a dynamic view that the overly static analysis of firm organizing cannot support. Moreover, adding the firm to the study...

  1. Firm Based Trade Models and Turkish Economy

    Directory of Open Access Journals (Sweden)

    Nilüfer ARGIN

    2015-12-01

    Full Text Available Among all international trade models, only The Firm Based Trade Models explains firm’s action and behavior in the world trade. The Firm Based Trade Models focuses on the trade behavior of individual firms that actually make intra industry trade. Firm Based Trade Models can explain globalization process truly. These approaches include multinational cooperation, supply chain and outsourcing also. Our paper aims to explain and analyze Turkish export with Firm Based Trade Models’ context. We use UNCTAD data on exports by SITC Rev 3 categorization to explain total export and 255 products and calculate intensive-extensive margins of Turkish firms.

  2. 77 FR 276 - Combined Notice of Filings #1

    Science.gov (United States)

    2012-01-04

    ..., LLC, AES Redondo Beach, L.L.C., Condon Wind Power, LLC, AES Huntington Beach LLC, AES Armenia Mountain... Applicants: AES Huntington Beach, L.L.C. Description: AES Huntington Beach Tariff Filing to be effective...

  3. Were the 1952 Kern County and 1933 Long Beach, California, Earthquakes Induced?

    Science.gov (United States)

    Hough, S. E.; Tsai, V. C.; Walker, R. L., II; Page, M. T.; Aminzadeh, F.

    2016-12-01

    Several recent studies have presented evidence that significant induced earthquakes occurred in a number of regions during the 20th century related to either production or early wastewater injection. We consider whether the Mw6.4 Long Beach and Mw7.3 1952 Kern County earthquakes might have been induced by production in the Huntington Beach and Wheeler Ridge oil fields, respectively. The Long Beach earthquake occurred within 9 months of the start of directional drilling that first exploited offshore tideland reserves at depths of ≈1200 m; the well location was within ≈3 km of the event epicenter. The Kern County earthquake occurred 111 days following the first exploitation of deep Eocene production horizons within the Wheeler Ridge field at depths reaching 3 km, within ≈1 km of the White Wolf fault (WWF); the epicenter of this earthquake is poorly constrained but the preferred epicenter is within ≈7 km of the well. While production in the Wheeler Ridge field would have reduced pore pressure, likely inhibiting failure on the WWF assuming a Coulomb failure criteria, we present a model based on analytical solutions with model parameters constrained from detailed industry data, whereby direct pore pressure effects were blocked by a normal fault that created an impermeable barrier close to the WWF, allowing the normal stress change associated with production to dominate, thereby promoting failure by unclamping the fault. Our proposed triggering mechanism is consistent with the observation that significant earthquakes are only rarely induced by production in proximity to major faults. Our results also suggest that significant induced earthquakes in southern California during the early 20th century might have been associated with industry practices that are no longer employed (i.e., production without water re-injection). The occurrence of significant earthquakes during the earthquake 20th century therefore does not necessarily imply a high likely of induced

  4. Huntington's disease: from molecular pathogenesis to clinical treatment.

    Science.gov (United States)

    Ross, Christopher A; Tabrizi, Sarah J

    2011-01-01

    Huntington's disease is a progressive, fatal, neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene, which encodes an abnormally long polyglutamine repeat in the huntingtin protein. Huntington's disease has served as a model for the study of other more common neurodegenerative disorders, such as Alzheimer's disease and Parkinson's disease. These disorders all share features including: delayed onset; selective neuronal vulnerability, despite widespread expression of disease-related proteins during the whole lifetime; abnormal protein processing and aggregation; and cellular toxic effects involving both cell autonomous and cell-cell interaction mechanisms. Pathogenic pathways of Huntington's disease are beginning to be unravelled, offering targets for treatments. Additionally, predictive genetic testing and findings of neuroimaging studies show that, as in some other neurodegenerative disorders, neurodegeneration in affected individuals begins many years before onset of diagnosable signs and symptoms of Huntington's disease, and it is accompanied by subtle cognitive, motor, and psychiatric changes (so-called prodromal disease). Thus, Huntington's disease is also emerging as a model for strategies to develop therapeutic interventions, not only to slow progression of manifest disease but also to delay, or ideally prevent, its onset.

  5. Investigational agents for the management of Huntington's disease.

    Science.gov (United States)

    Müller, Thomas

    2017-02-01

    An inherited, chronic progressive, neurodegenerative disorder is Huntington's disease, characterized by motor, cognitive, and psychiatric symptoms. Predictive genetic testing allows earlier diagnosis and identification of gene carriers for Huntington's disease. These individuals are ideal candidates for testing of therapeutic interventions for disease modification. Areas covered: According to queries in Pubmed, Embase and clinical register databases, research and clinical studies emerge on symptomatic and neuroprotective therapies in Huntington's disease. This review discusses novel agents for symptomatic therapy and disease modification. They are currently in phase I and II of drug development Expert opinion: There are promising, safe and well tolerated compounds for amelioration of motor and neuropsychiatric symptoms, but their efficacy still needs to be proven in clinical trials. Deterioration of mutant huntingtin expression, antiapoptotic or cell death inhibition as disease modifying concepts was efficacious in models of Huntington's disease. However, the risk for clinical trial failures is high not only due to ineffectiveness of the tested agent. Negative study outcomes may also result from design misconceptions, underestimation of the heterogeneity of Huntington's disease, too short study durations and too small study cohorts.

  6. Beach Advisory and Closing Online Notification (BEACON) system

    Science.gov (United States)

    Beach Advisory and Closing Online Notification system (BEACON) is a colletion of state and local data reported to EPA about beach closings and advisories. BEACON is the public-facing query of the Program tracking, Beach Advisories, Water quality standards, and Nutrients database (PRAWN) which tracks beach closing and advisory information.

  7. Age and morphodynamics of a sandy beach fronted by a macrotidal mud flat along the west coast of Korea: a lateral headland bypass model for beach-dune formation

    Science.gov (United States)

    Chang, Tae Soo; Hong, Seok Hwi; Chun, Seung Soo; Choi, Jeong-Heon

    2016-11-01

    The Dasari beach-dune system fronted by an intertidal mud flat is a typical example of numerous small beaches found both in embayments and along the open macrotidal west coast of Korea. The beach is frequently exposed to energetic wave action at high tide in winter. Although this coastal dune-sandy beach-intertidal mud flat system has previously been described, its origin and morphodynamic behavior has to date not been firmly established. To clarify these issues, elevation profiles and surficial sediment samples were collected seasonally along five monitoring transects across the tidal flat. In addition, box-cores as well as vibro- and drill-cores were acquired along the middle transect. Optically stimulated luminescence (OSL) and 14C- AMS (accelerator mass spectrometry) dating methods were applied to determine the age of the tidal flat, the beach and the dune deposits. The results show that Dasari beach is topographically composed of two distinct morphological and sedimentological sectors, comprising a high-tide sandy beach that merges seaward into an extensive low-tide tidal flat composed of mud. The transition between the two sectors is marked by a sharp break in slope and change in internal sedimentary structures. At the boundary, the subtle shoreward fining trend in mean grain size on the intertidal flat switches to a pronounced shoreward coarsening trend. Near the transition, mixing between the beach sand and the mud is observed. Another striking feature is a seasonal rotation of the beach system centered on the middle sector, with the northern sector eroding in winter and accreting in summer, and the southern sector accreting in winter and eroding in summer. The spatial grain-size pattern reveals that the beach is fed from the neighboring beach in the north by lateral headland bypassing, rather than onshore transport across the tidal flat, the intermittent lateral supply of sand explaining the seasonal rotation of the beach. Stratigraphically, the beach

  8. Age and morphodynamics of a sandy beach fronted by a macrotidal mud flat along the west coast of Korea: a lateral headland bypass model for beach-dune formation

    Science.gov (United States)

    Chang, Tae Soo; Hong, Seok Hwi; Chun, Seung Soo; Choi, Jeong-Heon

    2017-08-01

    The Dasari beach-dune system fronted by an intertidal mud flat is a typical example of numerous small beaches found both in embayments and along the open macrotidal west coast of Korea. The beach is frequently exposed to energetic wave action at high tide in winter. Although this coastal dune-sandy beach-intertidal mud flat system has previously been described, its origin and morphodynamic behavior has to date not been firmly established. To clarify these issues, elevation profiles and surficial sediment samples were collected seasonally along five monitoring transects across the tidal flat. In addition, box-cores as well as vibro- and drill-cores were acquired along the middle transect. Optically stimulated luminescence (OSL) and 14C- AMS (accelerator mass spectrometry) dating methods were applied to determine the age of the tidal flat, the beach and the dune deposits. The results show that Dasari beach is topographically composed of two distinct morphological and sedimentological sectors, comprising a high-tide sandy beach that merges seaward into an extensive low-tide tidal flat composed of mud. The transition between the two sectors is marked by a sharp break in slope and change in internal sedimentary structures. At the boundary, the subtle shoreward fining trend in mean grain size on the intertidal flat switches to a pronounced shoreward coarsening trend. Near the transition, mixing between the beach sand and the mud is observed. Another striking feature is a seasonal rotation of the beach system centered on the middle sector, with the northern sector eroding in winter and accreting in summer, and the southern sector accreting in winter and eroding in summer. The spatial grain-size pattern reveals that the beach is fed from the neighboring beach in the north by lateral headland bypassing, rather than onshore transport across the tidal flat, the intermittent lateral supply of sand explaining the seasonal rotation of the beach. Stratigraphically, the beach

  9. Diversification and firm performance: A study of Indian manufacturing firms

    OpenAIRE

    Ravichandran, Archana; Bhaduri, Saumitra

    2015-01-01

    The advantages and disadvantages of diversification and its impact on productivity or performance of a firm have been debated upon by academics and business professionals all over, although views on the topic still differ widely. While popular views are that related diversification increases value and unrelated diversification decreases value, the results of research conducted on the effects of overall diversification (without distinguishing between related and unrelated diversification) on p...

  10. Psychological features of youths at a selection to beach volley-ball

    Directory of Open Access Journals (Sweden)

    Samoday V.

    2010-04-01

    Full Text Available The psychological features of young sportsmen are considered on the initial stage of sporting preparation. The dynamics of mental condition of youths is certain from beach volley-ball on the stage of sporting selection. 25 young sportsmen of groups of initial preparation took part in research. Middle age of young sportsmen made 12 years. On the indexes of self-appraisal of mental condition at swingeing majority of youths exposed a few the overextension of feel, activity and location of spirit. On the psychological indexes of concentration, the middle level of their development is set firmness and concentration of attention.

  11. Single sperm analysis of the trinucleotide repeat in the Huntington`s disease gene

    Energy Technology Data Exchange (ETDEWEB)

    Leeflang, E.P.; Zhang, L.; Hubert, R. [Univ. of Southern California, Los Angeles, CA (United States)] [and others

    1994-09-01

    Huntington`s disease (HD) is one of several genetic diseases caused by trinucleotide repeat expansion. The CAG repeat is very unstable, with size changes occurring in more than 80% of transmissions. The degree of instability of this repeat in the male germline can be determined by analysis of individual sperm cells. An easy and sensitive PCR assay has been developed to amplify this trinucleotide repeat region from single sperm using two rounds of PCR. As many as 90% of the single sperm show amplification for the HD repeat. The PCR product can be easily detected on an ethidium bromide-stained agarose gel. Single sperm samples from an HD patient with 18 and 49 repeats were studied. We observed size variations for the expanded alleles while the size of the normal allele in sperm is very consistent. We did not detect any significant bias in the amplification of normal alleles over the larger HD alleles. Our preliminary study supports the observation made by PCR of total sperm that instability of the HD trinucleotide repeat occurs in the germline. HD preimplantation diagnosis on single embryo blastomeres may also possible.

  12. 22 Years of predictive testing for Huntington's disease: the experience of the UK Huntington's Prediction Consortium.

    Science.gov (United States)

    Baig, Sheharyar S; Strong, Mark; Rosser, Elisabeth; Taverner, Nicola V; Glew, Ruth; Miedzybrodzka, Zosia; Clarke, Angus; Craufurd, David; Quarrell, Oliver W

    2016-10-01

    Huntington's disease (HD) is a progressive neurodegenerative condition. At-risk individuals have accessed predictive testing via direct mutation testing since 1993. The UK Huntington's Prediction Consortium has collected anonymised data on UK predictive tests, annually, from 1993 to 2014: 9407 predictive tests were performed across 23 UK centres. Where gender was recorded, 4077 participants were male (44.3%) and 5122 were female (55.7%). The median age of participants was 37 years. The most common reason for predictive testing was to reduce uncertainty (70.5%). Of the 8441 predictive tests on individuals at 50% prior risk, 4629 (54.8%) were reported as mutation negative and 3790 (44.9%) were mutation positive, with 22 (0.3%) in the database being uninterpretable. Using a prevalence figure of 12.3 × 10(-5), the cumulative uptake of predictive testing in the 50% at-risk UK population from 1994 to 2014 was estimated at 17.4% (95% CI: 16.9-18.0%). We present the largest study conducted on predictive testing in HD. Our findings indicate that the vast majority of individuals at risk of HD (>80%) have not undergone predictive testing. Future therapies in HD will likely target presymptomatic individuals; therefore, identifying the at-risk population whose gene status is unknown is of significant public health value.

  13. A study on the trinucleotide repeat associated with Huntington`s disease in the Chinese

    Energy Technology Data Exchange (ETDEWEB)

    Bing-wen Soong; Jih-tsuu Wang [Neurological Institute, Taipei (Taiwan, Province of China)

    1994-09-01

    Analysis of the polymorphic (CAG)n repeat in the hungingtin gene in the chinese confirmed the presence of an expanded repeat on all Huntington`s disease chromosomes. Measurement of the specific CAG repeat sequence in 34 HD chromosomes from 15 unrelated families and 190 control chromosomes from the Chinese population showed a range from 9 to 29 repeats in normal subjects and 40 to 58 in affected subjects. The size distributions of normal and affected alleles did not overlap. A clear correlation bewteen early onset of symptoms and very high repeat number was seen, but the spread of the age-at-onset in the major repeat range producing characteristic HD it too wide to be of diagnostic value. There was also variability in the transmitted repeat size for both sexes in the HD size range. Maternal HD alleles showed a moderate instability with a preponderance of size decrease, while paternal HD alleles had a tendency to increase in repeat size on transmission, the degree of which appeared proportional to the initial size.

  14. The Frequency of Huntington Disease and Huntington Disease-Like 2 in the South African Population.

    Science.gov (United States)

    Baine, Fiona K; Krause, Amanda; Greenberg, L Jacquie

    2016-01-01

    Huntington disease (HD) has most recently been estimated to affect between 10.6 and 13.7 per 100,000 individuals in European populations. However, prevalence is known to differ geographically. In South Africa, the only published estimates are from a survey performed in the 1970s, an era when the disease was believed to be rare or absent in black individuals and molecular confirmation was absent. The disease phenotype in South Africa is currently attributable to mutations in both the huntington and junctophilin-3 genes, which underlie the well-known HD and the rarer HD-like 2 (HDL2) respectively. This study aimed at providing improved minimum estimates of disease frequency in South Africa, based on molecular genetic testing data. A review of all testing records for HD and HDL2 over a 20-year period was undertaken. HDL2 is virtually indistinguishable on clinical features, thus necessitating its inclusion. Based on molecular diagnostic records, minimum estimates of disease frequency are: 5.1, 2.1 and 0.25 (per 100,000 individuals) for the white, mixed ancestry and black population groups respectively. Although ascertainment remains incomplete, these minimum estimates suggest that disease frequencies are significantly higher than those previously reported in South Africa. © 2016 S. Karger AG, Basel.

  15. Association of Huntington's disease and schizophrenia-like psychosis in a Huntington's disease pedigree

    Directory of Open Access Journals (Sweden)

    Guimarães João

    2006-02-01

    Full Text Available Abstract Background Huntington's disease (HD is a dominantly inherited, neurodegenerative disorder due to expansion of a polymorphic trinucleotide repeat in the short arm of chromosome 4. Clinical manifestations consist of a triad of choreic movements, cognitive decline and psychiatric syndromes starting in the fourth to fifth decade. Psychiatric manifestations vary and may precede motor and cognitive changes. Personality changes and depression occur most commonly. Paranoid schizophrenia-like symptoms occur in 6% to 25% of cases. Case report We describe a 55 year-old woman with an 8 yearlong history of behavioural changes, multi-thematic delusions and auditory hallucinations. History and mental state examination were suggestive of paranoid schizophrenia. Neurological examination revealed discrete, involuntary movements affecting her arms and trunk. Genotyping detected an expanded allele (43 trinucleotide repeats. A three-generation-long family history of chorea and schizophrenia-like psychosis was found. Conclusion HD-families have been reported in which schizophrenia-like syndromes emerged in all or most HD-affected members long before they developed extra-pyramidal or cognitive changes. This has been attributed to more than mere coincidence. We hypothesise that in these families the HD gene is transmitted along with a low load of small-effect "psychosis genes" which, in the presence of the severe cognitive changes of HD, manifest as a schizophrenia-like phenotype. Further research is needed in order to clarify the links between genetic loading and the emergence of psychotic symptoms in Huntington's disease.

  16. Heterogeneity in Firm Performance During Economic Crisis

    Directory of Open Access Journals (Sweden)

    Sergio Bruni

    2014-06-01

    Full Text Available What happens to firms during periods of deep economic crisis? Did different types of firms perform differently under the economic crisis? With the aid of a rich database and focusing on the literature regarding the growth of firms, this paper investigates the relative profitability performance of Italian firms during the current economic crisis, exploring those factors, which help certain firms to do relatively better even in the slowdown period. Some preliminary results show that the Italian firms that are relatively young in age, with relatively better current liquidity and more focused on domestic market have performed better than other firms. Furthermore, firms operating in high-tech and in highly concentrated sectors have enjoyed a better performance in this period.

  17. How do Economic Crises Impact Firm Boundaries?

    DEFF Research Database (Denmark)

    Foss, Kirsten

    2010-01-01

    How economic crises impact the boundaries of firms has been offered virtually no attention in the literature on the theory of the firm. I review the best-known theories of the firm and identify the variables that matter for the explanation of firm boundaries. I then examine how an economic crisis...... may impact these variables and change efficient firm boundaries. The various theories of the firm have difficulties explaining how firms efficiently adapt their boundaries to such prominent characteristics of economic crisis as declining demand and increased costs of external finance. However, all...... these theories stress uncertainty as an antecedent of firm organization, and as uncertainty is also an important characteristic of an economic crisis I examine how uncertainty is allowed to play out in the various theories in order to identify what predictions we can derive from the theory regarding changes...

  18. 78 FR 33969 - Special Local Regulations; Daytona Beach Grand Prix of the Sea, Atlantic Ocean; Daytona Beach, FL

    Science.gov (United States)

    2013-06-06

    ... SECURITY Coast Guard 33 CFR Part 100 RIN 1625-AA08 Special Local Regulations; Daytona Beach Grand Prix of the Sea, Atlantic Ocean; Daytona Beach, FL AGENCY: Coast Guard, DHS. ACTION: Temporary final rule... east of Daytona Beach, Florida, during the Daytona Beach Grand Prix of the Sea, a series of...

  19. 75 FR 24997 - FPL Energy Point Beach, LLC; Point Beach Nuclear Plant, Units 1 and 2; Environmental Assessment...

    Science.gov (United States)

    2010-05-06

    ... From the Federal Register Online via the Government Publishing Office NUCLEAR REGULATORY COMMISSION FPL Energy Point Beach, LLC; Point Beach Nuclear Plant, Units 1 and 2; Environmental Assessment... Energy Point Beach, LLC (the licensee), for operation of the Point Beach Nuclear Plant, Units 1 and 2...

  20. Morphology and composition of beach-cast Posidonia oceanica litter on beaches with different exposures

    Science.gov (United States)

    Simeone, Simone; De Falco, Giovanni

    2012-05-01

    Posidonia oceanica seagrass litter is commonly found along sandy shores in the Mediterranean region, forming structures called banquettes, which are often removed in order to allow the beach to be used for tourism. This paper evaluates the relationship between the morphology and composition of banquettes and beach exposure to dominant waves. A Real Time Kinematic Differential Global Positioning System was used to evaluate the variability of banquettes and beach morphology over a period of 1 year. Banquette samples, collected at two different levels of the beach profile (i.e. foreshore and backshore), were used to evaluate the contribution of leaves, rhizomes and sediments to the total weight. Banquettes showed a higher volume, thickness and cross-shore length on exposed beaches, whereas narrower litter deposits were found on the sheltered beach. On exposed beaches, banquettes were deposited in beach zones characterized by changes in elevation. These changes in elevation were mainly due to the deposition and erosion of sediments and secondly to the deposition and or erosion of leaf litter. On sheltered beaches, the variability in beach morphology was low and was restricted to areas where the banquettes were located. The leaf/sediment ratio changed along the cross-shore profile. On the backshore, banquettes were a mixture of sediments and leaves, whereas leaves were the main component on the foreshore, independently of the beach exposure. The processes which control the morphodynamics in the swash zone could explain the variability of banquette composition along the cross-shore profile. Finally, this study highlighted that Posidonia oceanica seagrass litter plays an important role in the geomorphology of the beachface and its removal can have a harmful impact on the beaches.

  1. Morphodynamics of a mesotidal rocky beach: Palmeras beach, Gorgona Island National Natural Park, Colombia

    Science.gov (United States)

    Gómez-García, A. M.; Bernal, G. R.; Osorio, A. F.; Botero, V.

    2014-10-01

    The response of a rocky beach to different possible combinations of hydrodynamic conditions (tides, waves, oceanic currents) has been little studied. In this work, the morphodynamic response to different hydrodynamic forcing is evaluated from sedimentological and geomorphological analysis in seasonal and medium term (19 years) scale in Palmeras beach, located in the southwest of Gorgona Island National Natural Park (NNP), a mesotidal rocky island on the Colombian Pacific continental shelf. Palmeras is an important nesting area of two types of marine turtles, with no anthropogenic stress. In the last years, coastal erosion has reduced the beach width, restricting the safe areas for nesting and conservation of these species. Until now, the sinks, sources, reservoirs, rates, and paths of sediments were unknown, as well as their hydrodynamic forcing. The beach seasonal variability, from October 2010 to August 2012, was analyzed based on biweekly or monthly measurements of five beach profiles distributed every 200 m along the 1.2 km of beach length. The main paths for sediment transport were defined from the modeling of wave currents with the SMC model (Coastal Modeling System), as well as the oceanic currents, simulated for the dry and wet seasons of 2011 using the ELCOM model (Estuary and Lake COmputer Model). Extreme morphologic variations over a time span of 19 years were analyzed with the Hsu and Evans beach static equilibrium parabolic model, from one wave diffraction point which dominates the general beach plan shape. The beach lost 672 m3/m during the measuring period, and erosional processes were intensified during the wet season. The beach trends responded directly to a wave mean energy flux change, resulting in an increase of up to 14 m in the width northward and loss of sediments in the beach southward. This study showed that to obtain the integral morphodynamic behavior of a rocky beach it is necessary to combine information of hydrodynamic, sedimentology

  2. Exclusion testing in pregnancy for Huntington's disease.

    Science.gov (United States)

    Tyler, A; Quarrell, O W; Lazarou, L P; Meredith, A L; Harper, P S

    1990-01-01

    The results of DNA analysis are presented for a series of 90 couples, with one partner at 50% risk for Huntington's disease (HD), who were referred for exclusion testing in pregnancy over a three year period. Thirty-seven couples were studied in detail. The aims of the study were to evaluate attitudes towards prenatal testing, before pregnancy and afterwards, and the effectiveness of our counseling and methods of organising the service. Problems which could arise in relation to presymptomatic testing are documented. It is concluded that exclusion testing is a valuable form of prediction for some couples, particularly where family structure does not permit prediction for the person at risk. The need for intensive counselling was highlighted by the difficulties experienced by many couples in understanding how the test worked. Particular ethical and organisational problems may arise which require careful consideration beforehand and some recommendations are made. The proportion of couples who will continue to request exclusion testing as pre-symptomatic testing becomes more widely applicable remains unknown. PMID:2145437

  3. Cell-based technologies for Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mônica Santoro Haddad

    Full Text Available ABSTRACT Huntington's disease (HD is a fatal genetic disorder, which causes the progressive breakdown of neurons in the human brain. HD deteriorates human physical and mental abilities over time and has no cure. Stem cell-based technologies are promising novel treatments, and in HD, they aim to replace lost neurons and/or to prevent neural cell death. Herein we discuss the use of human fetal tissue (hFT, neural stem cells (NSCs of hFT origin or embryonic stem cells (ESCs and induced pluripotent stem cells (IPSCs, in clinical and pre-clinical studies. The in vivo use of mesenchymal stem cells (MSCs, which are derived from non-neural tissues, will also be discussed. All these studies prove the potential of stem cells for transplantation therapy in HD, demonstrating cell grafting and the ability to differentiate into mature neurons, resulting in behavioral improvements. We claim that there are still many problems to overcome before these technologies become available for HD patient treatment, such as: a safety regarding the use of NSCs and pluripotent stem cells, which are potentially teratogenic; b safety regarding the transplantation procedure itself, which represents a risk and needs to be better studied; and finally c technical and ethical issues regarding cells of fetal and embryonic origin.

  4. Comprehension of prosody in Huntington's disease.

    Science.gov (United States)

    Speedie, L J; Brake, N; Folstein, S E; Bowers, D; Heilman, K M

    1990-07-01

    Patients with Huntington's Disease (HD) who were without dementia were compared to unilateral stroke patients and controls as previously reported in 1983, to discover if they had a prosodic defect. Subjects were presented tape-recorded speech filtered sentences and asked to indicate the tone of voice as happy, sad or angry (affective prosody), or as a question, command or statement (propositional prosody). HD patients were impaired in comprehension of both types of prosody compared to controls but were not different from stroke patients. A second study compared early HD patients with at-risk siblings and spouse controls on comprehension of affective and propositional prosody, discrimination of both types of prosody, rhythm discrimination and tonal memory (Seashore tests). HD patients were impaired in both comprehension and discrimination of all types of prosody. HD patients were less accurate than at-risk patients on the tonal memory task but not on the rhythm discrimination task. These findings suggest compromise in ability to understand the more subtle prosodic aspects of communication which may contribute to social impairment of HD patients very early in the course of the disease.

  5. Hypothalamic-endocrine aspects in Huntington's disease.

    Science.gov (United States)

    Petersén, Asa; Björkqvist, Maria

    2006-08-01

    Huntington's disease (HD) is a hereditary and fatal disorder caused by an expanded CAG triplet repeat in the HD gene, resulting in a mutant form of the protein huntingtin. Wild-type and mutant huntingtin are expressed in most tissues of the body but the normal function of huntingtin is not fully known. In HD, the neuropathology is characterized by intranuclear and cytoplasmic inclusions of huntingtin aggregates, and cell death primarily in striatum and cerebral cortex. However, hypothalamic atrophy occurs at early stages of HD with loss of orexin- and somatostatin-containing cell populations. Several symptoms of HD such as sleep disturbances, alterations in circadian rhythm, and weight loss may be due to hypothalamic dysfunction. Endocrine changes including increased cortisol levels, reduced testosterone levels and increased prevalence of diabetes are found in HD patients. In HD mice, alterations in the hypothalamic-pituitary-adrenal axis occurs as well as pancreatic beta-cell and adipocyte dysfunction. Increasing evidence points towards important pathology of the hypothalamus and the endocrine system in HD. As many neuroendocrine factors are secreted into the cerebrospinal fluid, blood and urine, it is possible that their levels may reflect the disease state in the central nervous system. Investigating neuroendocrine changes in HD opens up the possibility of finding biomarkers to evaluate future therapies for HD, as well as of identifying novel targets for therapeutic interventions.

  6. DNA instability in replicating Huntington's disease lymphoblasts

    Directory of Open Access Journals (Sweden)

    Frati Luigi

    2009-02-01

    Full Text Available Abstract Background The expanded CAG repeat in the Huntington's disease (HD gene may display tissue-specific variability (e.g. triplet mosaicism in repeat length, the longest mutations involving mitotic (germ and glial cells and postmitotic (neurons cells. What contributes to the triplet mutability underlying the development of HD nevertheless remains unknown. We investigated whether, besides the increased DNA instability documented in postmitotic neurons, possible environmental and genetic mechanisms, related to cell replication, may concur to determine CAG repeat mutability. To test this hypothesis we used, as a model, cultured HD patients' lymphoblasts with various CAG repeat lengths. Results Although most lymphoblastoid cell lines (88% showed little or no repeat instability even after six or more months culture, in lymphoblasts with large expansion repeats beyond 60 CAG repeats the mutation size and triplet mosaicism always increased during replication, implying that the repeat mutability for highly expanded mutations may quantitatively depend on the triplet expansion size. None of the investigated genetic factors, potentially acting in cis to the mutation, significantly influence the repeat changes. Finally, in our experiments certain drugs controlled triplet expansion in two prone-to-expand HD cell lines carrying large CAG mutations. Conclusion Our data support quantitative evidence that the inherited CAG length of expanded alleles has a major influence on somatic repeat variation. The longest triplet expansions show wide somatic variations and may offer a mechanistic model to study triplet drug-controlled instability and genetic factors influencing it.

  7. Genetic diagnosis of Huntington's disease: cases report

    Institute of Scientific and Technical Information of China (English)

    Liao Ting-ting; Wu Wei; Wan Qi; Cui Yu-gui; Liu Jia-yin

    2011-01-01

    Objective:To assess the efficiency of the PCR combined DNA sequencing to ascertain CAG repeat size of Huntington's disease(HD)gene as for gene diagnosis of HD.Method:Three patients with HD were diagnosed genetically with the technology of polymerase chain reaction (PCR) and polyacrylamide gel electrophoresis by assessing the CAG repeat size of HD gene.DNA sequencing then was used as verification test for HD gene.Results:Nine members of three nuclear families were included in this study,three patients were HD proband.In those families,CAG repeats of all spouse of propositus were in normal range.CAG repeats of all propositus and their descendants with the normal allele were in normal range,while CAG copy number of the other mobigenous allele was obviously abnormal.Conclusion:PCR combined DNA sequencing can be used to effectively ascertain CAG repeat of HD gene.CAG-repeat expansion mutations were accounted for 99% of HD cases,so HD can be accurately diagnosed by this method.

  8. Genetic Testing for Huntington's Disease in Parkinsonism.

    Science.gov (United States)

    Rahman, M S; Nagai, Y; Popiel, H A; Fujikake, N; Okamoto, Y; Ahmed, M U; Islam, M A; Islam, M T; Ahmed, S; Rahman, K M; Uddin, M J; Dey, S K; Ahmed, Q; Hossain, M A; Jahan, N; Toda, T

    2010-10-01

    The study was conducted to find out Huntington's disease (HD) by genetic analysis from those presenting with parkinsonism in the Neurology department of Mymensingh Medical College & Hospital. A sample of about 5ml blood was collected by veni puncture in EDTA tube with informed consent from 9 patients & 7 healthy individuals after approval of the institutional ethics committee for genetic study. The neurological disorder along with a complete history and physical findings were recorded in a prescribed questionnaire by the neurologists of Mymensingh Medical College & Hospital. Extraction of genomic DNA from the venous blood using FlexiGene DNA kit (Qiagen, Japan) was performed in Faculty of Veterinary Science, Bangladesh Agricultural University, Mymensingh, Bangladesh. The extracted DNA was stored and accumulated and then these DNA were sent to Division of Clinical Genetics, Department of Medical Genetics, Osaka University Medical School, Suita, Osaka 565 0871, Japan for PCR and further analysis. PCR amplification of the CAG repeat in the 1T15 gene was performed with primers HD1 and HD3. HD PCR products revealed the DNA product of about 110bp (no. of CAG repeats=21) to 150bp (no. of CAG repeats=34) in both healthy individual and suspected PD patient DNA.

  9. Pridopidine for the treatment of Huntington's disease.

    Science.gov (United States)

    Shannon, Kathleen M

    2016-01-01

    Huntington's disease is a rare dominantly-inherited neurodegenerative disease with motor, cognitive and behavioral manifestations. It results from an expanded unstable trinucleotide repeat in the coding region of the huntingtin gene. Treatment is symptomatic, but a poor evidence baseguides selection of therapeutic agents. Non-choreic derangements in voluntary movement contribute to overall motor disability and are poorly addressed by current therapies. Pridopidine is a novel agent in the dopidine class believed to have 'state dependent' effects at dopamine receptors, thus show promise in the treatment of these disorders of voluntary movement. This review discusses the pharmacokinetics and pharmacodynamics of pridopidine and reviews clinical trials supporting development of the drug for HD. This information was culled from literature searches for dopidines, pridopidine, and HD experimental therapeutics in PubMed and at http://www.clinicaltrials.org . There is a compelling need to discover new treatments for motor disability in HD, particularly for non-choreic motor symptoms. While pridopidine failed to achieve its primary efficacy outcomes in 2 large trials, reproducible effects on secondary motor outcomes have fueled an ongoing trial studying higher doses and more focused clinical endpoints. This and phase III trials will define define the utility of pridopidine for HD.

  10. Lessons Learned from the Transgenic Huntington's Disease Rats

    Directory of Open Access Journals (Sweden)

    Rinske Vlamings

    2012-01-01

    Full Text Available Huntington's disease (HD is a fatal inherited disorder leading to selective neurodegeneration and neuropsychiatric symptoms. Currently, there is no treatment to slow down or to stop the disease. There is also no therapy to effectively reduce the symptoms. In the investigation of novel therapies, different animal models of Huntington's disease, varying from insects to nonhuman primates, have been created and used. Few years ago, the first transgenic rat model of HD, carrying a truncated huntingtin cDNA fragment with 51 CAG repeats under control of the native rat huntingtin promoter, was introduced. We have been using this animal model in our research and review here our experience with the behavioural, neurophysiological, and histopathological phenotype of the transgenic Huntington's disease rats with relevant literature.

  11. Variation within the Huntington's disease gene influences normal brain structure.

    Directory of Open Access Journals (Sweden)

    Mark Mühlau

    Full Text Available Genetics of the variability of normal and diseased brain structure largely remains to be elucidated. Expansions of certain trinucleotide repeats cause neurodegenerative disorders of which Huntington's disease constitutes the most common example. Here, we test the hypothesis that variation within the IT15 gene on chromosome 4, whose expansion causes Huntington's disease, influences normal human brain structure. In 278 normal subjects, we determined CAG repeat length within the IT15 gene on chromosome 4 and analyzed high-resolution T1-weighted magnetic resonance images by the use of voxel-based morphometry. We found an increase of GM with increasing long CAG repeat and its interaction with age within the pallidum, which is involved in Huntington's disease. Our study demonstrates that a certain trinucleotide repeat influences normal brain structure in humans. This result may have important implications for the understanding of both the healthy and diseased brain.

  12. Long-term effects of beach nourishment on intertidal invertebrates

    OpenAIRE

    Wooldridge, Tyler Brock

    2015-01-01

    Although beach nourishment is an increasingly popular means to remediate coastal erosion, no consensus exists regarding how long nourishment affects sandy beach intertidal invertebrates, key components of sandy beach ecosystems. We monitored the intertidal invertebrate community for fifteen months following a nourishment project at eight beaches across San Diego County. Each beach was split into nourished and control sections. Nearly all taxa showed major declines in abundance immediately fol...

  13. Human Capital and Wages in Exporting Firms

    DEFF Research Database (Denmark)

    Munch, Jakob Roland; Skaksen, Jan Rose

    2006-01-01

    This paper studies the link between a firms education level, export performance and wages of its workers. We argue that firms may escape intence competition in international markets by using high skilled workers to differentiate their products. This story is consistent with our empirical results....... an export wage premium, but it accrues to workers in firms with high skill intensities.Keywords: Exports, Wages, Human Capital, Rent Sharing, Matched Worker-Firm DataJEL Classification: J30, F10, I20...

  14. Does Labour Diversity affect Firm Productivity?

    DEFF Research Database (Denmark)

    Parrotta, Pierpaolo; Pozzoli, Dario; Pytlikova, Mariola

    that labor diversity in education significantly enhances a firm's value added. Conversely, diversity in ethnicity and demographics induces negative effects on firm productivity. Hence, the negative effects, coming from communication and integration costs connected to a more culturally and demographically......Using a matched employer-employee dataset, we analyze how workforce diversity in cultural background, education and demographic characteristics affects productivity of firms in Denmark. Implementing a structural estimation of the firms' production function (Ackerberg et al., 2006) we find...

  15. Socioemotional wealth preservation in family firms

    Directory of Open Access Journals (Sweden)

    Matias Kalm

    Full Text Available Abstract In this article, we review literature on socioemotional wealth. We explain how the concept of socioemotional wealth builds on previous family firm research showing that family-owners derive utility from the nonfinancial aspects of their firm. We also discuss how family firms' need for socioemotional wealth preservation explains behavioral differences between family and nonfamily firms in managerial decision making. Finally, we discuss the current state of socioemotional wealth research and propose potential directions for future research.

  16. Risk/Return Performance of Diversified Firms

    OpenAIRE

    Richard A. Bettis; Vijay Mahajan

    1985-01-01

    Based on a sample of 80 firms, this paper examines the risk/return performance of related and unrelated diversified firms at the level of accounting data. The results suggest that although on the average related diversified firms outperform unrelated diversified firms, related diversification offers no guarantee of a favorable risk/return performance. (Many low performers are related diversifiers.) In fact, different diversification strategies can result in similar risk/return performance. Ho...

  17. International Taxation and Multinational Firm Location Decisions

    OpenAIRE

    Barrios Cobos, Salvador; Huizinga, Harry; Laeven, Luc; Gaëtan J.A. Nicodème

    2008-01-01

    Using a large international firm-level data set, we estimate separate effects of host and parent country taxation on the location decisions of multinational firms. Both types of taxation are estimated to have a negative impact on the location of new foreign subsidiaries. In fact, the impact of parent country taxation is estimated to be relatively large, possibly reflecting its international discriminatory nature. For the cross-section of multinational firms, we find that parent firms tend to ...

  18. HRM in professional service firms

    DEFF Research Database (Denmark)

    Jørgensen, Frances

    2012-01-01

    Professional service firms (PSFs) present HR professionals with a number of unique challenges, as they share characteristics of both service and knowledge intensive organizations. While many of these firms are relying on High Commitment Work Practices (HCWPs) to enhance critical employee behaviors...... such as service quality and turnover, the analysis presented in this paper raises questions about traditional understandings of commitment in professional service environments. In particular, data from three Danish financial investment PSFs suggest that employees are more committed to developing and promoting...... on employee commitment. In the context of PSFs, we question the applicability of social exchange theory and instead draw upon the Ability-Motivation-Opportunity (AMO) framework (1982) to analyze how specific HRM practices contribute to the development of commitment, and to successful organizational outcomes...

  19. Technology Licensing and Firm Innovation

    DEFF Research Database (Denmark)

    Moreira, Solon

    that facilitate or constrain firms' ability to deal with licensed-in technologies have received little attention. This paper starts investigating in a longitudinal setting the effect of technology licensing on the number of patents produced by the licensee within the three years subsequent to the technology...... acquisition. The findings indicate that technology licensing is positively related to the number of inventions produced by the licensee in the years subsequent to the licensing deal. Subsequently, I investigate the moderating effect that organizational slack and myopia have on this main relationship....... The findings also suggest that high levels of Organizational Slack (available financial resources) strengthen the positive effect of licensing on innovation. However, higher levels of Organizational Myopia (the extent to which a firm draws on its own knowledge) can decrease the main effect of licensing....

  20. Simple models of firms emergence

    Science.gov (United States)

    Weisbuch, Gérard; Mangalagiu, Diana; Ben-Av, Radel; Solomon, Sorin

    2008-09-01

    We present a dynamical model of the emergence of firms as opposed to a flat labour market where entrepreneurs would recruit workers for each business opportunity. The model uses a preferential choice of partners based on previous collaborations experience. A sharp transition in the parameter space separates an ordered regime, where preferential links establish, from a disordered regime corresponding to a fast turnover of employees.

  1. Communication in an "Officeless firm"

    OpenAIRE

    Burchell, Brendan J.; Lai, Yi

    2008-01-01

    New technologies permit new types of organisations. This article describes and analyses one such organisation, an "officeless firm", where all employees work from their own homes and there is no central office. Drawing upon observations and interviews, the modes of communication and the nature of the interpersonal relationships that have permitted this organisation to succeed are described, along with the challenges that face this organisation in the future as it attempts to grow.

  2. Effects of beach replenishment on intertidal invertebrates: A 15-month, eight beach study.

    Science.gov (United States)

    Wooldridge, Tyler; Henter, Heather J.; Kohn, Joshua R.

    2016-06-01

    Beach replenishment is an increasingly popular means to remediate coastal erosion, but no consensus exists regarding how long replenishment affects sandy beach intertidal invertebrates, key components of beach ecosystems. We monitored the intertidal invertebrate community for fifteen months following a replenishment project at eight beaches, each with replenished and control sections, across San Diego County. Nearly all taxa showed major declines in abundance immediately following replenishment. Populations of talitrid amphipods and the bean clam Donax gouldii recovered within one year, sooner than in previous studies. On some beaches, populations of the mole crab Emerita analoga bloomed four months after replenishment and were more numerous on replenished portions of beaches at that time. Mole crab populations subsequently declined and no longer differed by treatment. The polychaete community, composed of Scolelepis sp. and several other numerically important taxa, showed a strong replenishment-induced reduction in abundance that persisted through the end of the study. The large negative effect of replenishment on polychaetes, coupled with their overall importance to the invertebrate community, resulted in a more than twofold reduction in overall invertebrate abundance on replenished beaches at 15 months. Such reductions may have far reaching consequences for sandy beach ecosystems, as community declines can reduce prey availability for shorebirds and fish. As this and other recent studies have revealed longer times for the recovery of intertidal invertebrates than previously observed, longer study periods and more cautious estimates regarding the magnitude, variability, and duration of impacts of beach replenishment for management decision-making are warranted.

  3. Firm-Level Corruption in Vietnam

    DEFF Research Database (Denmark)

    Rand, John; Tarp, Finn

    This paper uses a unique panel dataset on firm-level corruption. It contains quantitative information on bribe payments by a sample of formal and informal Vietnamese firms. We show that bribe incidence is highly associated with firm-level differences in (i) visibility, (ii) sunk costs, (iii...

  4. Reputational penalties to firms in antitrust investigations

    NARCIS (Netherlands)

    Broek, van den S.; Kemp, R.G.M.; Verschoor, W.F.C.; Vries, de A.

    2012-01-01

    We estimate the impact of legal penalties imposed on Dutch listed firms targeted by competition authorities from 1998 to 2008 on a firm's stock market value. On average, firms lose 2.3 percent of their market values when an antitrust investigation is uncovered. This corresponds to a total value loss

  5. Policies facilitating firm adjustment to globalization

    OpenAIRE

    Hoekman, Bernard; Javorcik, Beata Smarzynska

    2004-01-01

    The authors focus on policies facilitating firm adjustment to globalization. They briefly review the effects of trade and investment liberalization on firms, focusing on within-industry effects. They postulate that governments' role in supporting the process is to (1) ensure that firms face "right" incentives to adjust, and (2) intervene in areas where market failures are present. Their ma...

  6. Family firm research——A review

    Institute of Scientific and Technical Information of China (English)

    Qiang; Cheng

    2014-01-01

    This article reviews family firm studies in the finance and accounting literature,primarily those conducted using data from the United States and China. Family owners have unique features such as concentrated ownership, long investment horizon, and reputation concerns. Given the distinguishing features of family ownership and control, family firms face unique agency conflicts. We discuss the agency problems in family firms and review the findings of recent family firm studies. We call for more research to understand the unique family effects and encourage more research on Chinese family firms.Part I of the article discusses the fundaments of family firms: the prevalence of and the agency conflicts within family firms. Part II summarizes the findings of recent U.S. family firm studies. It reviews the evidence on the family firm premium(how, which, and when family firms are associated with a valuation premium), the manifestation of the agency conflict between majority and minority shareholders in family firms, earnings quality and corporate disclosure, and the determinants of family ownership and control. Part III discusses the prevalence and characteristics of Chinese family firms and reviews the findings of related studies. The article concludes with some suggestions for future research.

  7. Firm-Level Corruption in Vietnam

    DEFF Research Database (Denmark)

    Rand, John; Tarp, Finn

    This paper uses a unique panel dataset on firm-level corruption. It contains quantitative information on bribe payments by a sample of formal and informal Vietnamese firms. We show that bribe incidence is highly associated with firm-level differences in (i) visibility, (ii) sunk costs, (iii) abil...

  8. Reputational penalties to firms in antitrust investigations

    NARCIS (Netherlands)

    Broek, van den S.; Kemp, R.G.M.; Verschoor, W.F.C.; Vries, de A.

    2012-01-01

    We estimate the impact of legal penalties imposed on Dutch listed firms targeted by competition authorities from 1998 to 2008 on a firm's stock market value. On average, firms lose 2.3 percent of their market values when an antitrust investigation is uncovered. This corresponds to a total value loss

  9. Beach Nourishment History (1920s to 2000)

    Data.gov (United States)

    California Department of Resources — This is a dataset of beach nourishment history for the California Coastline from the 1920s to 2000. The original data was in tabular form (an Excel spreadsheet) and...

  10. Habitat--Offshore of Refugio Beach, California

    Data.gov (United States)

    U.S. Geological Survey, Department of the Interior — This part of SIM 3319 presents the habitat map of the seafloor (see sheet 7) offshore of Refugio Beach, California (vector data file is included in...

  11. Studies on beach changes at Visakhapatnam

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Rao, T.V.N.; Rao, D.P.

    experiences erosion during southwest monsoon and deposition during northeast monsoon and calm weather period. The annual sediment loss of about 75,000 cubic metres during the study period indicates the net erosional trend of the Visakhapatnam Beach and also...

  12. Folds--Offshore Refugio Beach, California

    Data.gov (United States)

    U.S. Geological Survey, Department of the Interior — This part of SIM 3319 presents folds for the geologic and geomorphic map (see sheets 10, SIM 3319) of Offshore Refugio Beach, California. The vector data file is...

  13. Contours--Offshore Refugio Beach, California

    Data.gov (United States)

    U.S. Geological Survey, Department of the Interior — This part of SIM 3319 presents bathymetric contours for several seafloor maps of Offshore Refugio Beach, California (vector data file is included in...

  14. Faults--Offshore Refugio Beach, California

    Data.gov (United States)

    U.S. Geological Survey, Department of the Interior — This part of SIM 3319 presents folds for the geologic and geomorphic map (see sheets 10, SIM 3319) of Offshore Refugio Beach, California. The vector data file is...

  15. Beach Nourishment History (1920s to 2000)

    Data.gov (United States)

    California Department of Resources — This is a dataset of beach nourishment history for the California Coastline from the 1920s to 2000. The original data was in tabular form (an Excel spreadsheet) and...

  16. Folds--Offshore Refugio Beach, California

    Data.gov (United States)

    U.S. Geological Survey, Department of the Interior — This part of SIM 3319 presents folds for the geologic and geomorphic map (see sheets 10, SIM 3319) of Offshore Refugio Beach, California. The vector data file is...

  17. Faults--Offshore Refugio Beach, California

    Data.gov (United States)

    U.S. Geological Survey, Department of the Interior — This part of SIM 3319 presents folds for the geologic and geomorphic map (see sheets 10, SIM 3319) of Offshore Refugio Beach, California. The vector data file is...

  18. Essays on Firm Behavior in Developing Economies

    Science.gov (United States)

    Abeberese, Ama Baafra

    The performance of firms is central to growth in developing economies. A burgeoning literature within development economics seeks to understand the behavior of firms in developing countries and the constraints to their performance. This dissertation explores two types of constraints---infrastructure-related constraints and trade-related constraints---faced by manufacturing firms in developing countries. Despite the widely acknowledged importance of infrastructure for economic growth, there has been relatively little research on how infrastructure affects the decisions of firms. Electricity, in particular, is commonly cited by firms in developing countries as a major obstacle to their performance. In the first two chapters, I analyze the responses of firms to two types of electricity constraints, namely electricity prices and electricity shortages. Chapter 1 provides evidence on how electricity prices affect a firm's industry choice and productivity growth using data on Indian manufacturing firms. I construct an instrument for electricity price as the interaction between the price of coal paid by power utilities, which is arguably exogenous to firm characteristics, and the initial share of thermal generation in a state's total electricity generation capacity. I find that, in response to an exogenous increase in electricity price, firms reduce their electricity consumption and switch to industries with less electricity-intensive production processes. I also find that firm output, machine intensity and labor productivity decline with an increase in electricity price. In addition to these level effects, I show that firm output and productivity growth rates are negatively affected by high electricity prices. These results suggest that electricity constraints faced by firms may limit a country's growth by leading firms to operate in industries with fewer productivity-enhancing opportunities. Chapter 2 examines the impact of electricity shortages on firm investment. I

  19. Founder Control,Ownership Structure and Firm Value:Evidence from Entrepreneurial Listed Firms in China

    Institute of Scientific and Technical Information of China (English)

    Lijun Xia

    2008-01-01

    In emerging markets, the deviation between the ultimate controlling shareholders’ voting rights and their cash flow rights(hereafter "DVC") in the listed firms is quite prevalent. DVC could be introduced due to the ultimate controlling shareholders’ opportunistic incentives, as well as by their incentives to improve firm efficiency. This study uses 229 listed firms ultimately controlled by individuals or families(hereafter "entrepreneurial firms") for 2004 in China, to investigate the effect of DVC on firm value and to determine whether it is different between founder and non-founder controlled firms. We find that DVC has a positive effect on firm value for founder controlled firms. This result implies that investors believe that their interests are better protected by founder controlled firms than by non-founder controlled firms.

  20. Toward an Integrative Theory of the Firm

    DEFF Research Database (Denmark)

    Li, Xin

    This paper argues that the existing four major theories of the firm, i.e., the transaction cost theory, resource-based view, the entrepreneurial theory, and the stakeholder theory, are all insightful yet partial because each of them has a particular focus on the phenomenon of the firm. To better...... understand the nature and behaviors of the firm, we need a comprehensive yet integrative theory. Toward this end, this paper proposes a relationship-based theory of the firm (R’BT) which claims that it is the relationships between the entrepreneur and other individuals or firms that determine the existence...

  1. Human Capital and Wages in Exporting Firms

    DEFF Research Database (Denmark)

    Munch, Jakob Roland; Skaksen, Jan Rose

    . Using a very rich matched worker-firm longitudinal dataset we find that firms with high export intensities pay higher wages. However, an interaction term between export intensity and skill intensity has a positive impact on wages and it absorbs the direct effect of the export intensity. That is, we find......This paper studies the link between a firms education level, export performance and wages of its workers. We argue that firms may escape intense competition in international markets by using high skilled workers to differentiate their products. This story is consistent with our empirical results...... an export wage premium, but it accrues to workers in firms with high skill intensities...

  2. Does Labor Diversity Affect Firm Performance?

    DEFF Research Database (Denmark)

    Pytlikova, Mariola; Pozzoli, Dario; Parrotta, Pierpaolo

    /education significantly enhances firm performance as measured by firm TFP. Conversely, diversity in demographics and ethnicity brings mixed results – both dimensions of workforce diversity have either no or negative effects on firm TFP. Hence, it seems as if the negative effects, coming from communication and integration...... costs connected to a more demographically and culturally diverse workforce, counteract the positive effects of diversity on firm TFP, coming from creativity and knowledge spillovers. However, we find that ethnic diversity is valuable for firms operating in industries characterized by above-average trade...... openness, giving support to the hypothesis that an ethnically diverse workforce provides information and access to global markets....

  3. Performance of Patenting Firms in Danish Manufacturing

    DEFF Research Database (Denmark)

    Madsen, Erik Strøjer; Smith, Valdemar; Nielsen, Anders Østergaard

    2000-01-01

    -patenting firms within the manufacturing sector in Denmark. Performance is measured both by growth in employment as well as in the return on equity and profit share in turnover. The results suggest that differences in performance of patenting and non-patenting firms are very small, which questions the political......Most countries focus on industries with high technology and the governments grant subsidies to innovating firms. However, there has been remarkable few studies of the performance of innovative firms or industries. This study examines the performance of patent active firms compared to the non...

  4. Firm Exit, Technological Progress and Trade

    DEFF Research Database (Denmark)

    Schröder, Philipp; Sørensen, Allan

    productivity firms survive longer, most firm closures are young firms, higher productivity exporters are more likely to continue to export compared to less productive exporters and market exits as well as firm closures are typically preceded by periods of contracting market shares. The present paper shows...... that the simple inclusion of exogenous economy wide technological progress into the standard Melitz (2003) model generates a tractable dynamic framework that generates endogenous exit decisions of firms in line with the stylized facts. Furthermore, we derive the effects of faster technological progress and trade...

  5. Endogenous Markups, Firm Productivity and International Trade:

    DEFF Research Database (Denmark)

    Bellone, Flora; Musso, Patrick; Nesta, Lionel

    In this paper, we test key micro-level theoretical predictions ofMelitz and Ottaviano (MO) (2008), a model of international trade with heterogenous firms and endogenous mark-ups. At the firm-level, the MO model predicts that: 1) firm markups are negatively related to domestic market size; 2......) markups are positively related to firm productivity; 3) markups are negatively related to import penetration; 4) markups are positively related to firm export intensity and markups are higher on the export market than on the domestic ones in the presence of trade barriers and/or if competitors...

  6. Do Treasure Islands Create Firm Value?

    DEFF Research Database (Denmark)

    Lai, Tat-kei; Ng, Travis

    They do! Otherwise, their use would not have been so prevalent among firms. How much firm value they create, however, is still an open question. Exploiting a political event in the U.K. that suddenly raised the cost of using tax havens, we find that there was a 0.87% reduction in cumulative...... abnormal return (CAR) among the sampled firms, corresponding to about £532 million in market capitalization. The firms of stronger corporate governance registered a stronger reduction in CAR. A simple linear extrapolation suggests that the firm value contributed by tax havens can be as much as £31 billion....

  7. Women in Top Management and Firm Performance

    DEFF Research Database (Denmark)

    Smith, Nina; Smith, Valdemar; Verner, Mette

    -observations for all Danish firms with more than 50 employees over the period 1994-2003, the analysis suggests that the proportion of women in top management jobs has from none to positive influence on firm performance. However, the results show that the strength of the effects of women in top management depends...... on how top CEOs are defined and on the method of estimation of the model. Next, the results point towards a positive influence on firm performance of the staff representation in the supervisory board of the firm but more women representing the shareholders in the supervisory board of the firm seems...

  8. The theory of the firm. Vol. 2

    DEFF Research Database (Denmark)

    Firms have for a long time been part of the explanatory set-up of economics. However, it is only recently that economists have felt the need for an economic theory addressing: why firms are different; why firms exist; what determines their boundaries relative to "the market"; and what determines...... their internal organization. This collection documents the rise of the modern theory of the firm during the last two to three decades. It reprints classic writings from a diversity of perspectives, including not only contractual theories of the firm, but also knowledge-based theories and theories of the firm...... as an information processor. In addition, the collection features perspectives from business strategy and business history as well as methodological and doctrinal historical perspectives. Including over 60 classic papers, these volumes collect together contributions on the theory of the firm, beginning with Ronald...

  9. Tetrabenazine is neuroprotective in Huntington's disease mice

    Directory of Open Access Journals (Sweden)

    Tang Tie-Shan

    2010-04-01

    Full Text Available Abstract Background Huntington's disease (HD is a neurodegenerative disorder caused by a polyglutamine (polyQ expansion in Huntingtin protein (Htt. PolyQ expansion in Httexp causes selective degeneration of striatal medium spiny neurons (MSN in HD patients. A number of previous studies suggested that dopamine signaling plays an important role in HD pathogenesis. A specific inhibitor of vesicular monoamine transporter (VMAT2 tetrabenazine (TBZ has been recently approved by Food and Drug Administration for treatment of HD patients in the USA. TBZ acts by reducing dopaminergic input to the striatum. Results In previous studies we demonstrated that long-term feeding with TBZ (combined with L-Dopa alleviated the motor deficits and reduced the striatal neuronal loss in the yeast artificial chromosome transgenic mouse model of HD (YAC128 mice. To further investigate a potential beneficial effects of TBZ for HD treatment, we here repeated TBZ evaluation in YAC128 mice starting TBZ treatment at 2 months of age ("early" TBZ group and at 6 months of age ("late" TBZ group. In agreement with our previous studies, we found that both "early" and "late" TBZ treatments alleviated motor deficits and reduced striatal cell loss in YAC128 mice. In addition, we have been able to recapitulate and quantify depression-like symptoms in TBZ-treated mice, reminiscent of common side effects observed in HD patients taking TBZ. Conclusions Our results further support therapeutic value of TBZ for treatment of HD but also highlight the need to develop more specific dopamine antagonists which are less prone to side-effects.

  10. Everyday cognition in prodromal Huntington disease.

    Science.gov (United States)

    Williams, Janet K; Kim, Ji-In; Downing, Nancy; Farias, Sarah; Harrington, Deborah L; Long, Jeffrey D; Mills, James A; Paulsen, Jane S

    2015-03-01

    Assessment of daily functions affected by cognitive loss in prodromal Huntington's disease (HD) is necessary in practice and clinical trials. We evaluated baseline and longitudinal sensitivity of the Everyday Cognition (ECog) scales in prodromal HD and compared self- and companion-ratings. Everyday cognition was self-assessed by 850 participants with prodromal HD and 768 companions. We examined internal structure using confirmatory factor analysis (CFA) on baseline data. For longitudinal analysis, we stratified participants into Low, Medium, and High disease progression groups. We examined ECog scores for group differences and participant-and-companion differences using linear mixed effects regression (LMER). Comparison with the Total Functional Capacity (TFC) scale was made. CFA revealed good fit of a 5-factor model having a global factor (total score), and subfactors (subscales) of memory, language, visuospatial perception, and executive function. At study entry, participants and companions in the Medium and High groups reported significantly worsened everyday cognition as well as significant functional decline over time. Losses became more pronounced and participant and companion ratings diverged as individuals progressed. TFC showed significant functional loss over time in the High group but not in the Medium group. Disease progression is associated with reduced self- and companion-reported everyday cognition in prodromal HD participants who are less than 13 years to estimated motor onset. Our findings suggest companion ratings are more sensitive than participants' for detecting longitudinal change in daily cognitive function. ECog appears more sensitive to specific functional changes in the prodrome of HD than the TFC. PsycINFO Database Record (c) 2015 APA, all rights reserved.

  11. An holistic approach to beach erosion vulnerability assessment.

    Science.gov (United States)

    Alexandrakis, George; Poulos, Serafim Ε

    2014-08-15

    Erosion is a major threat for coasts worldwide, beaches in particular, which constitute one of the most valuable coastal landforms. Vulnerability assessments related to beach erosion may contribute to planning measures to counteract erosion by identifying, quantifying and ranking vulnerability. Herein, we present a new index, the Beach Vulnerability Index (BVI), which combines simplicity in calculations, easily obtainable data and low processing capacity. This approach provides results not only for different beaches, but also for different sectors of the same beach and enables the identification of the relative significance of the processes involved. It functions through the numerical approximation of indicators that correspond to the mechanisms related to the processes that control beach evolution, such as sediment availability, wave climate, beach morhodynamics and sea level change. The BVI is also intended to be used as a managerial tool for beach sustainability, including resilience to climate change impact on beach erosion.

  12. 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.

  13. The firm-nature relationship

    DEFF Research Database (Denmark)

    Ulhøi, John Parm; Madsen, Henning

    2005-01-01

    Taking the stakeholder concept of the firm as a starting point, the paper presents data and analyses from a longitudinal study initiated in 1995 and repeated every four years. In discussing state-of-the-art insights and experiences, the paper identifies challenges which, if adequately addressed......, may help bring the discipline out of its theoretical and epistemological deadlock. The paper concludes that, unless academia proactively brings the environmental management discipline within its domain, reductionists are likely to dominate the agenda-setting in the ongoing environmental debate......, reducing it to a question of self-regulated eco-modernist actions. In closing, the paper addresses implications for academia and industry....

  14. The Counselor and Genetic Disease: Huntington's Disease as a Model.

    Science.gov (United States)

    Wexler, Nancy S.

    This speech offers a brief description of Huntington's Disease (HD): its causes, symptoms, and incidence. It then concentrates on the psychological problems of persons one of whose parents had the disease, and the role of the counselor in helping these humans cope with their fears about contacting it themselves. A relatively detailed case study is…

  15. Age, CAG repeat length, and clinical progression in Huntington's disease.

    Science.gov (United States)

    Rosenblatt, Adam; Kumar, Brahma V; Mo, Alisa; Welsh, Claire S; Margolis, Russell L; Ross, Christopher A

    2012-02-01

    The objective of this study was to further explore the effect of CAG repeat length on the rate of clinical progression in patients with Huntington's disease. The dataset included records for 569 subjects followed prospectively at the Baltimore Huntington's Disease Center. Participants were seen for a mean of 7.1 visits, with a mean follow-up of 8.2 years. Subjects were evaluated using the Quantified Neurologic Examination and its Motor Impairment subscale, the Mini-Mental State Examination, and the Huntington's disease Activities of Daily Living Scale. By itself, CAG repeat length showed a statistically significant but small effect on the progression of all clinical measures. Contrary to our previous expectations, controlling for age of onset increased the correlation between CAG repeat length and progression of all variables by 69% to 159%. Graphical models further supported the idea that individuals with smaller triplet expansions experience a more gradual decline. CAG repeat length becomes an important determinant of clinical prognosis when accounting for age of onset. This suggests that the aging process itself influences clinical outcomes in Huntington's disease. Inconsistent results in prior studies examining CAG repeat length and progression may indeed reflect a lack of age adjustment.

  16. Biological Markers of Cognition in Prodromal Huntington's Disease: A Review

    Science.gov (United States)

    Papp, Kathryn V.; Kaplan, Richard F.; Snyder, Peter J.

    2011-01-01

    Huntington's disease (HD), an autosomal-dominant genetic disorder, has historically been viewed as a degenerative movement disorder but it also includes psychiatric symptoms and progressive cognitive decline. There has been a lack of consensus in the literature about whether or not cognitive signs can be detected in carriers before clinical…

  17. Huntington II Simulation Program-POLUT. Teacher's Guide.

    Science.gov (United States)

    Braun, L.; And Others

    This teacher's guide is written to accompany the Huntington II Simulation Program - POLUT. POLUT is a program written in BASIC which provides simulation of the interaction between water and waste. It creates a context within which the user can control specific variables which effect the quality of a water resource. The teacher's guide provides…

  18. 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 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 ...

  19. Clinical and genetic features of Huntington disease in Sri Lanka.

    Science.gov (United States)

    Sumathipala, Dulika S; Jayasekara, Rohan W; Dissanayake, Vajira H W

    2013-12-05

    Huntington disease was one of the first neurological hereditary diseases for which genetic testing was made possible as early as 1993. The study describes the clinical and genetic characteristics of patients with Huntington disease in Sri Lanka. Data of 35 consecutive patients tested from 2007 to 2012 at the Human Genetics Unit, Faculty of Medicine, University of Colombo was analyzed retrospectively. Clinical data and genetic diagnostic results were reviewed. Statistical analysis was performed using descriptive statistics. Thirty patients had fully penetrant (FP) CAG repeat mutations and 5 had reduced penetrant (RP) CAG repeat mutations. In the FP group mean ages of onset and diagnosis were 37.5 and 40.4 years, while in the RP group it was 63.0 and 64.8 years respectively. The age of diagnosis ranged from 15 to 72 years, with 2 patients with Juvenile onset (60 years) Huntington disease. The symptoms at diagnosis were predominantly motor (32/35 -91%). Three patients had psychiatric and behavioral disorders. The age difference between onset and genetic diagnosis showed significant delay in females compared to males (p Huntington disease in the Sri Lankan study population were similar to that previously reported in literature.

  20. 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.

  1. Expression pattern of apoptosis-related markers in Huntington's disease

    NARCIS (Netherlands)

    Vis, José C; Schipper, Ellis; de Boer-van Huizen, Roelie T; Verbeek, Marcel M; de Waal, Rob M W; Wesseling, Pieter; ten Donkelaar, Hans J; Kremer, Berry

    2005-01-01

    Inappropriate apoptosis has been implicated in the mechanism of neuronal death in Huntington's disease (HD). In this study, we report the expression of apoptotic markers in HD caudate nucleus (grades 1-4) and compare this with controls without neurological disease. Terminal transferase-mediated biot

  2. Shoreface storm morphodynamics and mega-rip evolution at an embayed beach: Bondi Beach, NSW, Australia

    Science.gov (United States)

    McCarroll, R. Jak; Brander, Robert W.; Turner, Ian L.; Leeuwen, Ben Van

    2016-03-01

    Embayed beach dynamics differ from open beaches due to the nature of headland control. Their resultant morphologies and morphodynamic behaviour are poorly understood due in part to a critical lack of surfzone and nearshore bathymetry observations. This study describes the morphodynamic storm response of a high-energy intermediate, 850 m long embayed beach over a three week period spanning a cluster of storms. A headland and subaqueous ridge protects the northern end of the beach, resulting in an alongshore wave height gradient. Contrary to existing beach state conceptual models, under energetic forcing the beach did not 'reset' or enter a 'cellular mega-rip' beach state. The protected northern end persisted in a low energy state, while the wave exposed southern section transitioned from transverse-bar-and-rip to a complex double-bar system, a process previously undescribed in the literature. Bar-rip morphology at the exposed end of the beach migrated offshore to greater depths, leaving an inner-reflective beach and longshore trough, while a mega-rip channel with 3 m relief developed at the exposed headland. The number of rip channels remained near constant over multiple storm events. Offshore sediment flux was 350 m3/m at the exposed headland and 20 m3/m at the protected end. Alongshore bathymetric non-uniformity decreased over the sub-aerial beach and inner surfzone, but increased in the outer surfzone and beyond. Suggested mechanisms for the persistence of 3D morphology during the cluster of storms include: (i) wave refraction to shore normal within the embayment; (ii) alongshore energy gradients; and (iii) pre-existing bar-rip morphology. Formation of the complex multi-bar state may be related to antecedent morphology, headland geometry, substrate gradient and localised hydrodynamic interactions near the headland. A new conceptual embayed beach state model is proposed for asymmetric, transitional embayed beaches. The model describes a pre-storm embayment where

  3. Haeundae beach in Korea: Seasonal-to-decadal wave statistics and impulsive beach responses to typhoons

    Science.gov (United States)

    Lee, Hee Jun; Do, Jong-Dae; Kim, Sun Sin; Park, Won-Kyung; Jun, Kicheon

    2016-12-01

    Haeundae Beach represents Korean pocket beaches that are currently erosional and dominated by summertime typhoons. The decadal wave characteristics 9 km offshore of Haeundae Beach were analyzed using the WAM model that was validated through the 2007 wave observations. The wave statistics modelled for 1979-2007 indicates that the seasonal mean significant wave height (H s) is highest (0.6-0.7 m) in summer due to typhoons, in contrast to the lowest (around 0.5 m) autumn analog. The wave direction is also pronouncedly seasonal with the principal bearings of SSW and NE in the summer and winter seasons, respectively. The WAM results additionally show that the H s has gradually increased over the region of Haeundae Beach since 1993. Beach profiling during June-November 2014 shows the opposite processes of the typhoon and fair-weather on beach sands. During a typhoon, foreshore sands were eroded and then accumulated as sand bars on the surf zone. In the subsequent fair-weather, the sand bars moved back to the beach resulting in the surf-zone erosion and foreshore accretion. A total of 5 cycles of these beach-wide sand movements yielded a net retreat (up to 20 m) of the shoreline associated with large foreshore erosion. However, the surf zone only slightly accumulated as a result of the sand cycles. This was attributed to the sand escape offshore from the westernmost tip of the beach. The present study may provide an important clue to understanding the erosional processes in Haeundae Beach.

  4. Founder Control, Ownership Structure and Firm Value: Evidence from Entrepreneurial Listed Firms in China

    Directory of Open Access Journals (Sweden)

    Lijun Xia

    2008-06-01

    Full Text Available In emerging markets, the deviation between the ultimate controlling shareholders' voting rights and their cash flow rights (hereafter “DVC” in the listed firms is quite prevalent. DVC could be introduced due to the ultimate controlling shareholders' opportunistic incentives, as well as by their incentives to improve firm efficiency. This study uses 229 listed firms ultimately controlled by individuals or families (hereafter “entrepreneurial firms” for 2004 in China, to investigate the effect of DVC on firm value and to determine whether it is different between founder and non-founder controlled firms. We find that DVC has a positive effect on firm value for founder controlled firms. This result implies that investors believe that their interests are better protected by founder controlled firms than by non-founder controlled firms.

  5. Employing Real Time PCR for the Diagnosis of Huntington Disease

    Directory of Open Access Journals (Sweden)

    Frouzandeh Mahjoubi

    2013-07-01

    Full Text Available Background: Huntington disease (HD is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia. The onset age of this disease is varied but usually is between the ages 40-50. Huntington's disease is caused by a triplet-repeat expansion in the IT15 gene (also known as huntingtin or HD which is located on chromosome 4p3.1. Since many clinical picture of HD are indistinguishable from other distinct genetic disorders molecular test such as PCR is the only way to confirm the disease. The aim of this study was to introduce a new and fast technique for the diagnosis of Huntington disease.Materials and Methods: Blood specimens were collected from individuals suspected for Huntington disease and also people with no symptoms and family history of this disease. DNAs were extracted according to standard protocol. Using conventional PCR, patient positive for Huntington disease were diagnosed. Then employing real time PCR on the basis of difference between melting temperature (Tm a new and fast diagnostic method was introduced.Results: Among 29 patients suspected to be HD only 8 HD patients were confirmed using PCR and real time PCR. The numbers of CAG repeat were between 42-50 and melting temperatures were between 89-92.Conclusion: The concept of using melting temperature in real time PCR protocol presented in here could be employed for the rapid diagnosis of the diseases caused by the increased in triple repeat sequences. It is fast, robust and has the potential use for the prenatal diagnosis.

  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. Firm Projects, NPV and Risk

    CERN Document Server

    Hudakova, J

    2005-01-01

    An investor is estimating net present value of a firm project and performs risk analysis. Usually it is created portfolio hierarchies and make comparison of variants of project based on these hierarchies. Then one finds that portfolio which corresponds to the particular needs of individual groups within the firm. We have formulated a new type of NPV analysis based on the fact that normal distribution of NPV is observed for some projects in some industries. The expected risk of the project is given by variance, in which there is the standard deviation of the year n cash flow, the standard deviation of the investment I in the time zero, the correlation coefficient of the year n cash flow deviation from the average and of the investment I at time zero deviation from the mean investment at time zero, the correlation coefficient of the year n cash flow deviation from the average and of the year n' cash flow deviation from the average. The aim function of the investor into the project was found. The investor is cha...

  8. Threats to sandy beach ecosystems: A review

    Science.gov (United States)

    Defeo, Omar; McLachlan, Anton; Schoeman, David S.; Schlacher, Thomas A.; Dugan, Jenifer; Jones, Alan; Lastra, Mariano; Scapini, Felicita

    2009-01-01

    We provide a brief synopsis of the unique physical and ecological attributes of sandy beach ecosystems and review the main anthropogenic pressures acting on the world's single largest type of open shoreline. Threats to beaches arise from a range of stressors which span a spectrum of impact scales from localised effects (e.g. trampling) to a truly global reach (e.g. sea-level rise). These pressures act at multiple temporal and spatial scales, translating into ecological impacts that are manifested across several dimensions in time and space so that today almost every beach on every coastline is threatened by human activities. Press disturbances (whatever the impact source involved) are becoming increasingly common, operating on time scales of years to decades. However, long-term data sets that describe either the natural dynamics of beach systems or the human impacts on beaches are scarce and fragmentary. A top priority is to implement long-term field experiments and monitoring programmes that quantify the dynamics of key ecological attributes on sandy beaches. Because of the inertia associated with global climate change and human population growth, no realistic management scenario will alleviate these threats in the short term. The immediate priority is to avoid further development of coastal areas likely to be directly impacted by retreating shorelines. There is also scope for improvement in experimental design to better distinguish natural variability from anthropogenic impacts. Sea-level rise and other effects of global warming are expected to intensify other anthropogenic pressures, and could cause unprecedented ecological impacts. The definition of the relevant scales of analysis, which will vary according to the magnitude of the impact and the organisational level under analysis, and the recognition of a physical-biological coupling at different scales, should be included in approaches to quantify impacts. Zoning strategies and marine reserves, which have not

  9. The role of beach morphodynamic state on infragravity swash on beaches: field observations.

    Science.gov (United States)

    Gomes da Silva, Paula; González, Mauricio; Medina, Raul

    2017-04-01

    The runup generated by waves can be defined as the maximum height above sea water level on the coastline and is an important criterion for costal structures/nourishment design and erosion/flooding risk analysis. Given the complexity of nonlinear processes involved in the runup generation, its prediction is commonly made by means of empirical formulations that relate wave and beach parameters. The most accepted parametrization presented till the moment was proposed by Stockdon et al. (2006), in which the runup exceeded by 2 percent of the waves (R2) is described in terms of setup (η - the steady superelevation of the mean water level caused by breaking waves) and incident and infragravity swash (Sinc and Sig- time-varying fluctuations around the setup caused by non-breaking waves). Such formulation has been widely accepted and its efficiency was appraised in many works. Nevertheless, although empirical parametrization of infragravity swash using incident wave's parameters shows reasonable skill, the correlation can still present considerable scatter. The amount of infragravity energy on swash is directly related to the morphodynamic beach state, in a way that beach profiles classified as reflective (low wave energy, coarse sediment and higher beach slope) tend to show lower Sig values than dissipative ones (high wave energy, fine sediment and lower beach slope). However, since Stockdon's formula for predicting infragravity swash consider only wave parameters, its use implies that beaches receiving the same wave energy but with different grain size and beach slope would present the same Sig values. This work assumed the hypothesis that the scatter verified on the predictions of the infragravity swash is mainly related to the lack of information about the beach state in Stockdon formula. Based on that, a field campaign was designed and carried out in Somo-El Puntal beach, north Spain, with the aim of generating data to be analyzed in terms of infragravity swash. An

  10. Beach science in the Great Lakes

    Science.gov (United States)

    Nevers, Meredith B.; Byappanahalli, Murulee N.; Edge, Thomas A.; Whitman, Richard L.

    2014-01-01

    Monitoring beach waters for human health has led to an increase and evolution of science in the Great Lakes, which includes microbiology, limnology, hydrology, meteorology, epidemiology, and metagenomics, among others. In recent years, concerns over the accuracy of water quality standards at protecting human health have led to a significant interest in understanding the risk associated with water contact in both freshwater and marine environments. Historically, surface waters have been monitored for fecal indicator bacteria (fecal coliforms, Escherichia coli, enterococci), but shortcomings of the analytical test (lengthy assay) have resulted in a re-focusing of scientific efforts to improve public health protection. Research has led to the discovery of widespread populations of fecal indicator bacteria present in natural habitats such as soils, beach sand, and stranded algae. Microbial source tracking has been used to identify the source of these bacteria and subsequently assess their impact on human health. As a result of many findings, attempts have been made to improve monitoring efficiency and efficacy with the use of empirical predictive models and molecular rapid tests. All along, beach managers have actively incorporated new findings into their monitoring programs. With the abundance of research conducted and information gained over the last 25 years, “Beach Science” has emerged, and the Great Lakes have been a focal point for much of the ground-breaking work. Here, we review the accumulated research on microbiological water quality of Great Lakes beaches and provide a historic context to the collaborative efforts that have advanced this emerging science.

  11. Beach Management & Analysis of the Visitors’ Remarks

    Directory of Open Access Journals (Sweden)

    Perihan Paksoy

    2014-07-01

    Full Text Available User perceptions can become vital especially at beach preferences as cleanliness, safety and amenities are some of the apparent factors that will affect. With the awareness of probable adaptation of beach users’ demands into policy recommendations, a case study has been carried out at Black Sea Coast of İstanbul at Şile beaches. Şile has been chosen in this study purposefully as it is a touristic district of İstanbul which has aimed to earn Blue Flag award previously. Secondly, it receives high amount of visitors especially during the peak periods in weekends; as it has a very close location to the city, people are choosing here most of the time just for the day. In this research with factors about human use of beach and impacts like cleanliness and sufficiency of amenities (showers, toilets, changing cubicles, parks etc. and the number of lifeguards are studied. Regarding the findings, the researchers consequently highlight recommendations for Şile beach management which could enhance the visitor experience.

  12. Beach Management & Analysis of Visitors’ Remarks

    Directory of Open Access Journals (Sweden)

    Perihan Paksoy

    2016-01-01

    Full Text Available User perceptions can become vital especially at beach preferences as cleanliness, safety and amenities are some of the apparent factors that will affect. With the awareness of probable adaptation of beach users’ demands into policy recommendations, a case study has been carried out at Black Sea Coast of İstanbul at Şile beaches. Şile has been chosen in this study purposefully as it is a touristic district of İstanbul which has aimed to earn Blue Flag award previously. Secondly, it receives high amount of visitors especially during the peak periods in weekends; as it has a very close location to the city, people are choosing here most of the time just for the day. In this research with factors about human use of beach and impacts like cleanliness and sufficiency of amenities (showers, toilets, changing cubicles, parks etc. and the number of lifeguards are studied. Regarding the findings, the researchers consequently highlight recommendations for Şile beach management which could enhance the visitor experience.

  13. Decomposing Firm-level Sales Variation

    DEFF Research Database (Denmark)

    Munch, Jakob Roland; Nguyen, Daniel Xuyen

    , and that for the median product it drives 31% of the sales variation. When we remove first-time exports from our sample, the median value increases to 40%, implying that firm-destination-specific effects are most important the first year. We conclude that while firm-specific productivity can account for some......We measure the contribution of firm-specific effects to overall sales variation within a destination and find it remarkably low. Our empirical decomposition is structurally motivated by a heterogeneity model of exporting involving destination-specific, firm-specific, and firm......-destination-specific latent effects with incidental truncation. We use a highly detailed dataset with exports by products and destinations for all Danish manufacturing fi…rms. We fi…nd the contribution of firm-specific heterogeneity to within-destination sales variation varies greatly across HS6 products...

  14. Decomposing Firm-level Sales Variation

    DEFF Research Database (Denmark)

    Munch, Jakob Roland; Nguyen, Daniel Xuyen

    , and that for the median product it drives 31% of the sales variation. When we remove first-time exports from our sample, the median value increases to 40%, implying that firm-destination-specific effects are most important the first year. We conclude that while firm-specific productivity can account for some......We measure the contribution of firm-specific effects to overall sales variation within a destination and find it remarkably low. Our empirical decomposition is structurally motivated by a heterogeneity model of exporting involving destination-specific, firm-specific, and firm......-destination-specific latent effects with incidental truncation. We use a highly detailed dataset with exports by products and destinations for all Danish manufacturing fi…rms. We fi…nd the contribution of firm-specific heterogeneity to within-destination sales variation varies greatly across HS6 products...

  15. Foreign Exchange Exposures of Korean Firms

    Directory of Open Access Journals (Sweden)

    Sungbin Cho

    2011-03-01

    Full Text Available We measure foreign exchange exposures as sensitivity of firm's value to FX premium in the CAPM plus FX premium model, and try to find determinants of the exposures; using data of non-financial companies listed in the Korea Exchange from the year 2007 to 2008. Main findings are as follows. If Korean won depreciates, only a small number of firms is benefitted while majority of firms are harmed to the contrary of common knowledge. As a firm's export increases, the foreign exchange exposure increases up to a certain level and after that it declines. And, smaller firms of negative foreign exchange exposures are more sensitive to foreign exchange changes. These suggest heterogeneous effects of foreign exchange rates on industries and firms.

  16. Paradoxes and Innovation in Family Firms

    DEFF Research Database (Denmark)

    Ingram, Amy E.; Lewis, Marianne W.; Barton, Sid

    2016-01-01

    Scholars stress that family firms are inherently paradoxical, and that tensions, such as tradition versus change, family liquidity versus business growth, and founder control versus successor autonomy, can both inhibit and foster innovation. Further, theorists propose that firms led by paradoxical...... thinkers are more likely to manage these tensions and fuel innovative behavior. Leveraging family business and organizational paradox literatures, this multi-stage exploratory study develops measures of paradoxical tensions and paradoxical thinking in family firms, and tests these propositions. Findings...

  17. Accounting Firms: Global Spread with Limited Transnationalization

    OpenAIRE

    Jochen Zimmermann; Jan-Christoph Volckmer

    2012-01-01

    As financial markets have increasingly globalised, the regulatory framework is still nationally fragmented. One core regulatory element is the provision of assurance services by accounting firms. The Big 4 - the leading international providers of audit services Ð are not as international as are accounting standards or their clients. The paper traces three facets of the transnationalization of accounting firms: explanations for internationalization rooted in the theory of the firm, their modes...

  18. Do African Manufacturing Firms Learn from Exporting?

    OpenAIRE

    Paul Collier; Marcel Fafchamps; Francis Teal; Stefan Dercon

    2002-01-01

    In this paper, we use firm-level panel data for the manufacturing sector in four African countries to estimate the effect of exporting on efficiency. Estimating simultaneously a production function and an export regression that control for unobserved firm effects, we find both significant efficiency gains from exporting, supporting the learning-byexporting hypothesis, and evidence for self-selection of more efficient firms into exporting. The evidence of learning-by-exporting suggests that Af...

  19. Does Innovation Mediate Good Firm Performance?

    OpenAIRE

    Llanto, Gilberto M.; del Prado, Fatima

    2015-01-01

    Private firms invest in physical capital and human resource but they are also advised to invest in innovations to be more productive and profitable. Innovations refer to the development, deployment, and economic utilization of new products, processes, and services. It is important for firms to know whether investment in innovations is investment well-spent. Our empirical results provided an affirmative response to the question raised in this paper: "Does innovation mediate good firm performan...

  20. EPA Office of Water (OW): Beaches NHDPlus Indexed Dataset

    Data.gov (United States)

    U.S. Environmental Protection Agency — The Beaches Environmental Assessment and Coastal Health (BEACH) Program focuses on the following five areas to meet the goals of improving public health and...

  1. Evaluation of Subterranean Subsidence at Seal Beach National Wildlife Refuge

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — The purpose of this study was to determine the amount of subsurface subsidence at Naval Weapons Station Seal Beach (NWSSB) areas which include Seal Beach National...

  2. EPA Office of Water (OW): Beaches PRAWN Attribute Data

    Data.gov (United States)

    U.S. Environmental Protection Agency — The Beaches Environmental Assessment and Coastal Health (BEACH) Program focuses on the following five areas to meet the goals of improving public health and...

  3. Beach processes between Mulgund and Shiroda, west coast of India

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Nayak, B.U.; Sanilkumar, V.; Pathak, K.C.

    Study on beach processes for an year shows seasonal changes without annual net erosion. The beaches are stable and regain the maximum profiles during February to April. Distribution of longshore current direction is not uniform along the study...

  4. Climate induced changes in beach morphology and sediment dynamics, Machilipatnam

    Digital Repository Service at National Institute of Oceanography (India)

    Chauhan, O.S.

    The wave climate, littoral current patterns, monthly and seasonal longshore drift rates, beach profile changes, and sediment budget of the beach sediments were determined along Machilipatnam, Andhra Pradesh (India) for the NE, SW monsoons...

  5. Beach Advisory and Closing Online Notification (BEACON) system

    Data.gov (United States)

    U.S. Environmental Protection Agency — Beach Advisory and Closing Online Notification system (BEACON) is a colletion of state and local data reported to EPA about beach closings and advisories. BEACON is...

  6. A simplified model of pathogenic pollution for managing beaches ...

    African Journals Online (AJOL)

    A simplified model of pathogenic pollution for managing beaches. ... key physical processes involved in mixing and dispersion of pathogenic pollution at ... Key words: beach-water quality model, pathogenic pollution, storm-water runoff, E. coli

  7. An Aspirational Community Theory of the Firm

    OpenAIRE

    2013-01-01

    All of the three major theories of the firm, i.e., the transaction cost theory, knowledge-based theory and the entrepreneurship theory, offer some insightful analyses of the nature of the firm. However, they all have limitations and weaknesses in answering the fundamental question of the existence of the firm. In addition, they are all partial due to their particular focus on the multifaceted phenomenon of the firm. We argue that it is necessary and sufficient to develop a comprehensive yet i...

  8. Does Labour Diversity affect Firm Productivity?

    DEFF Research Database (Denmark)

    Parrotta, Pierpaolo; Pozzoli, Dario; Pytlikova, Mariola

    Using a matched employer-employee dataset, we analyze how workforce diversity in cultural background, education and demographic characteristics affects productivity of firms in Denmark. Implementing a structural estimation of the firms' production function (Ackerberg et al., 2006) we find...... that labor diversity in education significantly enhances a firm's value added. Conversely, diversity in ethnicity and demographics induces negative effects on firm productivity. Hence, the negative effects, coming from communication and integration costs connected to a more culturally and demographically...... diverse workforce, seem to outweigh the positive effects coming from creativity and knowledge spillovers....

  9. Human Capital and Wages in Exporting Firms

    DEFF Research Database (Denmark)

    Munch, Jakob Roland; Skaksen, Jan Rose

    This paper studies the link between a firms education level, export performance and wages of its workers. We argue that firms may escape intense competition in international markets by using high skilled workers to differentiate their products. This story is consistent with our empirical results....... Using a very rich matched worker-firm longitudinal dataset we find that firms with high export intensities pay higher wages. However, an interaction term between export intensity and skill intensity has a positive impact on wages and it absorbs the direct effect of the export intensity. That is, we find...

  10. Shared vision promotes family firm performance.

    Science.gov (United States)

    Neff, John E

    2015-01-01

    A clear picture of the influential drivers of private family firm performance has proven to be an elusive target. The unique characteristics of private family owned firms necessitate a broader, non-financial approach to reveal firm performance drivers. This research study sought to specify and evaluate the themes that distinguish successful family firms from less successful family firms. In addition, this study explored the possibility that these themes collectively form an effective organizational culture that improves longer-term firm performance. At an organizational level of analysis, research findings identified four significant variables: Shared Vision (PNS), Role Clarity (RCL), Confidence in Management (CON), and Professional Networking (OLN) that positively impacted family firm financial performance. Shared Vision exhibited the strongest positive influence among the significant factors. In addition, Family Functionality (APGAR), the functional integrity of the family itself, exhibited a significant supporting role. Taken together, the variables collectively represent an effective family business culture (EFBC) that positively impacted the long-term financial sustainability of family owned firms. The index of effective family business culture also exhibited potential as a predictive non-financial model of family firm performance.

  11. Shared Vision promotes family firm performance

    Directory of Open Access Journals (Sweden)

    John Edward Neff

    2015-05-01

    Full Text Available A clear picture of the influential drivers of private family firm performance has proven to be an elusive target. The unique characteristics of private family owned firms necessitate a broader, non-financial approach to reveal firm performance drivers. This research study sought to specify and evaluate the themes that distinguish successful family firms from less successful family firms. In addition, this study explored the possibility that these themes collectively form an effective organizational culture that improves longer-term firm performance. At an organizational level of analysis, research findings identified four significant variables: Shared Vision (PNS, Role Clarity (RCL, Confidence in Management (CON, and Professional Networking (OLN that positively impacted family firm financial performance. Shared Vision exhibited the strongest positive influence among the significant factors. In addition, Family Functionality (APGAR, the functional integrity of the family itself exhibited a significant supporting role. Taken together, the variables collectively represent an effective family business culture (EFBC that positively impacted the long-term financial sustainability of family owned firms. The index of effective family business culture also exhibited potential as a predictive non-financial model of family firm performance.

  12. International working capital practices of Ghanaian firms

    Directory of Open Access Journals (Sweden)

    J. Abor

    2005-12-01

    Full Text Available International working capital management is important to firms frequently operating in the international market. This article investigates the international working capital practices of top Ghanaian firms involved in international trade. The objective of the study is to ascertain the extent to which Ghanaian firms use international working capital management vehicles. The article focuses on two main areas of international working capital management; international cash management and international sales and accounts receivables management. The results of this study reveal low level of use of international working capital vehicles among Ghanaian firms. Recommendations are made in this regard.

  13. Industrial environmental practices in Polish Firms

    DEFF Research Database (Denmark)

    Kræmer, Trine Pipi

    The study investigates how discursive developments in Poland interact with industrial environmental practices in five production firms. The analysis of the discursive development covers the period from the end of World War I to the turn of the century. The areas in focus are identity, industry......, and environment. The five case firms all had a Communist past. The firms represent three different industrial sectors; i.e. textile, energy, and publishing and printing industries. Furthermore, the firms are both private and state owned as well as in the process of privatisation....

  14. EAS Test Firm Accrediting Bodies (TFAB)

    Data.gov (United States)

    Federal Communications Commission — EAS (Equipment Authorization System). The accreditation bodies for testing laboratories are referred to as Test Firm Accrediting Bodies (TFABs). They are responsible...

  15. Targeting the Cholinergic System to Develop a Novel Therapy for Huntington's Disease.

    Science.gov (United States)

    D'Souza, Gary X; Waldvogel, Henry J

    2016-12-15

    In this review, we outline the role of the cholinergic system in Huntington's disease, and briefly describe the dysfunction of cholinergic transmission, cholinergic neurons, cholinergic receptors and cholinergic survival factors observed in post-mortem human brains and animal models of Huntington's disease. We postulate how the dysfunctional cholinergic system can be targeted to develop novel therapies for Huntington's disease, and discuss the beneficial effects of cholinergic therapies in pre-clinical and clinical studies.

  16. Crime in Huntington's disease: a study of registered offences among patients, relatives, and controls

    OpenAIRE

    Jensen, P; Fenger, K; Bolwig, T; Sorensen, S. A.

    1998-01-01

    OBJECTIVES—Criminal behaviour has been described as a problem in Huntington's disease, but systematic studies including control groups have been missing. Based on information from Danish registries, rates and types of crime committed by patients with Huntington's disease, non-affected relatives, and controls were studied.
METHODS—99 males and 151 females with Huntington's disease were compared with 334 non-affected first degree relatives (134 men and 200 women) and to matche...

  17. Through the sands of time: Beach litter trends from nine cleaned north cornish beaches.

    Science.gov (United States)

    Watts, Andrew J R; Porter, Adam; Hembrow, Neil; Sharpe, Jolyon; Galloway, Tamara S; Lewis, Ceri

    2017-09-01

    Marine litter and its accumulation on beaches is an issue of major current concern due to its significant environmental and economic impacts. Yet our understanding of spatio-temporal trends in beach litter and the drivers of these trends are currently limited by the availability of robust long term data sets. Here we present a unique data set collected systematically once a month, every month over a six year period for nine beaches along the North Coast of Cornwall, U.K. to investigate the key drivers of beach litter in the Bude, Padstow and Porthcothan areas. Overall, an average of 0.02 litter items m(-2) per month were collected during the six year study, with Bude beaches (Summerleaze, Crooklets and Widemouth) the most impacted (0.03 ± 0.004 litter items m(-2) per month). The amount of litter collected each month decreased by 18% and 71% respectively for Padstow (Polzeath, Trevone and Harlyn) and Bude areas over the 6 years, possibly related to the regular cleaning, however litter increased by 120% despite this monthly cleaning effort on the Padstow area beaches. Importantly, at all nine beaches the litter was dominated by small, fragmented plastic pieces and rope fibres, which account for 32% and 17% of all litter items collected, respectively. The weathered nature of these plastics indicates they have been in the marine environment for an extended period of time. So, whilst classifying the original source of these plastics is not possible, it can be concluded they are not the result of recent public littering. This data highlights both the extent of the marine litter problem and that current efforts to reduce littering by beach users will only tackle a fraction of this litter. Such information is vital for developing effective management strategies for beach and marine litter at both regional and global levels. Copyright © 2017 Elsevier Ltd. All rights reserved.

  18. Improving the firm's environmental conduct

    DEFF Research Database (Denmark)

    Knudsen, Thorbjørn; Koed Madsen, Tage

    2001-01-01

    lead to strategic advantage and, thus, economic gains at the firm level. In view of the great importance of this claim, the purpose of the present article was to apply resource-based insights in order to develop this reasoning further and provide an empirical test of three hypotheses related...... to the claim. Our empirical test filled a gap in previous research and offered evidence in support of this claim as well as support for two related hypotheses developed on the basis of the resource-based view.......  It has recently been argued that growing societal pressures for better environmental conduct could induce environmental innovation, thereby entailing lower costs due to increased value and/or more efficient resource allocation. This has led to the claim that improved environmental conduct may...

  19. Knowledge management in the firm

    DEFF Research Database (Denmark)

    Rasmussen, Palle; Nielsen, Peter

    2011-01-01

    of the importance of deliberately combining various approaches to innovation and learning in order to include a maximum of actors as sources in building knowledge assets and strategies. Research limitations/implications – Most of the empirical examples are from private sector enterprises, even though......Purpose – The purpose of this paper is to set focus on, and discuss the concept of knowledge, and show how the interrelations between knowledge and other concepts, such as learning, have become a decisive element in managing human resources and firm performance. Design....../methodology/approach – The dimensions of knowledge management are identified and related to learning, organizational configurations, human resources management and institutional environments in order to identify and percent the most important approaches to knowledge management and the development over time. Findings – Creating...

  20. Temporal trend of litter contamination at Cassino beach, Southern Brazil

    OpenAIRE

    Tourinho, Paula da Silva; Fillmann,Gilberto

    2011-01-01

    This study was undertaken to evaluate temporal trends of litter contamination at Cassino beach, Southern Brazil. Surveys were conducted between 1994 and 1995, and then resumed from 2003 to 2006. Litter composition was related to both land- anmarinebased origins. However, seasonal and spatial variations showed that in situ deposition by beach users is still a major source of litter at Cassino beach. The results indicated a significant increase in litter amount at Cassino beach over the survey ...

  1. Tidal flow separation at protruding beach nourishments

    OpenAIRE

    Rademacher, Max; de Schipper, Matthieu A.; Swinkels, Cilia; MacMahan, Jamie H.; Reniers, Ad J.H.M.

    2017-01-01

    The article of record as published may be found at http://dx.doi.org/10.1002/2016JC011942 In recent years, the application of large-scale beach nourishments has been discussed, with the Sand Motor in the Netherlands as the first real-world example. Such protruding beach nourishments have an impact on tidal currents, potentially leading to tidal flow separation and the generation of tidal eddies of length scales larger than the nourishment itself. The present study examines the cha...

  2. Landscape Visual Quality and Meiofauna Biodiversity on Sandy Beaches

    Science.gov (United States)

    Felix, Gabriela; Marenzi, Rosemeri C.; Polette, Marcos; Netto, Sérgio A.

    2016-10-01

    Sandy beaches are central economic assets, attracting more recreational users than other coastal ecosystems. However, urbanization and landscape modification can compromise both the functional integrity and the attractiveness of beach ecosystems. Our study aimed at investigating the relationship between sandy beach artificialization and the landscape perception by the users, and between sandy beach visual attractiveness and biodiversity. We conducted visual and biodiversity assessments of urbanized and semiurbanized sandy beaches in Brazil and Uruguay. We specifically examined meiofauna as an indicator of biodiversity. We hypothesized that urbanization of sandy beaches results in a higher number of landscape detractors that negatively affect user evaluation, and that lower-rated beach units support lower levels of biodiversity. We found that urbanized beach units were rated lower than semiurbanized units, indicating that visual quality was sensitive to human interventions. Our expectations regarding the relationship between landscape perception and biodiversity were only partially met; only few structural and functional descriptors of meiofauna assemblages differed among classes of visual quality. However, lower-rated beach units exhibited signs of lower environmental quality, indicated by higher oligochaete densities and significant differences in meiofauna structure. We conclude that managing sandy beaches needs to advance beyond assessment of aesthetic parameters to also include the structure and function of beach ecosystems. Use of such supporting tools for managing sandy beaches is particularly important in view of sea level rise and increasing coastal development.

  3. Intertidal beach slope predictions compared to field data

    NARCIS (Netherlands)

    Madsen, A.J.; Plant, N.G.

    2001-01-01

    This paper presents a test of a very simple model for predicting beach slope changes. The model assumes that these changes are a function of both the incident wave conditions and the beach slope itself. Following other studies, we hypothesized that the beach slope evolves towards an equilibrium

  4. Tracer Studies In A Laboratory Beach Subjected To Waves

    Science.gov (United States)

    This work investigated the washout of dissolved nutrients from beaches due to waves by conducting tracer studies in a laboratory beach facility. The effects of waves were studied in the case where the beach was subjected to the tide, and that in which no tidal action was present...

  5. Tracer Studies In A Laboratory Beach Subjected To Waves

    Science.gov (United States)

    This work investigated the washout of dissolved nutrients from beaches due to waves by conducting tracer studies in a laboratory beach facility. The effects of waves were studied in the case where the beach was subjected to the tide, and that in which no tidal action was present...

  6. Beachwatch : The effect of daily morphodynamics on seasonal beach evolution

    NARCIS (Netherlands)

    Quartel, S.

    2007-01-01

    Not only the storm intensity, but also the capacity of the beach to recover during fair weather conditions, influences the erosion trends of beaches. Susanne Quartel concludes this in her thesis in which the daily changes of the intertidal beach of Noordwijk aan Zee, the Netherlands, are described.

  7. Macrofauna and meiofauna of two sandy beaches at Mombasa, Kenya

    Digital Repository Service at National Institute of Oceanography (India)

    Ansari, Z.A.; Ingole, B.S.; Parulekar, A.H.

    density was maximum around high water mark at the English point, a more sheltered beach On the more exposed Nyali Beach the meiofauna was aggregated downshore and highest numbers were recorded between mid and low water mark On both the beaches...

  8. Fine particle deposition at Vainguinim tourist beach, Goa, India

    Digital Repository Service at National Institute of Oceanography (India)

    Chandramohan, P.; Jayakumar, S.; SanilKumar, V.; Ilangovan, D.

    . The beach sediments consist primarily shell fragments and quartz, with heavy mineral composed of ilmenits, magnetite and manganese. The black stain of the fine-grained heavy minerals deposited on the beach face reduces the aesthetics of the beach. This paper...

  9. Intertidal beach slope predictions compared to field data

    NARCIS (Netherlands)

    Madsen, A.J.; Plant, N.G.

    2001-01-01

    This paper presents a test of a very simple model for predicting beach slope changes. The model assumes that these changes are a function of both the incident wave conditions and the beach slope itself. Following other studies, we hypothesized that the beach slope evolves towards an equilibrium valu

  10. The Effects of Firm Experience and Relational Resources on Firm Product Development Capabilities

    OpenAIRE

    2014-01-01

    Given that the focus of strategic management research is understanding the determinants of firm performance, and that product development capabilities have been shown to influence firm performance, the research question this dissertation attempts to answer is: what factors influence a firm's product development capabilities? Building on the resource based view and evolutionary theory, this dissertation proposes that firms leverage knowledge generating resources to modify or develop their rou...

  11. A Firm-Specific Analysis of the Exchange-Rate Exposure of Dutch Firms

    NARCIS (Netherlands)

    A. de Jong (Abe); J. Ligterink; V. Macrae

    2002-01-01

    textabstractWe examine the relationship between exchange-rate changes and stock returns for a sample of Dutch firms over 1994-1998. We find that over 50% of the firms are significantly exposed to exchange-rate risk. Furthermore, all firms with significant exchange-rate exposure benefit from a

  12. A firm-specific exposure analyis of the exchange-rate exposure of Dutch firms

    NARCIS (Netherlands)

    de Jong, A.; Ligterink, J.; Macrae, V.

    2006-01-01

    We examine the relationship between exchange-rate changes and stock returns for a sample of Dutch firms over 1994-1998. We find that over 50 per cent of the firms are significantly exposed to exchange-rate risk. Furthermore, all firms with significant exchange-rate exposure benefit from a

  13. A firm-specific exposure analyis of the exchange-rate exposure of Dutch firms

    NARCIS (Netherlands)

    de Jong, A.; Ligterink, J.; Macrae, V.

    2006-01-01

    We examine the relationship between exchange-rate changes and stock returns for a sample of Dutch firms over 1994-1998. We find that over 50 per cent of the firms are significantly exposed to exchange-rate risk. Furthermore, all firms with significant exchange-rate exposure benefit from a depreciati

  14. Antibody engineering and therapeutics, The Annual Meeting of the Antibody Society: December 8-12, 2013, Huntington Beach, CA.

    Science.gov (United States)

    Almagro, Juan Carlos; Gilliland, Gary L; Breden, Felix; Scott, Jamie K; Sok, Devin; Pauthner, Matthias; Reichert, Janice M; Helguera, Gustavo; Andrabi, Raiees; Mabry, Robert; Bléry, Mathieu; Voss, James E; Laurén, Juha; Abuqayyas, Lubna; Barghorn, Stefan; Ben-Jacob, Eshel; Crowe, James E; Huston, James S; Johnston, Stephen Albert; Krauland, Eric; Lund-Johansen, Fridtjof; Marasco, Wayne A; Parren, Paul W H I; Xu, Kai Y

    2014-01-01

    The 24th Antibody Engineering & Therapeutics meeting brought together a broad range of participants who were updated on the latest advances in antibody research and development. Organized by IBC Life Sciences, the gathering is the annual meeting of The Antibody Society, which serves as the scientific sponsor. Preconference workshops on 3D modeling and delineation of clonal lineages were featured, and the conference included sessions on a wide variety of topics relevant to researchers, including systems biology; antibody deep sequencing and repertoires; the effects of antibody gene variation and usage on antibody response; directed evolution; knowledge-based design; antibodies in a complex environment; polyreactive antibodies and polyspecificity; the interface between antibody therapy and cellular immunity in cancer; antibodies in cardiometabolic medicine; antibody pharmacokinetics, distribution and off-target toxicity; optimizing antibody formats for immunotherapy; polyclonals, oligoclonals and bispecifics; antibody discovery platforms; and antibody-drug conjugates.

  15. Foreign Entry and Heterogeneous Growth of Firms

    DEFF Research Database (Denmark)

    Deng, Paul Duo; Jefferson, Gary H.

    We adopt the framework of Schumpeterian creative destruction formalized by Aghion et al. (2009) to analyze the impact of foreign entry on the productivity growth of domestic firms. In the face of foreign entry, domestic firms exhibit heterogeneous patterns of growth depending on their technological...

  16. Identifying High Growth Firms in India

    DEFF Research Database (Denmark)

    Aggarwal, Aradhna; Sato, Takahiro

    Over the past two decades, considerable interest has grown in high growth firms (HGFs). However, the concept of HGFs still remains controversial. One of the most controversial issues is size and age of these firms. The present study argues that the current literature on HGFs may offer little help...

  17. Managing firm competitiveness in global markets

    NARCIS (Netherlands)

    Gehlhar, M.; Regmi, A.; Stefanou, S.E.; Zoumas, B.

    2006-01-01

    The globalization profile of US food firms is mixed. US sales from foreign direct investment is now over six times the level of exports, while US processed food trade balance has moved from +$9 billion in 1995 to - $7 billion in 2004. Competitive forces drive firms to seek new areas of growth, with

  18. Foreign Entry and Heterogeneous Growth of Firms

    DEFF Research Database (Denmark)

    Deng, Paul Duo; Jefferson, Gary H.

    We adopt the framework of Schumpeterian creative destruction formalized by Aghion et al. (2009) to analyze the impact of foreign entry on the productivity growth of domestic firms. In the face of foreign entry, domestic firms exhibit heterogeneous patterns of growth depending on their technologic...

  19. Trade credit: Elusive insurance of firm growth

    NARCIS (Netherlands)

    Bams, Dennis; Bos, Jaap; Pisa, Magdalena

    2016-01-01

    Firms depend heavily on trade credit. This paper introduces a trade credit network into a structural model of the economy. In an empirical analysis of the model, we find that trade credit is an elusive insurance: as long as a firm is financially unconstrained and times are good, more trade credit en

  20. Agency Costs, Firm Value, and Corporate Investment

    NARCIS (Netherlands)

    M. Teixeira de Vasconcelos (Manuel)

    2012-01-01

    textabstractOften firms lack the necessary internal resources to pursue all profitable investment opportunities at their disposal. One of the most important roles of financial markets is to allocate resources from different economic agents to the firms that will better employ them, thereby enabling

  1. Impacts of IST on firms and organisations

    DEFF Research Database (Denmark)

    Jørgensen, Ulrik

    2003-01-01

    A review of socio-economic research on the impact and role of IST in firms and organisations. The study is carried out in ESTO for IPTS.......A review of socio-economic research on the impact and role of IST in firms and organisations. The study is carried out in ESTO for IPTS....

  2. Trade credit: Elusive insurance of firm growth

    NARCIS (Netherlands)

    Bams, Dennis; Bos, Jaap; Pisa, Magdalena

    2016-01-01

    Firms depend heavily on trade credit. This paper introduces a trade credit network into a structural model of the economy. In an empirical analysis of the model, we find that trade credit is an elusive insurance: as long as a firm is financially unconstrained and times are good, more trade credit

  3. Agency Costs, Firm Value, and Corporate Investment

    NARCIS (Netherlands)

    M. Teixeira de Vasconcelos (Manuel)

    2012-01-01

    textabstractOften firms lack the necessary internal resources to pursue all profitable investment opportunities at their disposal. One of the most important roles of financial markets is to allocate resources from different economic agents to the firms that will better employ them, thereby enabling

  4. Electronic Commerce, Digital Information, and the Firm.

    Science.gov (United States)

    Rosenbaum, Howard

    2000-01-01

    Discussion of the social context of electronic commerce (ecommerce) focuses on information imperatives, or rules that are critical for ecommerce firms. Concludes with a discussion of the organizational changes that can be expected to accompany the incorporation of these imperatives into the mission and core business processes of ecommerce firms.…

  5. Architectural firms: workforce, business strategy and performance

    Directory of Open Access Journals (Sweden)

    Adedapo Adewunmi Oluwatayo

    2011-12-01

    Full Text Available The intent of this research was to investigate the relationship between the workforce, business strategy and performance of architectural firms. Data was collected from 92 firms randomly selected from the cities where architectural firms were most concentrated in Nigeria using questionnaires. Hierarchical regression analysis was carried out to investigate the direct and indirect impacts of the workforce of architectural firms on their performance. The findings confirm the significant positive impact. With business strategy controlled, the specific characteristics of the workforce and its management which influenced performance were the number of architects, the work structure, and the age and experience of the principal partners. The impact of the number of non- architecture professionals and staff participation in decision-making on performance was moderated by the business strategy adopted by the firms. The results suggest that workforce characteristics are more important than the management of the workforce in determining performance of architectural firms. This is contrary to the results of previous studies which suggest higher importance of the management. This probably indicates the peculiarity of architectural firm as a professional service firm in the construction industry.

  6. Tax Compliance over the Firm Life Course

    NARCIS (Netherlands)

    Stam, F.C.; Verbeeten, F.H.M.

    2017-01-01

    This article provides a new model of tax compliance over the firm life course, focusing on the dynamics in the underlying motivations and capacities for tax compliance. We review and structure the relevant literature on the early life course of firms: the traditional stages of growth models and a le

  7. Market Imperfections and Firm-Sponsored Training

    NARCIS (Netherlands)

    Picchio, M.; van Ours, J.C.

    2010-01-01

    Recent human capital theories predict that labor market frictions and product market competition influence firm-sponsored training. Using matched worker-firm data from Dutch manufacturing, our paper empirically assesses the validity of these predictions. We find that a decrease in labor market frict

  8. New workplace practices and firm performance:

    DEFF Research Database (Denmark)

    Cristini, Annalisa; Pozzoli, Dario

    that the adoption of innovation practices has spread substantially more across the British manufacturing firms than across the Italian ones; however our results also indicate that the practices' association with the firms' VA is much lower in Britain than in Italy. The counterfactual analysis shows that had...

  9. Internationalization of Emerging Economies and Firms

    DEFF Research Database (Denmark)

    countries and firms. The impact of country and firm specific factors, the role of institutions and governments, the strive for compensation of initial disadvantages, the struggle for finding ways to counterbalance for late coming into the international arena in the process of internationalization...... are brought under scrutiny in this volume. The chapters provide insights from diverse contexts ranging from Brazil to Malaysia....

  10. Psychic Distance, Innovation, and Firm Performance

    NARCIS (Netherlands)

    Azar, Goudarz; Drogendijk, Rian

    2014-01-01

    Previous research suggests that internationalization improves a firm's ability to innovate, but the effect of internationalizing into specific target markets or destinations on the innovation ability of firms has not been fully investigated. This study examined whether the psychic distance between t

  11. Electronic Commerce, Digital Information, and the Firm.

    Science.gov (United States)

    Rosenbaum, Howard

    2000-01-01

    Discussion of the social context of electronic commerce (ecommerce) focuses on information imperatives, or rules that are critical for ecommerce firms. Concludes with a discussion of the organizational changes that can be expected to accompany the incorporation of these imperatives into the mission and core business processes of ecommerce firms.…

  12. Strategic Resources and Family Firm Performance

    NARCIS (Netherlands)

    Matser, I.A.

    2013-01-01

    Most companies in the Netherlands can be labeled as family firms (according to the GEEF definition (Mandle, 2008; Flören et al., 2010). The family firm can be regarded as an open system model comprising three overlapping, interacting, and interdependent subsystems: owners, family, and managers (Moor

  13. Knowledge Sharing in Knowledge-Intensive Firms

    DEFF Research Database (Denmark)

    Gupta, Akshey; Michailova, Snejina

    2004-01-01

    This paper is a study of the knowledge-sharing difficulties experienced by three departments in a knowledge-intensive firm. The case organization is a global consulting firm that has been on the forefront of knowledge management and has won several knowledge management related international accla...

  14. Trade credit: Elusive insurance of firm growth

    NARCIS (Netherlands)

    Bams, Dennis; Bos, Jaap; Pisa, Magdalena

    2016-01-01

    Firms depend heavily on trade credit. This paper introduces a trade credit network into a structural model of the economy. In an empirical analysis of the model, we find that trade credit is an elusive insurance: as long as a firm is financially unconstrained and times are good, more trade credit en

  15. Market Imperfections and Firm-Sponsored Training

    NARCIS (Netherlands)

    Picchio, M.; van Ours, J.C.

    2010-01-01

    Recent human capital theories predict that labor market frictions and product market competition influence firm-sponsored training. Using matched worker-firm data from Dutch manufacturing, our paper empirically assesses the validity of these predictions. We find that a decrease in labor market frict

  16. A new approach to firm evaluation

    NARCIS (Netherlands)

    E.M. Vermeulen (Erik); J. Spronk (Jaap); D. van der Wijst (Nico)

    1993-01-01

    textabstractIn this paper, a method is developed to evaluate firms on the basis of the risks they face. In accordance with the multi-factor method, risk is represented as a vector of sensitivities to unexpected changes of risk factors. Subsequently, the sensitivities themselves are related to firm c

  17. Managing firm competitiveness in global markets

    NARCIS (Netherlands)

    Gehlhar, M.; Regmi, A.; Stefanou, S.E.; Zoumas, B.

    2006-01-01

    The globalization profile of US food firms is mixed. US sales from foreign direct investment is now over six times the level of exports, while US processed food trade balance has moved from +$9 billion in 1995 to - $7 billion in 2004. Competitive forces drive firms to seek new areas of growth, with

  18. Bibliography of sandy beaches and sandy beach organisms on the African continent

    CSIR Research Space (South Africa)

    Bally, R

    1986-01-01

    Full Text Available This bibliography covers the literature relating to sandy beaches on the African continent and outlying islands. The bibliography lists biological, chemical, geographical and geological references and covers shallow marine sediments, surf zones off...

  19. 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...

  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. Abnormalities in the tricarboxylic Acid cycle in Huntington disease and in a Huntington disease mouse model.

    Science.gov (United States)

    Naseri, Nima N; Xu, Hui; Bonica, Joseph; Vonsattel, Jean Paul G; Cortes, Etty P; Park, Larry C; Arjomand, Jamshid; Gibson, Gary E

    2015-06-01

    Glucose metabolism is reduced in the brains of patients with Huntington disease (HD). The mechanisms underlying this deficit, its link to the pathology of the disease, and the vulnerability of the striatum in HD remain unknown. Abnormalities in some of the key mitochondrial enzymes involved in glucose metabolism, including the pyruvate dehydrogenase complex (PDHC) and the tricarboxylic acid (TCA) cycle, may contribute to these deficits. Here, activities for these enzymes and select protein levels were measured in human postmortem cortex and in striatum and cortex of an HD mouse model (Q175); mRNA levels encoding for these enzymes were also measured in the Q175 mouse cortex. The activities of PDHC and nearly all of the TCA cycle enzymes were dramatically lower (-50% to 90%) in humans than in mice. The activity of succinate dehydrogenase increased with HD in human (35%) and mouse (23%) cortex. No other changes were detected in the human HD cortex or mouse striatum. In Q175 cortex, there were increased activities of PDHC (+12%) and aconitase (+32%). Increased mRNA levels for succinyl thiokinase (+88%) and isocitrate dehydrogenase (+64%) suggested an upregulation of the TCA cycle. These patterns of change differ from those reported in other diseases, which may offer unique metabolic therapeutic opportunities for HD patients.

  2. An Aspirational Community Theory of the Firm

    DEFF Research Database (Denmark)

    Li, Xin

    contractual labor and aspirational labor, the former being defined as services provided by a laborer in fulfillment of a contract while the latter as services provided by a laborer in pursuit of an aspiration; and argue that firms exist because while the market may be effective and efficient for allocating...... propose an aspirational community theory of the firm (ACT) as a candidate theory by conceptualizing the firm as an aspirational community, the core of which is a group of like-minded people sharing similar or same aspiration/vision. To explain the existence of the firm, we make a distinction between...... contractual labor it is less effective and efficient than the firms for allocating aspirational labor....

  3. Choice of Ownership Structure and Firm Performance

    DEFF Research Database (Denmark)

    Jones, Derek C; Kalmi, Panu; Mygind, Niels

    2003-01-01

    In this paper we use rich panel data for a representative sample of Estonian enterprises to analysediverse issues related to the determinants of ownership structures and ownership changes afterprivatisation. A key focus is to determine whether ownership changes are related to economicefficiency....... While employee owned firms are found to be much more prone than other firms toswitch ownership categories, often `employee owned' firms remain `insider-owned' as ownershippasses from current employees to managers and former employees. Logit analyses of thedeterminants of ownership structures...... and ownership changes provides mixed support for severalhypotheses. As predicted: (i) wealth and resource constraints play a crucial role in thedetermination of ownership, with foreigners buying firms with the highest equity levels andinsiders buying firms with the lowest equity valuations; (ii) risk aversion...

  4. Commercialization of Innovations and Firm Performance

    DEFF Research Database (Denmark)

    Madsen, Erik Strøjer; Smith, Valdemar

    The decision on investment in R&D is very important and highly risky for firms' performance and survival in their business. This paper focuses on the commercialization of firms' investment in innovation and how these decisions affect their performance. The study uses a large innovation survey...... of Danish firms holding information on their R&D investment as well as their expenses on marketing which gives knowledge of the commercialization of their innovations. The results suggest a highly significant commercialization of innovation, which means that firms' follows a strategy of investment both...... in innovation and marketing making R&D and marketing complements in their investment decision. However, there is only week evidence for the hypothesis that commercialization of innovations also creates more value for the firms in the short run.  ...

  5. Commercialization of Innovations and Firm Performance

    DEFF Research Database (Denmark)

    Madsen, Erik Strøjer; Smith, Valdemar

    The decision on investment in R&D is very important and highly risky for firms' performance and survival in their business. This paper focuses on the commercialization of firms' investment in innovation and how these decisions affect their performance. The study uses a large innovation survey...... of Danish firms holding information on their R&D investment as well as their expenses on marketing which gives knowledge of the commercialization of their innovations. The results suggest a highly significant commercialization of innovation, which means that firms' follows a strategy of investment both...... in innovation and marketing making R&D and marketing complements in their investment decision. However, there is only week evidence for the hypothesis that commercialization of innovations also creates more value for the firms in the short run....

  6. How does firm performance influence market orientation?

    DEFF Research Database (Denmark)

    Sørensen, Hans Eibe; Stieglitz, Nils

    This paper contributes by investigating how firm performance influences its market orientation. We draw on the aspiration-level model from the behavioral theory of the firm to develop testable propositions that substantiate and extend prior market orientation research. Specifically, we address ho...... performance influences firms' market-oriented search behavior (responsive or proactive) and the allocation of attention (customer and competitor orientation) as well as the formation of aspiration levels. Research and managerial implications are discussed.......This paper contributes by investigating how firm performance influences its market orientation. We draw on the aspiration-level model from the behavioral theory of the firm to develop testable propositions that substantiate and extend prior market orientation research. Specifically, we address how...

  7. Effect of firm variables on patent price

    Directory of Open Access Journals (Sweden)

    Shyam Sreekumaran Nair

    2012-03-01

    Full Text Available In this study, using singleton patent auction price data from Ocean Tomo, LLC, we analyse the effect of firm variables on patent price. Patents owned by small firms attract higher price than patents owned by large firms, if they engage in multi-country filings. The patents owned by small firms get cited more than the patents owned by large firms. The patents owned by individual inventors attract a higher price than the patents owned by organisations when multi-country filings are not included. We believe that the lack of resources is preventing individual inventors from engaging in multi-country filings and maximising the revenue from their invention. A larger representative data should be used to replicate the results before generalising it.

  8. Pluripotent hybrid stem cells from transgenic Huntington's disease monkey.

    Science.gov (United States)

    Laowtammathron, Chuti; Chan, Anthony W S

    2013-01-01

    Huntington's disease (HD) is a devastating disease that currently has no cure. Transgenic HD monkeys have developed key neuropathological and cognitive behavioral impairments similar to HD patients. Thus, pluripotent stem cells derived from transgenic HD monkeys could be a useful comparative model for clarifying HD pathogenesis and developing novel therapeutic approaches, which could be validated in HD monkeys. In order to create personal pluripotent stem cells from HD monkeys, here we present a tetraploid technique for deriving pluripotent hybrid HD monkey stem cells.

  9. Striatal degeneration impairs language learning: evidence from Huntington's disease.

    Science.gov (United States)

    De Diego-Balaguer, R; Couette, M; Dolbeau, G; Dürr, A; Youssov, K; Bachoud-Lévi, A-C

    2008-11-01

    Although the role of the striatum in language processing is still largely unclear, a number of recent proposals have outlined its specific contribution. Different studies report evidence converging to a picture where the striatum may be involved in those aspects of rule-application requiring non-automatized behaviour. This is the main characteristic of the earliest phases of language acquisition that require the online detection of distant dependencies and the creation of syntactic categories by means of rule learning. Learning of sequences and categorization processes in non-language domains has been known to require striatal recruitment. Thus, we hypothesized that the striatum should play a prominent role in the extraction of rules in learning a language. We studied 13 pre-symptomatic gene-carriers and 22 early stage patients of Huntington's disease (pre-HD), both characterized by a progressive degeneration of the striatum and 21 late stage patients Huntington's disease (18 stage II, two stage III and one stage IV) where cortical degeneration accompanies striatal degeneration. When presented with a simplified artificial language where words and rules could be extracted, early stage Huntington's disease patients (stage I) were impaired in the learning test, demonstrating a greater impairment in rule than word learning compared to the 20 age- and education-matched controls. Huntington's disease patients at later stages were impaired both on word and rule learning. While spared in their overall performance, gene-carriers having learned a set of abstract artificial language rules were then impaired in the transfer of those rules to similar artificial language structures. The correlation analyses among several neuropsychological tests assessing executive function showed that rule learning correlated with tests requiring working memory and attentional control, while word learning correlated with a test involving episodic memory. These learning impairments significantly

  10. Long-term outcome of presymptomatic testing in Huntington disease

    OpenAIRE

    Gargiulo, Marcela; Lejeune, Séverine; Tanguy, Marie-Laure; Lahlou-Laforêt, Khadija; Faudet, Anne; Cohen, David; Feingold, Josué; Durr, Alexandra

    2008-01-01

    Our study on long-term outcome of presymptomatic testing for Huntington disease had two aims: the comparison of the psychological well-being and social adjustment of carriers and non-carriers of the mutation, and the identification of psychological determinants to improve care/support of testees. We performed a cross-sectional study of 351 persons who underwent presymptomatic testing. Those who had motor signs were excluded from the comparison of asymptomatic carrier and non-carriers. A struc...

  11. Morphological changes of the beaches of Goa

    Digital Repository Service at National Institute of Oceanography (India)

    Murty, C.S.; Veerayya, M.; Varadachari, V.V.R.

    with the onset of the southwest monsoon wind and wave conditions followed by slower rates during the subsequent period of the monsoon. This continues till August when the beaches have minimum sediment storage. The wave climates during the postmonsoon and winter...

  12. Coastal erosion project, Diani beach, Kenya

    NARCIS (Netherlands)

    Ballot, J.; Hoyng, C.; Kateman, I.; Smits, M.; De Winter, R.

    2006-01-01

    Master project report. Since the seventies, the establishment of hotels and other facilities has increased the pressure on the Kenyan coast. During the last decade, hotel managers and residents in Diani Beach have been experiencing problems with erosion. The only measures taken to address the

  13. The ecology of sandy beaches in Natal

    African Journals Online (AJOL)

    estuary(32°25' 45"E128° 15'35"S),Blythdale (31 ° 16'20"EI. 29° 16'25"S) ... Bentho-planktonic mysids were collected with a sledge which skims off ... Along each transect a series of .... times more carbonate than the southern beaches. At So-.

  14. Coastal erosion project, Diani beach, Kenya

    NARCIS (Netherlands)

    Ballot, J.; Hoyng, C.; Kateman, I.; Smits, M.; De Winter, R.

    2006-01-01

    Master project report. Since the seventies, the establishment of hotels and other facilities has increased the pressure on the Kenyan coast. During the last decade, hotel managers and residents in Diani Beach have been experiencing problems with erosion. The only measures taken to address the proble

  15. Alongshore variability of nourished and natural beaches

    NARCIS (Netherlands)

    De Schipper, M.A.

    2014-01-01

    Alongshore variability in topography (i.e. height differences in bed level along the coast) can exist on both natural and nourished beaches. An important question prior to implementation of a nourishment project is how alongshore variability is going to evolve and, related to this variability, the e

  16. Interstitial meiofauna of Namib sandy beaches

    African Journals Online (AJOL)

    1988-03-16

    Mar 16, 1988 ... sandy beaches on the Namibian coast, Langstrand and Cape Cross. A transverse ... prominent in the mid-shore at Cape Cross but occurred in low numbers at Langstrand , where archiannelids ... Koop (1983) recorded the faunal composition of local .... four replicate sediment cores were taken at 15 cm.

  17. Cross-Shore Exchange on Natural Beaches

    Science.gov (United States)

    2014-09-01

    public health cost estimates of contaminated coastal waters: A case study of gastroenteritis at Southern California beaches. Environ. Sci. Technol., 40...Jones, J. Svejkovsky, G. V. Leipzig, and A. Brown, 2001: Generation of enterococci bacteria in a coastal saltwater marsh and its impact on surf zone

  18. The structural involvement of the cingulate cortex in premanifest and early Huntington's disease.

    Science.gov (United States)

    Hobbs, Nicola Z; Pedrick, Amy V; Say, Miranda J; Frost, Chris; Dar Santos, Rachelle; Coleman, Allison; Sturrock, Aaron; Craufurd, David; Stout, Julie C; Leavitt, Blair R; Barnes, Josephine; Tabrizi, Sarah J; Scahill, Rachael I

    2011-08-01

    The impact of Huntington's disease neuropathology on the structure of the cingulate is uncertain, with evidence of both cortical enlargement and atrophy in this structure in early clinical disease. We sought to determine differences in cingulate volume between premanifest Huntington's disease and early Huntington's disease groups compared with controls using detailed manual measurements. Thirty controls, 30 subjects with premanifest Huntington's disease, and 30 subjects with early Huntington's disease were selected from the Vancouver site of the TRACK-HD study. Subjects underwent 3 Tesla magnetic resonance imaging and motor, cognitive, and neuropsychiatric assessment. The cingulate was manually delineated and subdivided into rostral, caudal, and posterior segments. Group differences in volume and associations with performance on 4 tasks thought to utilize cingulate function were examined, with adjustment for appropriate covariates. Cingulate volumes were, on average, 1.7 mL smaller in early Huntington's disease (P=.001) and 0.9 mL smaller in premanifest Huntington's disease (P=.1) compared with controls. Smaller volumes in subsections of the cingulate were associated with impaired recognition of negative emotions (P=.04), heightened depression (P=.009), and worse visual working memory performance (P=.01). There was no evidence of associations between volume and ability on a performance-monitoring task. This study disputes previous findings of enlargement of the cingulate cortex in Huntington's disease and instead suggests that the cingulate undergoes structural degeneration during early Huntington's disease with directionally consistent, nonsignificant differences seen in premanifest Huntington's disease. Cingulate atrophy may contribute to deficits in mood, emotional processing, and visual working memory in Huntington's disease.

  19. Preimplantation genetic diagnosis for Huntington's disease with exclusion testing.

    Science.gov (United States)

    Sermon, Karen; De Rijcke, Martine; Lissens, Willy; De Vos, Anick; Platteau, Peter; Bonduelle, Maryse; Devroey, Paul; Van Steirteghem, André; Liebaers, Inge

    2002-10-01

    Huntington's disease is an autosomal dominant, late-onset disorder, for which the gene and the causative mutation have been known since 1993. Some at-risk patients choose for presymptomatic testing and can make reproductive choices accordingly. Others however, prefer not to know their carrier status, but may still wish to prevent the birth of a carrier child. For these patients, exclusion testing after prenatal sampling has been an option for many years. A disadvantage of this test is that unaffected pregnancies may be terminated if the parent at risk (50%) has not inherited the grandparental Huntington gene, leading to serious moral and ethical objections. As an alternative, preimplantation genetic diagnosis (PGD) on embryos obtained in vitro may be proposed, after which only embryos free of risk are replaced. Embryos can then be selected, either by the amplification of the CAG repeat in the embryos without communicating results to the patients (ie non-disclosure testing), which brings its own practical and moral problems, or exclusion testing. We describe here the first PGD cycles for exclusion testing for Huntington's disease in five couples. Three couples have had at least one PGD cycle so far. One pregnancy ensued and a healthy female baby was delivered.

  20. iPSC-based drug screening for Huntington's disease.

    Science.gov (United States)

    Zhang, Ningzhe; Bailus, Barbara J; Ring, Karen L; Ellerby, Lisa M

    2016-05-01

    Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, caused by an expansion of the CAG repeat in exon 1 of the huntingtin gene. The disease generally manifests in middle age with both physical and mental symptoms. There are no effective treatments or cures and death usually occurs 10-20 years after initial symptoms. Since the original identification of the Huntington disease associated gene, in 1993, a variety of models have been created and used to advance our understanding of HD. The most recent advances have utilized stem cell models derived from HD-patient induced pluripotent stem cells (iPSCs) offering a variety of screening and model options that were not previously available. The discovery and advancement of technology to make human iPSCs has allowed for a more thorough characterization of human HD on a cellular and developmental level. The interaction between the genome editing and the stem cell fields promises to further expand the variety of HD cellular models available for researchers. In this review, we will discuss the history of Huntington's disease models, common screening assays, currently available models and future directions for modeling HD using iPSCs-derived from HD patients. This article is part of a Special Issue entitled SI: PSC and the brain. Copyright © 2015 Elsevier B.V. All rights reserved.

  1. Informativeness of Early Huntington Disease Signs about Gene Status.

    Science.gov (United States)

    Oster, Emily; Eberly, Shirley W; Dorsey, E Ray; Kayson-Rubin, Elise; Oakes, David; Shoulson, Ira

    2015-01-01

    The cohort-level risk of Huntington disease (HD) is related to the age and symptom level of the cohort, but this relationship has not been made precise. To predict the evolving likelihood of carrying the Huntington disease (HD) gene for at-risk adults using age and sign level. Using data from adults with early signs and symptoms of HD linked to information on genetic status, we use Bayes' theorem to calculate the probability that an undiagnosed individual of a certain age and sign level has an expanded CAG repeat. Both age and sign levels have substantial influence on the likelihood of HD onset, and the probability of eventual diagnosis changes as those at risk age and exhibit (or fail to exhibit) symptoms. For example, our data suggest that in a cohort of individuals age 26 with a Unified Huntington's Disease Rating Scale (UHDRS) motor score of 7-10 70% of them will carry the HD mutation. For individuals age 56, the same motor score suggests only a 40% chance of carrying the mutation. Early motor signs of HD, overall and the chorea subscore, were highly predictive of disease onset at any age. However, body mass index (BMI) and cognitive performance scores were not as highly predictive. These results suggest that if researchers or clinicians are looking for early clues of HD, it may be more foretelling to look at motor rather than cognitive signs. Application of similar approaches could be used with other adult-onset genetic conditions.

  2. Contribution of imaging studies and neuro physiologic investigations to the diagnosis of Huntington`s chorea; L`imagerie medicale et les explorations neuro-physiologiques dans le diagnostic de la choree de Huntington

    Energy Technology Data Exchange (ETDEWEB)

    Paquet, J.M.; Turpin, J.CI. [Centre Hospitalier Universitaire, 51 - Reims (France)

    1997-05-01

    Although Huntington`s disease was described in 1872, its diagnosis continues to rest on clinical grounds. Recently developed techniques for imaging the brain (computed tomography and magnetic resonance imaging) or studying its function (single photon emission computed tomography and positron emission tomography) have demonstrated only non specific abnormalities at the early stages of the disease, thus failing to improve the pre-symptomatic diagnosis. Neuro-physiologic investigations (evoked potentials, electromyogram, electroencephalogram) have been similarly unrewarding. Investigations are useful only as an laid to the differential diagnosis. Molecular biology technology is the only available tool for identifying high-risk individuals and establishing a definitive diagnosis of Huntington`s disease. (authors). 10 refs.

  3. Trophic niche shifts driven by phytoplankton in sandy beach ecosystems

    Science.gov (United States)

    Bergamino, Leandro; Martínez, Ana; Han, Eunah; Lercari, Diego; Defeo, Omar

    2016-10-01

    Stable isotopes (δ13C and δ15N) together with chlorophyll a and densities of surf diatoms were used to analyze changes in trophic niches of species in two sandy beaches of Uruguay with contrasting morphodynamics (i.e. dissipative vs. reflective). Consumers and food sources were collected over four seasons, including sediment organic matter (SOM), suspended particulate organic matter (POM) and the surf zone diatom Asterionellopsis guyunusae. Circular statistics and a Bayesian isotope mixing model were used to quantify food web differences between beaches. Consumers changed their trophic niche between beaches in the same direction of the food web space towards higher reliance on surf diatoms in the dissipative beach. Mixing models indicated that A. guyunusae was the primary nutrition source for suspension feeders in the dissipative beach, explaining their change in dietary niche compared to the reflective beach where the proportional contribution of surf diatoms was low. The high C/N ratios in A. guyunusae indicated its high nutritional value and N content, and may help to explain the high assimilation by suspension feeders at the dissipative beach. Furthermore, density of A. guyunusae was higher in the dissipative than in the reflective beach, and cell density was positively correlated with chlorophyll a only in the dissipative beach. Therefore, surf diatoms are important drivers in the dynamics of sandy beach food webs, determining the trophic niche space and productivity. Our study provides valuable insights on shifting foraging behavior by beach fauna in response to changes in resource availability.

  4. Knowledge Management Systems: A Comparison of Law Firms and Consulting Firms

    Directory of Open Access Journals (Sweden)

    Petter Gottschalk

    2000-01-01

    Full Text Available This paper reports results from a survey of Norwegian law firms on the use of information technology to support inter-organizational knowledge management. Two predictors of IT support were significant: firm cooperation and knowledge cooperation. Inter-organizational trust was not a significant predictor. Software and systems most frequently used include word processing, electronic mail and legal databases. It has been argued that law firms are old fashioned and not ready for extensive use of information technology. An impression is created that other professional service firms such as consulting firms are much more advanced. To evaluate the relative performance of law firms in the area of IT support for knowledge trans-fer an identical survey was conducted among a limited number of consulting firms in Norway. Survey results indicate that the average IT use in consulting firms was slightly higher than in law firms, but the most interesting differences were found in the different systems and software used rather than the level of IT use. Consulting firms are high-level users of general information sources on the Internet, while law firms are high-level users of structured information in databases.

  5. The effect of beach slope on tidal influenced saltwater intrusion

    Science.gov (United States)

    Zhao, Z.; Shen, C.; Jin, G.; Xin, P.; Hua, G.; Tao, X.; Zhao, J.

    2015-12-01

    Beach slope changes the tidal induced saltwater-freshwater circulations in coastal aquifers. However, the effect of beach slope on tidal influenced saltwater-freshwater mixing process is far from understood. Based on sand flume experiments and numerical simulations, we investigated the intrusion process of saltwater into freshwater under tidal forcing and variable beach slopes. The sand flume experiment results show that milder slope induces larger upper saline plume (USP) and seaward salt wedge interface (SWI) under tidal forcing. While, the steady state SWI keeps stagnant with different beach slopes. Consistent with the previous research, our numerical simulations also show a lager flux exchange across the milder beach induced by the tidal fluctuations. The groundwater table fluctuates more intensify with deeper beach slope. The next step of our study will pay attention to the effect of beach slope on the instability of USP which induces the salt-fingering flow.

  6. Job Creation by Firms in Denmark

    DEFF Research Database (Denmark)

    Ibsen, Rikke Falkner; Westergård-Nielsen, Niels

    (firm) is the legal entity. A company can have many sites, and one of the ways companies can grow is by expanding with multiple sites. This can happen by mergers and acquisitions but can also happen by creating "daughter workplaces". It is therefore essential to look at workplaces and firms at the same...... time. A complication here is that firms switch ID over time because of change of ownership, mergers and divisions. Data must be corrected so that these administrative issues will not affect the survival of firms. The data are used in a way where we can cover firm birth and firm death, spin......-offs and mergers. The analysis will make it possible to differentiate between net and gross creation of jobs because we can follow each single individual in and out of jobs. We have for Denmark found that size on its own does not have a big impact, but young firms are much more likely to contribute to a positive...

  7. Mexican Americans and the American Nation: A Response to Professor Huntington

    Science.gov (United States)

    Telles, Edward

    2006-01-01

    This essay is based on a talk I delivered at Texas A&M University on December 10, 2005, in response to an earlier lecture at the university by Professor Samuel P. Huntington. It relies on social science evidence to first address Huntington's contention that Mexicans are overwhelming American borders. It then turns to evidence that Mexican…

  8. PROBLEMS OF MODERNIZATION IN THE WORKS OF S. HUNTINGTON: THEORY AND PRACTICE

    Directory of Open Access Journals (Sweden)

    Britikova E. A.

    2016-06-01

    Full Text Available The article discusses the interpretation of the mechanisms of modernization of the American scientist - Samuel Huntington, which sees modernization as a complex process with a very uncertain result. As a representative of the multilinear approach, Samuel Huntington proves the uniqueness of the modernization paths of each individual national system

  9. 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

    2004-01-01

    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…

  10. Views of CEOs on Firm Performance

    Directory of Open Access Journals (Sweden)

    Dario Berginc

    2014-11-01

    Full Text Available Researching the managerial perspective of performance can contribute to a better understanding of firm performance, and offer a valuable contribution to research on objective performance measurement. The aim of this article is to examine how managers evaluate firm performance and which factors, in their opinion, have the biggest influence on performance. We particularly wanted to investigate the influence of top management and owners on firm performance. We conducted a qualitative study among the CEOs of some of the most successful Slovenian firms. The results show that CEOs connect positive performance with long-term growth and satisfying the needs of key stakeholders (employees, customers and owners, but short-term positive financial performance represents the basic foundation. The second most important factor is a united top management, capable of generating new ideas and acting as a role model to employees. This can lead to a higher degree of engagement by employees and a better understanding of the firm's goals. Owners can contribute to a positive firm performance with their active, strategic and long-term orientation, and with their ability to set clear goals and to trust top management in the long run. Another important factor that influences firm performance is the ownership structure. In the opinion of CEOs, dominant and private ownership has a more positive impact on firm performance than dispersed and state ownership. The present research findings provide several examples of good and bad practice, and highlight opportunities for further research, from an in-depth study of individual factors of firm performance to the search for a more comprehensive model of performance factors, based on a larger sample of CEOs and other managers.

  11. Huntington disease and Huntington disease-like in a case series from Brazil.

    Science.gov (United States)

    Castilhos, R M; Souza, A F D; Furtado, G V; Gheno, T C; Silva, A L; Vargas, F R; Lima, M-A F D; Barsottini, O; Pedroso, J L; Godeiro, C; Salarini, D; Pereira, E T; Lin, K; Toralles, M-B; Saute, J A M; Rieder, C R; Quintas, M; Sequeiros, J; Alonso, I; Saraiva-Pereira, M L; Jardim, L B

    2014-10-01

    The aim of this study was to identify the relative frequency of Huntington's disease (HD) and HD-like (HDL) disorders HDL1, HDL2, spinocerebellar ataxia type 2 (SCA2), SCA17, dentatorubral-pallidoluysian degeneration (DRPLA), benign hereditary chorea, neuroferritinopathy and chorea-acanthocytosis (CHAC), in a series of Brazilian families. Patients were recruited in seven centers if they or their relatives presented at least chorea, besides other findings. Molecular studies of HTT, ATXN2, TBP, ATN1, JPH3, FTL, NKX2-1/TITF1 and VPS13A genes were performed. A total of 104 families were ascertained from 2001 to 2012: 71 families from South, 25 from Southeast and 8 from Northeast Brazil. There were 93 HD, 4 HDL2 and 1 SCA2 families. Eleven of 104 index cases did not have a family history: 10 with HD. Clinical characteristics were similar between HD and non-HD cases. In HD, the median expanded (CAG)n (range) was 44 (40-81) units; R(2) between expanded HTT and age-at-onset (AO) was 0.55 (p=0.0001, Pearson). HDL2 was found in Rio de Janeiro (2 of 9 families) and Rio Grande do Sul states (2 of 68 families). We detected HD in 89.4%, HDL2 in 3.8% and SCA2 in 1% of 104 Brazilian families. There were no cases of HDL1, SCA17, DRPLA, neuroferritinopathy, benign hereditary chorea or CHAC. Only six families (5.8%) remained without diagnosis.

  12. Managing the Knowledge-Intensive Firm

    CERN Document Server

    Ejler, Nicolaj; Czerniawska, Fiona

    2011-01-01

    Over the last decade, there has been a substantial rise in the number of knowledge-intensive firms. The core assets of these businesses are the people themselves and so, successful managers of knowledge-intensive firms must inspire their employees and target their individual ambitions, to ensure high performance.This book examines what sets knowledge-intensive firms apart from other types of organizations, and the resultant organizational and strategic differences in business models, talent management, and client-handling approaches. A new model for transforming the management of knowledge-int

  13. The innovative behaviour of tourism firms

    DEFF Research Database (Denmark)

    Sundbo, Jon; Orfila-Sintes, Francina; Sørensen, Flemming

    2007-01-01

    are investigated by analysing quantitative as well as qualitative data comparing Spain and Denmark. A taxonomy of tourism firms is suggested and the firms’ characteristics which influence their innovativeness are presented. Additionally, the role of innovation networks is discussed, as is the role of innovation......Tourism firms operate in a competitive sector where innovating is often a condition for survival. This article presents a theoretical framework for understanding tourist firms’ innovative behaviour and innovation systems in tourism. The innovativeness of tourism firms and its determinants...

  14. Does Labor Diversity Affect Firm Performance?

    DEFF Research Database (Denmark)

    Pytlikova, Mariola; Pozzoli, Dario; Parrotta, Pierpaolo

    Using an employer-employee dataset, we analyze how diversity in cultural background, skills and demographic characteristics affects total factor productivity (TFP) of firms in Denmark. Implementing structural estimation of firms’ production function, we find evidence that labor diversity in skill...... openness, giving support to the hypothesis that an ethnically diverse workforce provides information and access to global markets....... costs connected to a more demographically and culturally diverse workforce, counteract the positive effects of diversity on firm TFP, coming from creativity and knowledge spillovers. However, we find that ethnic diversity is valuable for firms operating in industries characterized by above-average trade...

  15. Agility in a small software firm

    DEFF Research Database (Denmark)

    Schmidt, Thomas; Mathiassen, Lars

    2009-01-01

    Danish software firm, TeachTech Inc., through the lens of Haeckel's sense-and-respond approach. Our analysis suggests that: the firm has appropriate sense-and-respond cycles, but improving process modularity and human resource flexibility, could increase its ability to respond faster and more effectively......; the firm focuses on specific business goals, but these are not clearly explicated and expressed as empowering governing values enabling a quick and coordinated response; complex and demanding challenges are related to dynamically reassigning commitments and the supporting mechanisms are insufficient...

  16. Pro-social Motivation beyond Firm Boundaries

    DEFF Research Database (Denmark)

    Foss, Nicolai; Milagres, Rosileia

    2014-01-01

    An influential body of literature in macro-management research (notably, organization theory and strategic management) associates pro-social motivation solely with firm-like organizations, suggesting that such motivation cannot thrive under more market-like arrangements. We question this argument...... on theoretical, as well as empirical, grounds. As to the latter, we discuss the specific case of a network of firms in Brazil, the Genolyptus network. We argue that this particular network manifests strong pro-social motivations. This implies that pro-social motivations may thrive beyond corporate boundaries......-social motivations in non-firm governance structures....

  17. Firm-Specific Foreign Exchange Exposure Identification

    DEFF Research Database (Denmark)

    Aabo, Tom; Brodin, Danielle

    2014-01-01

    Previous studies have used the stock market approach to find the aggregate number of (firms with) foreign exchange exposures in a given country, region, or industry. Methodologies have differed in many aspects but two of the most basic differences relate to observation frequency and the choice......-financial firms and find limited consistency in the detected exchange rate exposures when altering methodology in terms of observation frequency and choice of market index. The results put a question mark to the validity of the stock market approach for exchange rate exposure identification at the firm...

  18. Banking Firm, Equity and Value at Risk

    Directory of Open Access Journals (Sweden)

    Udo Broll

    2012-12-01

    Full Text Available The paper focuses on the interaction between the solvency probability of a banking firm and the diversification potential of its asset portfolio when determining optimal equity capital. The purpose of this paper is to incorporate value at risk (VaR into the firm-theoretical model of a banking firm facing the risk of asset return. Given the necessity to achieve a confidence level for solvency, we demonstrate that diversification reduces the amount of equity. Notably, the VaR concept excludes a separation of equity policy and asset-liability management.

  19. Internationalisation of Firms in Developing Countries

    DEFF Research Database (Denmark)

    Kuada, John

    The last three decades have witnessed a substantial amount of research into the intrenationalisation process of firms. The focus of most of these studies have, however, been on the downstream aspects of internationalisation with the assumption that all firms initiate their internationalisation...... process with export and proceed in a path-dependent manner towards the location of production activities abroad. The present apper challenges this perspective, arguing that internationalisation of firms may be initiated through either or both upstream and downstream activities. It therefore proposes...

  20. Internationalisation of Firms in Developing Countries

    DEFF Research Database (Denmark)

    Kuada, John

    The last three decades have witnessed a substantial amount of research into the intrenationalisation process of firms. The focus of most of these studies have, however, been on the downstream aspects of internationalisation with the assumption that all firms initiate their internationalisation...... process with export and proceed in a path-dependent manner towards the location of production activities abroad. The present apper challenges this perspective, arguing that internationalisation of firms may be initiated through either or both upstream and downstream activities. It therefore proposes...

  1. Entrepreneurial Orientation in Small Established Firms

    DEFF Research Database (Denmark)

    Middel, Rick; Lassen, Astrid Heidemann; Laugen, Bjørge Timenes

    2010-01-01

    While research findings have provided important insights on radical innovation for small start-ups and large established organizations, there is still a gap in knowledge on the ability of the well-established small firm to engage in radical innovation. This paper addresses this gap in knowledge...... by discussing how small established firms exhibit entrepreneurial orientation and how does this influence their involvement in development of radical innovation. Through the case studies it became apparent that the small established firms do embody several characteristics which can be associated both......-taking and autonomy plays a very important role, and that the three dimensions are often intertwined....

  2. Mesoscale Morphological Change, Beach Rotation and Storm Climate Influences along a Macrotidal Embayed Beach

    Directory of Open Access Journals (Sweden)

    Tony Thomas

    2015-09-01

    Full Text Available Cross-shore profiles and environmental forcing were used to analyse morphological change of a headland bay beach: Tenby, West Wales (51.66 N; −4.71 W over a mesoscale timeframe (1996–2013. Beach profile variations were attuned with longer term shoreline change identified by previous research showing southern erosion and northern accretion within the subaerial zone and were statistically significant in both sectors although centrally there was little or no significance. Conversely a statistically significant volume loss was shown at all profile locations within the intertidal zone. There were negative phase relationships between volume changes at the beach extremities, indicative of beach rotation and results were statistically significant (p < 0.01 within both subaerial (R2 = 0.59 and intertidal (R2 = 0.70 zones. This was confirmed qualitatively by time-series analysis and further cross correlation analysis showed trend reversal time-lagged associations between sediment exchanges at either end of the beach. Wave height and storm events displayed summer/winter trends which explained longer term one directional rotation at this location. In line with previous regional research, environmental forcing suggests that imposed changes are influenced by variations in southwesterly wind regimes. Winter storms are generated by Atlantic southwesterly winds and cause a south toward north sediment exchange, while southeasterly conditions that cause a trend reversal are generally limited to the summer period when waves are less energetic. Natural and man-made embayed beaches are a common coastal feature and many experience shoreline changes, jeopardising protective and recreational beach functions. In order to facilitate effective and sustainable coastal zone management strategies, an understanding of the morphological variability of these systems is needed. Therefore, this macrotidal research dealing with rotational processes across the entire intertidal

  3. Measurement of caudate nucleus area - a more accurate measurement for Huntington's disease

    Energy Technology Data Exchange (ETDEWEB)

    Wardlaw, J.M.; Abernethy, L.J. (Royal Infirmary, London (United Kingdom). Dept. of Radiology); Sellar, R.J. (Western General Hospital, Edinburgh (United Kingdom). Dept. of Neuroradiology)

    1991-08-01

    Caudate nucleus atrophy occurs in Huntington's disease and methods of measuring this have been described using axial CT, but these are indirect and lack sensitivity. We measured caudate nucleus area (blind to the subjects' clinical state) in 30 subjects with or at risk of Huntington's disease, and in 100 normal age matched controls. Fifteen subjects with established symptomatic Huntington's disease, 3 with early symptoms, and 3 presymptomatic subjects (2 showing a high probability for the Huntington's disease gene on genetic testing, and one who has since developed symptoms) were correctly identified. Three normal (gene negative) family members were also correctly identified. Outcome is awaited in 6. CT caudate area measurement is simple and reproducible and we have found it to be a useful confirmatory test for Huntington's disease. (orig.).

  4. Clinical and counselling implications of preimplantation genetic diagnosis for Huntington's disease in the UK.

    Science.gov (United States)

    Lashwood, A; Flinter, F

    2001-01-01

    Huntington's disease is an autosomal dominant neurodegenerative disorder that usually occurs in adult life. Individuals at risk can have a gene test before the onset of symptoms, and prenatal diagnosis is available. Preimplantation genetic diagnosis (PGD) for Huntington's disease is now available for couples in whom one partner has the gene for Huntington's disease. A licence to practise PGD is required from the Human Fertilisation and Embryology Authority, and there are several complex issues relating to PGD for Huntington's disease that require consideration. The partner of the Huntington's disease gene carrier should have a presymptomatic test to ensure accuracy in a PGD cycle. There should be a delay between blood sampling and testing for Huntington's disease to allow time for reflection and withdrawal from testing. All PGD treatment has an associated risk of misdiagnosis. If confirmatory prenatal testing is not undertaken after a successful PGD cycle, no confirmation of diagnosis will be obtained at birth. Guidelines indicate that individuals who are at risk cannot be tested before 18 years. There is concern over the ability of a child or adolescent to make an informed choice about testing before this age. Confirmatory testing at birth after PGD would be in direct contravention of these guidelines. In the UK, the law requires consideration of the welfare of children born after assisted conception treatment. Presenting symptoms of Huntington's disease may affect the parenting abilities of an affected individual. There is a need for an assessment of a patient's current Huntington's disease status and their planned provision of care of children if Huntington's disease affects parenting. It has been necessary to create a detailed working protocol for the management of PGD for Huntington's disease to address these issues.

  5. The Relationship between Ownership Structure, Firm Specific Characteristics and Capital Structure: Evidence from Malaysian Middle-capital Public Listed Firms

    National Research Council Canada - National Science Library

    Hanita Kadir Shahar; Azira Abdul Adzis; Nureliana Baderi

    2016-01-01

    .... By employing a total of 38 middle-capital firms covering period from 2008 to 2012, the results show that debt level in firms with high ownership concentration is significantly different from firms...

  6. Service-Oriented Strategies for Manufacturing Firms

    DEFF Research Database (Denmark)

    Tan, Adrian; McAloone, Tim C.; Matzen, Detlef

    2009-01-01

    This chapter establishes PSS (Product/Service-Systems) approaches in the context of manufacturing firms and their existing product-oriented business. PSS can be seen as a strategy for manufacturing firms to gain competitive advantage in the market, but what market conditions and organisational fa......, customers and partners in business planning and strategy to reap the full benefits. Finally a path to how manufacturers can make the change from product to service-orientation is traced.......This chapter establishes PSS (Product/Service-Systems) approaches in the context of manufacturing firms and their existing product-oriented business. PSS can be seen as a strategy for manufacturing firms to gain competitive advantage in the market, but what market conditions and organisational...

  7. Judo Economics in Markets with Multiple Firms

    Directory of Open Access Journals (Sweden)

    Daniel Cracau

    2014-01-01

    Full Text Available We study a sequential Bertrand game with one dominant market incumbent and multiple small entrants selling homogeneous products. Whilst the equilibrium for the case of a single entrant is well known from Gelman and Salop (1983, we derive properties of the N-firm equilibrium and present an algorithm that can be used to calculate this equilibrium. The algorithm is based on a recursive manipulation of polynomials that derive the optimisation problem that each of the market entrants is facing. Using this algorithm we derive the exact equilibrium for the cases of two and three small entrants. For more than three entrants only approximate results are possible. We use numerical results to gain further understanding of the equilibrium for an increasing number of firms and in particular for the case where N diverges to infinity. Similarly to the two-firm Judo equilibrium, we see that a capacity limitation for the small firms is necessary to achieve positive profits.

  8. Ethnic Diversity and Firms' Export Behavior

    DEFF Research Database (Denmark)

    Parrotta, Pierpaolo; Pozzoli, Dario; Sala, Davide

    2016-01-01

    Media are reporting of companies that are increasing the diversity of their workforce to expand their business internationally. This paper investigates whether these examples constitute pieces of evidence that diversity promotes firms' internationalization. Indeed, diverse companies are like a co...

  9. Foreign Exchange Exposure of Korean Firms

    National Research Council Canada - National Science Library

    Ji-Seon Kim

    2002-01-01

    The purpose of this study is to examine the relationship between the movements of exchange rate and value of Korean firms, so-called foreign exchange rate exposure using newly devised model to find the strong evidence...

  10. Small Firm Internationalization, Innovation, and Growth

    NARCIS (Netherlands)

    Boermans, M.A.; Roelfsema, H.J.

    2016-01-01

    This paper studies the effect of internationalization on innovation and firm performance (employment growth and sales growth) taking the interdependencies among the variables into account. Given the potential endogeneity, this study uses theory-driven instrumental variables and structural equation

  11. EAS 2.948 Listed Test Firms

    Data.gov (United States)

    Federal Communications Commission — EAS (Equipment Authorization System). The following firms have submitted the information required by Section 2.948 of the FCC Rules for measuring devices subject to...

  12. Firm Performance-A Social Networks Perspective

    Directory of Open Access Journals (Sweden)

    Jane Tung

    2012-01-01

    Full Text Available Problem statement: The study aims to offer a discussion on social networks and their effect on firm performance and also to illustrate the ways by which the firms utilize social capital through networks. Approach: The literature review and arguments were conducted to provide a systematic discussion of social networks and their effects. Results: The study had provided with a detailed understanding of the strategies that had been found to be highly significant in successful organizational performance. The results of this discussion suggested that social networks affect firm performance in a positive manner and can contribute to sustainable competitive advantage. Conclusion: It confirms that building up of social networks for big and small firms provides valuable links and solves numerous issues which auger well for the companies and should be treated with great importance.

  13. New Environmental Practices in Polish Production Firms

    DEFF Research Database (Denmark)

    Kræmer, Trine Pipi

    2002-01-01

    Based on five case studies in Poland, the paper discusses, how a specific environmental policy influences the firms? industrial environmental practices. The study illustrates, how the Polish environmental policy, dominated by environmental charges on emissions, is extremely effective in improving...

  14. Taxing the Financially Integrated Multinational Firm

    DEFF Research Database (Denmark)

    Johannesen, Niels

    This paper develops a theoretical model of corporate taxation in the presence of financially integrated multinational firms. Under the assumption that multinational firms at least partly use internal loans to finance foreign investment, we find that the optimal corporate tax rate is positive from...... the perspective of a small, open economy. This finding contrasts the standard result that the optimal source based capital tax is zero. Intuitively, to the extent that multinational firms finance investment in country i with loans from affiliates in country j, the burden of corporate taxes in the latter country...... partly fall on investment and thus workers in the former country. This tax exporting mechanism introduces a scope for corporate taxes, which is not present in standard models of international taxation. Accounting for the internal capital markets of multinational firms thus represents a way to resolve...

  15. Integrated protecting plan for beach erosion. A case study in Plaka beach, E. Crete, Greece

    Science.gov (United States)

    Petrakis, Stelios; Alexandrakis, George; Kozyrakis, George; Hatziyanni, Eleni; Kampanis, Nikolaos

    2015-04-01

    Coastal zones are among the most active areas on Earth, being subjected to extreme wind / wave conditions, thus vulnerable to erosion. In Greece and Crete in particular, beach zones are extremely important for the welfare of the inhabitants, since, apart for the important biological and archaeological value of the beach zones, the socio-economic value is critical since a great number of human activities are concentrated in such areas (touristic facilities, fishing harbors etc.). The present study investigates the erosional procedures observed in Plaka beach, E. Crete, Greece, a highly touristic developed area with great archaeological interest and proposes a cost-effective solution. The factors taken into consideration for the proposed solution in reducing the erosion of the beach were the study of the climatological, geological and geomorphological regime of the area, the recent (~70 years) shifting of the coastline through the study of topographic maps, aerial photographs and satellite images, the creation of detailed bathymetric and seabed classification maps of the area and finally, a risk analysis in terms of erosional phenomena. On the basis of the above, it is concluded that the area under investigation is subjected to an erosional rate of about 1 m/10 years and the total land-loss for the past 70 years is about 4600 m2. Through the simulation of the wave regime we studied 3 possible scenarios, the "do-nothing" scenario, the construction of a detached submerged breakwater at the depth of 3 meters and, finally, the armoring of the existing beach-wall through the placement of appropriate size and material boulders, forming an artificial slope for the reducing of the wave breaking energy and a small scale nourishment plan. As a result, through the modeling of the above, the most appropriate and cost-effective solution was found to be the third, armoring of the existing coastal wall and nourishment of the beach periodically, thus the further undermining of the

  16. Customer profitability analysis in a particular firm

    OpenAIRE

    Turková, Karolína

    2015-01-01

    The bachelor thesis entitled "Customer profitability analysis in a particular firm" deals with approaches which are possible to use in an enterprise practice when analyzing customer profitability. The theoretical part describes tools and methods of management accounting which are used to analyze the profitability of customers. The application part consists of appraisal of the firm's current system of analyzing customer profitability and proposal of a new system. In the conclusion some recomme...

  17. Taxing the Financially Integrated Multinational Firm

    DEFF Research Database (Denmark)

    Johannesen, Niels

    This paper develops a theoretical model of corporate taxation in the presence of financially integrated multinational firms. Under the assumption that multinational firms at least partly use internal loans to finance foreign investment, we find that the optimal corporate tax rate is positive from...... the tension between standard theory predicting zero capital taxes and the casual observation that countries tend to employ corporate taxes at fairly high rates....

  18. Structure, Employment and Performance in Biotech Firms

    OpenAIRE

    Lund Jensen, Rasmus; Dahlgren, Henrich; Valentin, Finn

    2007-01-01

    This report studies employment effects associated with the adoption of modern biotechnology in Danish industry. In this context we also examine industry structure, patterns of job creation, key outputs such as patents and the pipeline of projects in clinical trials. To see the development of Danish biotech firms in a relevant context we compare a Danish segment of biotech firms with a matching Swedish segment. From an overall assessment modern biotechnology, despite the three decades elap...

  19. The Firm as an Internal Capital Market

    DEFF Research Database (Denmark)

    Duus, Henrik Johannsen

    1996-01-01

    A review of current strategy literature shows that the portfolio view seems to have become unfashionable. This article argues in favor of a revitalization of the portfolio view, and attempts to show that with a few changes, it can be used together with the modern theories of the firm and its stra...... strategy. At the same time it shows how a heterodox portfolio model may be used to control the competence development of firms situated in turbulent and complex settings....

  20. Facilitated inter-firm collaboration in Ghana

    DEFF Research Database (Denmark)

    Kuada, John Ernest; Sørensen, Olav Jull

    2005-01-01

    Since the mid-1980s, aid agencies have endorsed the need to support private enterprise development in developing countries as an instrument for overall economic development and poverty reduction. Facilitation of collaboration between developed and developing country firms has become one of the mo...... the mechanisms of third-party inter-firm facilitation and assesses how effective the catalyst institutions are in nurturing collaborations between companies in developed and developing countries. The discussions are illustrated with case examples drawn from Ghana....