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  1. La fouille du fort Saint-Georges à Chinon (Indre-et-Loire. Premiers résultats The excavation of fort Saint-Georges at Chinon (Indre-et-Loire. First results

    Directory of Open Access Journals (Sweden)

    Bruno Dufaÿ

    2006-05-01

    Full Text Available Cette note présente les premiers résultats des fouilles menées en 2003 et 2004 sur la quasi-totalité du fort Saint-Georges à Chinon (Indre-et-Loire. Celui-ci est l’un des trois éléments de la forteresse médiévale qui domine la ville. La fouille a permis de préciser la fonction du fort, construit dans la deuxième moitié du XIIe s., à l’époque où Chinon est le centre administratif des possessions continentales des Plantagenêt, rois d’Angleterre. Du point de vue militaire, il formait une fortification avancée, protégeant le château principal, selon une structure que Richard Cœur de Lion appliquera au Château Gaillard. À l’intérieur, de vastes bâtiments constituaient des logis, conçus peut-être au départ pour héberger la chancellerie royale.This article presents the first results of the excavations undertaken in 2003 and 2004 over almost all of the Fort Saint-Georges at Chinon (Indre-et-Loire, one of three elements of the medieval fortress which dominates the town. The excavation enabled us to clarify the function of the fort, built in the 2nd half of the 12th century at a time when Chinon was the administrative centre of the continental possesions of the Plantagenet King of England. From a military point of view, it formed an advanced fortification protecting the main castle, within a structure that Richard the Lionheart would apply to the Chayeau Gaillard. Inside, some vast buildings made up the dwellings, designed perhaps initially to house the royal chanceller.

  2. Tay-Sachs - resources

    Science.gov (United States)

    The following organizations provide information on Tay-Sachs disease : US National Library of Medicine -- ghr.nlm.nih.gov/condition/tay-sachs-disease March of Dimes -- www.marchofdimes.org/complications/tay-sachs- ...

  3. Tay-Sachs Disease

    Science.gov (United States)

    Tay-Sachs disease is a rare, inherited disease. It is a type of lipid metabolism disorder. It causes too ... cells, causing mental and physical problems. . Infants with Tay-Sachs disease appear to develop normally for the first few ...

  4. Simulation of groundwater flow to assess future withdrawals associated with Base Realignment and Closure (BRAC) at Fort George G. Meade, Maryland

    Science.gov (United States)

    Raffensperger, Jeff P.; Fleming, Brandon J.; Banks, William S.L.; Horn, Marilee A.; Nardi, Mark R.; Andreasen, David C.

    2010-01-01

    Increased groundwater withdrawals from confined aquifers in the Maryland Coastal Plain to supply anticipated growth at Fort George G. Meade (Fort Meade) and surrounding areas resulting from the Department of Defense Base Realignment and Closure Program may have adverse effects in the outcrop or near-outcrop areas. Specifically, increased pumping from the Potomac Group aquifers (principally the Patuxent aquifer) could potentially reduce base flow in small streams below rates necessary for healthy biological functioning. Additionally, water levels may be lowered near, or possibly below, the top of the aquifer within the confined-unconfined transition zone near the outcrop area. A three-dimensional groundwater flow model was created to incorporate and analyze data on water withdrawals, streamflow, and hydraulic head in the region. The model is based on an earlier model developed to assess the effects of future withdrawals from well fields in Anne Arundel County, Maryland and surrounding areas, and includes some of the same features, including model extent, boundary conditions, and vertical discretization (layering). The resolution (horizontal grid discretization) of the earlier model limited its ability to simulate the effects of withdrawals on the outcrop and near-outcrop areas. The model developed for this study included a block-shaped higher-resolution local grid, referred to as the child model, centered on Fort Meade, which was coupled to the coarser-grid parent model using the shared node Local Grid Refinement capability of MODFLOW-LGR. A more detailed stream network was incorporated into the child model. In addition, for part of the transient simulation period, stress periods were reduced in length from 1 year to 3 months, to allow for simulation of the effects of seasonally varying withdrawals and recharge on the groundwater-flow system and simulated streamflow. This required revision of the database on withdrawals and estimation of seasonal variations in

  5. SAChES: Scalable Adaptive Chain-Ensemble Sampling.

    Energy Technology Data Exchange (ETDEWEB)

    Swiler, Laura Painton [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Ray, Jaideep [Sandia National Lab. (SNL-CA), Livermore, CA (United States); Ebeida, Mohamed Salah [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Huang, Maoyi [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Hou, Zhangshuan [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Bao, Jie [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Ren, Huiying [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2017-08-01

    We present the development of a parallel Markov Chain Monte Carlo (MCMC) method called SAChES, Scalable Adaptive Chain-Ensemble Sampling. This capability is targed to Bayesian calibration of com- putationally expensive simulation models. SAChES involves a hybrid of two methods: Differential Evo- lution Monte Carlo followed by Adaptive Metropolis. Both methods involve parallel chains. Differential evolution allows one to explore high-dimensional parameter spaces using loosely coupled (i.e., largely asynchronous) chains. Loose coupling allows the use of large chain ensembles, with far more chains than the number of parameters to explore. This reduces per-chain sampling burden, enables high-dimensional inversions and the use of computationally expensive forward models. The large number of chains can also ameliorate the impact of silent-errors, which may affect only a few chains. The chain ensemble can also be sampled to provide an initial condition when an aberrant chain is re-spawned. Adaptive Metropolis takes the best points from the differential evolution and efficiently hones in on the poste- rior density. The multitude of chains in SAChES is leveraged to (1) enable efficient exploration of the parameter space; and (2) ensure robustness to silent errors which may be unavoidable in extreme-scale computational platforms of the future. This report outlines SAChES, describes four papers that are the result of the project, and discusses some additional results.

  6. Prenatal Diagnosis Of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Özgür Özyüncü

    2010-04-01

    CONCLUSION: Tay-Sachs disease can be diagnosed prenatally by measuring hexosaminidase enzyme activity in fetal tissue samples with an acceptable complication rate. Prenatal diagnosis should be offered to families who have affected siblings with Tay-Sachs disease.

  7. Enfermedad de Tay-Sachs

    Directory of Open Access Journals (Sweden)

    Albia Josefina Pozo Alonso

    Full Text Available La enfermedad de Tay-Sachs es un trastorno neurodegenerativo progresivo de herencia autosómica recesiva. Se debe a la deficiencia de la enzima β-hexosaminidasa A, que provoca una acumulación de gangliósidos GM2 en los lisosomas. Se incluye dentro de las esfingolipidosis. De las esfingolipidosis que presentan mancha rojo cereza en la mácula, la enfermedad de Tay-Sachs es la única en la que no se evidencia hepatoesplenomegalia. La variante más frecuente se inicia en la lactancia. Se presenta un lactante del sexo masculino al que se le realizó el diagnóstico de esta entidad a los 8 meses de edad. A partir de los 4 meses comenzó a presentar una reacción de sobresalto. A los 6 meses comenzó a perder habilidades previamente adquiridas y crisis epilépticas mioclónicas. Se constató una disminución de la actividad específica de la enzima hexosaminidasa A en leucocitos.

  8. Management and Data Management Plan for Remedial Investigation at Fort George G. Meade Landfill and Preliminary Assessment/Site Investigation at the Former Gaithersburg NIKE Control and Launch Areas

    National Research Council Canada - National Science Library

    Edwards, D

    1989-01-01

    Work assignments under this contract will include a Preliminary Assessment/Site Investigation at the former Gaithersburg NIKE Control and Launch Areas and a Remedial Investigation at the Fort Meade...

  9. Ethical Issues with Genetic Testing for Tay-Sachs.

    Science.gov (United States)

    Clayton, Tricia

    Several genetic disorders are specific to Jewish heritage; one of the most devastating is Tay-Sachs disease.Tay-Sachs is a fatal hereditary disease, causing progressive neurological problems for which there is no cure. Ethical issues surrounding genetic testing for Tay-Sachs within the Jewish community continue to be complex and multifaceted. A perspective of Tay-Sachs, using rights-based ethics and virtue ethics as a theoretical framework, is explored.

  10. Tay-Sachs and Sandhoff Diseases

    Science.gov (United States)

    ... more common in these groups: Creole of northern Argentina Metis Indians in Saskatchewan, Canada Lebanese If you or your partner is part of these ethnic groups, or you have a family history of Tay-Sachs disease or Sandhoff disease, tell ...

  11. Tay Sachs disease: an autopsy case report.

    Science.gov (United States)

    Jadhav, Meenal Vitthal; Landge, Meenal P; Sawaimoon, Satyakam K; Harke, Arun B; Deshmukh, Sanjay D

    2005-10-01

    This report describes a case report of a postmortem performed on a 5-year old patient of Tay-Sachs disease, presenting with failure to thrive, muscular flaccidity, and cherry-red spots on macula on fundoscopy. There was no history of similarly affected sibling or any other family member. The diagnosis was confirmed by enzyme studies. At postmortem, there was no organomegaly. The brain, on microscopy, showed vacuolated swollen neurons.

  12. The National Tay Sachs and Allied Diseases Association.

    Science.gov (United States)

    Zeitlin, Paula

    1986-01-01

    The National Tay-Sachs and Allied Diseases Association is involved in education, research, and prevention of Tay-Sachs, an inherited metabolic disorder which destroys the central nervous system, and over 30 related disorders. The group features a parent peer group network and a support group for carrier couples. (CL)

  13. National Tay-Sachs and Allied Diseases Association, Inc.

    Science.gov (United States)

    Exceptional Parent, 1977

    1977-01-01

    Reviewed are the history and organization, purpose and programs, and public services of the National Tay-Sachs and Allied Diseases Association, an organization geared toward eradicating Tay-Sachs disease (a hereditary disorder affecting primarily Jewish infants which generally leads to deterioration and death by the child's fifth year). (SBH)

  14. The metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue

    Science.gov (United States)

    Tallman, John F.; Johnson, William G.; Brady, Roscoe O.

    1972-01-01

    The catabolism of Tay-Sachs ganglioside, N-acetylgalactosaminyl- (N-acetylneuraminosyl) -galactosylglucosylceramide, has been studied in lysosomal preparations from normal human brain and brain obtained at biopsy from Tay-Sachs patients. Utilizing Tay-Sachs ganglioside labeled with 14C in the N-acetylgalactosaminyl portion or 3H in the N-acetylneuraminosyl portion, the catabolism of Tay-Sachs ganglioside may be initiated by either the removal of the molecule of N-acetylgalactosamine or N-acetylneuraminic acid. The activity of the N-acetylgalactosamine-cleaving enzyme (hexosaminidase) is drastically diminished in such preparations from Tay-Sachs brain whereas the activity of the N-acetylneuraminic acid-cleaving enzyme (neuraminidase) is at a normal level. Total hexosaminidase activity as measured with an artificial fluorogenic substrate is increased in tissues obtained from patients with the B variant form of Tay-Sachs disease and it is virtually absent in the O-variant patients. The addition of purified neuraminidase and various purified hexosaminidases exerted only a minimal synergistic effect on the hydrolysis of Tay-Sachs ganglioside in the lysosomal preparations from the control or patient with the O variant of Tay-Sachs disease. Images PMID:4639018

  15. Survey on serum acetylecholinesterase (SAChE activity in selected farming communities at risk of organophosphate exposure, Southern Ghana

    Directory of Open Access Journals (Sweden)

    Ato Kwamena Tetteh

    2017-12-01

    Material and Methods ― The study was cross-sectional, involving 963 participants who consented to being screened for serum acetylcholinesterase (SAChE activity, using the dry chemistry test strips. The SAChE activity was then categorized into normal, low or high risk of developing symptoms associated with OP poisoning. Results ― The total group included 54.5% females and 45.5% males. Majority were crop farmers (46.3% and students/pupils (30.8%. The remaining 22.9% consisted mainly of traders, teachers, fishermen/fish processors, drivers and dressmakers. Very low SAChE activity unit of 30 U/ml. By this, a significant majority had either low or high risk of developing OP poisoning (χ2=4.43, 95%CI: 4.12–4.65, p=0.014 compared with those with normal activity. Most of the participants were in the 10–19 years age group of which 15.6% were in the high risk category, while 13.6% had low risk of OP poisoning. Reduced SAChE activity was predominant among all age groups compared with those with normal activity of the enzyme. As well, 27.3% who were females had reduced SAChE activity rate <30 U/ml (high risk of OP poisoning. Similarly, 23.4% of males were also at high risk of OP poisoning. There was however no statistical significance between the sex with regards to the numbers at risk of OP poisoning (χ2=4.80, 95% CI: 4.25–4.75, p=0.160. Conclusion ― This study provides some evidence of exposure, deduced from reduced SAChE activity, which potentially puts participants at a high risk of developing symptoms associated with OP poisoning. A follow-up study might be able to suggest to physicians in Ghana considering diagnostics of OP poisoning, especially for clients from farming communities where pesticides are used extensively.

  16. Fortællingen

    DEFF Research Database (Denmark)

    Hejlsted, Annemette

    Fortællingen - teori og analyse introducerer til teorier om fortællingen og præsenterer et sæt af analytiske tilgange til fortællinger af enhver art. Bogen lægger vægt på læsersynsvinklen og retter opmærksomheden mod de vilkår for menings- og betydningsdannelse, der kendetegner fortællingen. Begr....... Begreber om plot, fortællingens verden, karakterer, fortæller, modus og genre behandles, og deres anvendelse demonstreres på dansk og nordisk litteratur - med inddragelse af eksempler fra film og tv-reklamer....

  17. Tay Sachs and Related Storage Diseases: Family Planning

    Science.gov (United States)

    Schneiderman, Gerald; And Others

    1978-01-01

    Based on interviews with 24 families, the article discusses family planning and the choices available to those families in which a child has previously died from Tay-Sachs or related lipid storage diseases. (IM)

  18. Sajan D George

    Indian Academy of Sciences (India)

    Home; Journals; Resonance – Journal of Science Education. Sajan D George. Articles written in Resonance – Journal of Science Education. Volume 17 Issue 7 July 2012 pp 682-690 General Article. Microfluidics – A Lab in Your Palm · Dileep Mampallil Sajan D George · More Details Fulltext PDF ...

  19. Late-onset Tay-Sachs disease.

    Science.gov (United States)

    Barritt, Andrew W; Anderson, Stuart J; Leigh, P Nigel; Ridha, Basil H

    2017-10-01

    We discuss the assessment and differential diagnoses of a young adult Hungarian man with a 1-year history of a progressive and symmetric amyotrophic lateral sclerosis-like syndrome, along with irregular action tremor and stimulus-sensitive myoclonus of the arms. MR scan of the brain showed isolated cerebellar atrophy and formal neuropsychometric testing identified significant subclinical deficits in attention, processing speed and memory. We suspected a form of GM 2 gangliosidosis, and white cell enzyme analysis showed markedly reduced enzymatic activity of β-hexosaminidase A. Genetic testing subsequently revealed two heterozygous pathogenic mutations in the HEXA gene (c.1499delT p.(Leu500fs) and c.805G>A p.(Gly269Ser)), confirming the very rare diagnosis of adult-onset Tay-Sachs disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  20. Energy in the Kantowski–Sachs space-time using teleparallel ...

    Indian Academy of Sciences (India)

    Energy in the Kantowski–Sachs space-time using teleparallel geometry ... Kantowski–Sachs metric; teleparallelism; gravitational energy. Abstract. The purpose of this paper is to examine the energy content of the inflationary Universe described by Kantowski–Sachs space-time in quasilocal approach of teleparallel gravity ...

  1. A critique of Jeffrey D. Sachs's The end of poverty.

    Science.gov (United States)

    Henwood, Doug

    2006-01-01

    Jeffrey Sachs's The End of Poverty is a manifesto and how-to guide on ending extreme poverty around the world; it promotes the U.N. Millennium Development Goals. Sachs achieved fame with his policy package for the "stabilization" of Bolivia (which did nothing to relieve Bolivia's poverty), and became advisor to the Yeltsin government in Russia and to Poland, Slovenia, and Estonia as they began their transitions to capitalism (the last three mixed successes; Russia a thorough disaster). Sachs later became more prominent as a critic of development orthodoxy, and was economic advisor to the Jubilee 2000 movement. The End of Poverty is full of sharp critiques of Western imperialism, but his views on the rest of the development business are more conventional.

  2. Tay-Sachs disease in Jacob sheep.

    Science.gov (United States)

    Torres, Paola A; Zeng, Bai Jin; Porter, Brian F; Alroy, Joseph; Horak, Fred; Horak, Joan; Kolodny, Edwin H

    2010-12-01

    Autopsy studies of four Jacob sheep dying within their first 6-8 months of a progressive neurodegenerative disorder suggested the presence of a neuronal storage disease. Lysosomal enzyme studies of brain and liver from an affected animal revealed diminished activity of hexosaminidase A (Hex A) measured with an artificial substrate specific for this component of β-hexosaminidase. Absence of Hex A activity was confirmed by cellulose acetate electrophoresis. Brain lipid analyses demonstrated the presence of increased concentrations of G(M2)-ganglioside and asialo-G(M2)-ganglioside. The hexa cDNA of Jacob sheep was cloned and sequenced revealing an identical number of nucleotides and exons as in human HexA and 86% homology in nucleotide sequence. A missense mutation was found in the hexa cDNA of the affected sheep caused by a single nucleotide change at the end of exon 11 resulting in skipping of exon 11. Transfection of normal sheep hexa cDNA into COS1 cells and human Hex A-deficient cells led to expression of Hex S but no increase in Hex A indicating absence of cross-species dimerization of sheep Hex α-subunit with human Hex β-subunits. Using restriction site analysis, the heterozygote frequency of this mutation in Jacob sheep was determined in three geographically separate flocks to average 14%. This large naturally occurring animal model of Tay-Sachs disease is the first to offer promise as a means for trials of gene therapy applicable to human infants. Copyright © 2010 Elsevier Inc. All rights reserved.

  3. Planck 2015 results: XXI. The integrated Sachs-Wolfe effect

    DEFF Research Database (Denmark)

    Ade, P. A R; Aghanim, N.; Arnaud, M.

    2016-01-01

    This paper presents a study of the integrated Sachs-Wolfe (ISW) effect from the Planck 2015 temperature and polarization data release. This secondary cosmic microwave background (CMB) anisotropy caused by the large-scale time-evolving gravitational potential is probed from different perspectives....

  4. Rapid detection of fetal Mendelian disorders: Tay-Sachs disease.

    Science.gov (United States)

    Guetta, Esther; Peleg, Leah

    2008-01-01

    Tay-Sachs disease is an autosomal recessive storage disease caused by the impaired activity of the lysosomal enzyme hexosaminidase A. In this fatal disease, the sphingolipid GM2 ganglioside accumulates in the neurons. Due to high carrier rates and the severity of the disease, population screening and prenatal diagnosis of Tay-Sachs disease are routinely carried out in Israel. Laboratory diagnosis of Tay-Sachs is carried out with biochemical and DNA-based methods in peripheral and umbilical cord blood, amniotic fluid, and chorionic villi samples. The assay of hexosaminidase A (Hex A) activity is carried out with synthetic substrates, 4-methylumbelliferyl-6-sulfo-N-acetyl-beta-glucosaminide (4-MUGS) and 4-methylumbelliferil-N-acetyl-beta-glucosamine (4-MUG), and the DNA-based analysis involves testing for the presence of specific known mutations in the alpha-subunit gene of Hex A. Prenatal diagnosis of Tay-Sachs disease is accomplished within 24-48 h from sampling. The preferred strategy is to simultaneously carry out enzymatic analysis in the amniotic fluid supernatant or in chorionic villi and molecular DNA-based testing in an amniotic fluid cell-pellet or in chorionic villi.

  5. George's cosmic treasure hunt

    CERN Document Server

    Hawking, Lucy; Parsons, Gary

    2009-01-01

    George and Annie explore the galaxy in this cosmic adventure from Stephen Hawking and Lucy Hawking, complete with essays from Professor Hawking about the latest in space travel. George is heartbroken when he learns that his friend Annie and her father are moving to the US. Eric has a new job working for the space program, looking for signs of life in the Universe. Eric leaves George with a gift—a book called The User’s Guide to the Universe. But Annie and Eric haven’t been gone for very long when Annie believes that she is being contacted by aliens, who have a terrible warning for her. George joins her in the US to help her with her quest—and before he knows it, he, Annie, Cosmos, and Annie’s annoying cousin Emmett have been swept up in a cosmic treasure hunt, spanning the whole galaxy and beyond. Lucy Hawking's own experiences in zero-gravity flight and interviews with astronauts at Cape Kennedy and the Johnson Space Center lend the book a sense of realism and excitement that is sure to fire up ima...

  6. Dodson, Prof. George Guy

    Indian Academy of Sciences (India)

    Home; Fellowship. Fellow Profile. Elected: 2003 Honorary. Dodson, Prof. George Guy FRS. Date of birth: 13 January 1937. Date of death: 24 December 2012. Last known address: Division of Protein Structure, National Institute for Medical, Research, Mill Hill, London NW7 1AA, U.K.. YouTube; Twitter; Facebook; Blog ...

  7. Series, Prof. George William

    Indian Academy of Sciences (India)

    Home; Fellowship. Fellow Profile. Elected: 1984 Honorary. Series, Prof. George William. Date of birth: 22 February 1920. Date of death: 2 January 1995. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. 29th Mid-year meeting. Posted on 19 January 2018. The 29th Mid-year meeting of the Academy will be held ...

  8. George Sigerson: Charcot's translator.

    Science.gov (United States)

    Lyons, J B

    1997-04-01

    Senator George Sigerson (1836-1925), Dublin's first neurologist, was also a significant contributor to Anglo-Irish literature. His medical career and literary accomplishments are outlined, the focus of the article being Sigerson's friendly relationship with Charcot (with whom he corresponded), and whose Leçons sur les maladies du système nerveux he translated.

  9. Who Framed George Lakoff?

    Science.gov (United States)

    Goldstein, Evan R.

    2008-01-01

    In this article, a noted linguist reflects on his tumultuous foray into politics. For years George P. Lakoff has been at the center of some of the biggest intellectual disagreements in linguistics (most famously with Noam Chomsky) and has helped create an important interdisciplinary field of study, cognitive linguistics, that is reshaping people's…

  10. Hommage à Georges Duby

    Directory of Open Access Journals (Sweden)

    Dominique Iogna-Prat

    2006-09-01

    Full Text Available Dix ans après le décès du grand maître des études médiévales, le temps est sans nul doute venu de revisiter l’œuvre de Georges Duby et d’en discuter l’actualité du point de vue de l’histoire sociale du Moyen Âge. À une seule exception – Claudie Amado, qui fut son assistante –, les historiens invités n’ont pas travaillé au contact direct de Georges Duby ; il leur a été demandé de faire, chacun dans leur domaine, le bilan des apports du maître et de mettre en perspective ses principales thèses ...

  11. CERN honours Georges Charpak

    CERN Multimedia

    2009-01-01

    CERN pays tribute to the work of Georges Charpak at a colloquium in honour of his 85th birthday. var flash_video_player=get_video_player_path(); insert_player_for_external('Video/Public/Movies/2009/CERN-MOVIE-2009-008/CERN-MOVIE-2009-008-0753-kbps-480x360-25-fps-audio-64-kbps-44-kHz-stereo', 'mms://mediastream.cern.ch/MediaArchive/Video/Public/Movies/2009/CERN-MOVIE-2009-008/CERN-MOVIE-2009-008-Multirate-200-to-753-kbps-480x360.wmv', 'false', 480, 360, 'https://mediastream.cern.ch/MediaArchive/Video/Public/Movies/2009/CERN-MOVIE-2009-008/CERN-MOVIE-2009-008-posterframe-480x360-at-10-percent.jpg', '1167500', true, 'Video/Public/Movies/2009/CERN-MOVIE-2009-008/CERN-MOVIE-2009-008-0600-kbps-maxH-360-25-fps-audio-128-kbps-48-kHz-stereo.mp4'); Watch the video conference of Georges Charpak.   On 9 March CERN’s Main Auditorium was the venue for a fascinating and moving celebration marking the 85th birthday of Georges Charpak, who was awarded the Nobel Prize for Physics in 1992 for his inven...

  12. Choroidal Coloboma in a Case of Tay-Sachs Disease

    OpenAIRE

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  13. Choroidal coloboma in a case of tay-sachs disease.

    Science.gov (United States)

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  14. Distribution of a pseudodeficiency allele among Tay-Sachs carriers

    Energy Technology Data Exchange (ETDEWEB)

    Tomczak, J.; Grebner, E.E. (Thomas Jefferson Univ., Philadelphia, PA (United States)); Boogen, C. (Univ. of Essen Medical School (Germany))

    1993-08-01

    Recently Triggs-Raine et al. (1992) identified a new mutation in the gene coding for the [alpha]-subunit of [beta]-hexosaminidase A (hex A), the enzyme whose deficiency causes Tay-Sachs disease. This mutation, a C[sub 739]-to-T transition in exon 7, results in an altered enzyme that is active (albeit at reduced levels) in cells but that has essentially no activity in serum. This so-called pseudodeficient allele was first detected in compound heterozygotes who also carried a Tay-Sachs disease allele and therefore had no detectable hex A in their serum but who were in good health. Carriers of this apparently benign mutation are generally indistinguishable from carriers of a lethal mutation by means of routine enzyme-based screening tests, because the product of the pseudodeficient allele is not detectable in serum and has decreased activity in cells. This suggests that some individuals who have been classified as Tay-Sachs carriers are actually carriers of the pseudodeficient allele and are not at risk to have a child affected with Tay-Sachs disease. The pseudodeficient allele may also be responsible for some inconclusive diagnoses, where leukocyte values fall below the normal range but are still above the carrier range. The fact that there are now two mutant alleles (the psuedodeficient and the adult) that are indistinguishable from the lethal infantile mutations by means of enzyme assay yet that are phenotypically very different and that together may account for as much as 12% of enzyme-defined carriers on the basis of the data here suggests that DNA analysis should be part of a comprehensive screening program. It will be particularly useful to identify the mutations in couples at risk, before they undergo prenatal diagnosis. DNA analysis will also resolve some inconclusive diagnoses.

  15. Choroidal Coloboma in a Case of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Nasreen Raees Ahmed

    2014-01-01

    Full Text Available Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  16. George de Hevesy

    International Nuclear Information System (INIS)

    Levi, H.

    1985-01-01

    George de Hevesy (1875-1966) worked with Rutherford in Manchester in the early 1900's and with Niels Bohr in Denmark after the first world war. With Coster he discovered an unknown element, later named Hafrivm. In the 1930's he moved to Copenhagen and developed radiotracer techniques using the new artificially produced radioisotopes. This work found wide application in medicine and biology and other branches of science and technology and for his pioneer work de Hevesy was awarded a Nobel Prize in 1944. (UK)

  17. Georges Perec's literary staging

    Directory of Open Access Journals (Sweden)

    Ewelina Woźniak-Czech

    2017-12-01

    Full Text Available The purpose of this sketch to present Georges Perec’s work in the context of drama. As Perec wrote just one play for the theater, it’s hard to call him a dramatist. However, it is easy to see that all the literary tricks he uses in his works are a part of a well-thought-out game. The author of Life a User’s Manual employs and interweaves various literary conventions, prompts dialogue between his own works and draws the reader into the very center of his tricks, nuances and simulations. Perec’s writing could be deemed a pure act of dramatization, where the author himself, to some extent, takes on the role of director, making his audience not so much the recipients of his texts but the actors performing them.

  18. Georges Didi-Huberman

    OpenAIRE

    Hagelstein, Maud

    2012-01-01

    Georges Didi-Huberman est un philosophe et un historien de l'art français né à Saint-Étienne le 13 juin 1953. Il a été pensionnaire à l'Académie de France à Rome (Villa Médicis) et résident à la Fondation Berenson de la Villa I Tatti à Florence. Il enseigne à l’École des hautes études en sciences sociales où il est maître de conférences depuis 1990. Bibliographie Invention de l’hystérie. Charcot et l’Iconographie photographique de la Salpêtrière, sur l'École de la Salpêtrière, Macula, 1982. M...

  19. The white matter of the human cerebrum: part I The occipital lobe by Heinrich Sachs.

    Science.gov (United States)

    Forkel, Stephanie J; Mahmood, Sajedha; Vergani, Francesco; Catani, Marco

    2015-01-01

    This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  20. The white matter of the human cerebrum: Part I The occipital lobe by Heinrich Sachs

    Science.gov (United States)

    Forkel, Stephanie J.; Mahmood, Sajedha; Vergani, Francesco; Catani, Marco

    2015-01-01

    This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work. PMID:25527430

  1. Fortæller

    DEFF Research Database (Denmark)

    Larsen, Gorm

    2012-01-01

    Siden Gerard Genettes ”Discours du récit” (1972) er distinktionen mellem hvem, der taler, og hvem, der ser, blevet cementeret som et grundparadigme i narratologien og litteraturteorien. Genettes pointe var, at den etablerede narrative teori – som fx Wayne C. Booths The Rhetoric of Fiction (1961...... narratologi blevet forsøgt udfordret, enten fordi det hævdes, at en tekst ikke nødvendigvis er udstyret med en fortæller, eller fordi begrebet om fortæller antages at bero på en misvisende og reduktiv antropomorficering. Eller omvendt fordi der i Genettes begrebsdannelse ligger en forkastelse af...... forestillingen om en implicit forfatter (implied author) og dermed også en afvisning af en upålidelige fortæller. Kapitlet præsenterer begreberne fortæller og synsvinkel i narratologien med afsæt i Genettes bestemmelser og diskutere de problemer, der opstår i kølvandet herpå. Det være sig både de rent...

  2. Fortællerfiktionen

    DEFF Research Database (Denmark)

    Reitan, Rolf

    Bogen er en kritisk nærlæsning af Gérard Genettes Discours du récit og viser, hvorden den franske teoretiker løser og forenkler en række centrale problemer i traditionel fortælleteori, idet han uudtalt forudsætter et fiktionsbegreb, som han eksplicit afviser som narratologisk relevant. Det...

  3. An inducible mouse model of late onset Tay-Sachs disease.

    Science.gov (United States)

    Jeyakumar, Mylvaganam; Smith, David; Eliott-Smith, Elena; Cortina-Borja, Mario; Reinkensmeier, Gabriele; Butters, Terry D; Lemm, Thorsten; Sandhoff, Konrad; Perry, V Hugh; Dwek, Raymond A; Platt, Frances M

    2002-08-01

    Mouse models of the G(M2) gangliosidoses, Tay-Sachs and Sandhoff disease, are null for the hexosaminidase alpha and beta subunits respectively. The Sandhoff (Hexb-/-) mouse has severe neurological disease and mimics the human infantile onset variant. However, the Tay-Sachs (Hexa-/-) mouse model lacks an overt phenotype as mice can partially bypass the blocked catabolic pathway and escape disease. We have investigated whether a subset of Tay-Sachs mice develop late onset disease. We have found that approximately 65% of the mice develop one or more clinical signs of the disease within their natural life span (n = 52, P disease at an earlier age (n = 21, P Tay-Sachs mice confirmed that pregnancy induces late onset Tay-Sachs disease. Onset of symptoms correlated with reduced up-regulation of hexosaminidase B, a component of the bypass pathway.

  4. Kantowski--Sachs cosmological models as big-bang models

    International Nuclear Information System (INIS)

    Weber, E.

    1985-01-01

    In the presence of a nonzero cosmological constant Λ, we classify the anisotropic cosmological models of the Kantowski--Sachs type by means of the quantities epsilon 2 0 , q 0 , summation 0 corresponding, respectively, to the relative root-mean-square deviation from isotropy, the deceleration parameter, and the density parameter of the perfect fluid at a given time t = t 0 . We obtain for Λ>0 a set of big-bang models of zero measure as well as a set of cosmological models of nonzero measure evolving toward the de Sitter solution

  5. Rapid identification of HEXA mutations in Tay-Sachs patients.

    Science.gov (United States)

    Giraud, Carole; Dussau, Jeanne; Azouguene, Emilie; Feillet, François; Puech, Jean-Philippe; Caillaud, Catherine

    2010-02-19

    Tay-Sachs disease (TSD) is a recessively inherited neurodegenerative disorder due to mutations in the HEXA gene resulting in a beta-hexosaminidase A (Hex A) deficiency. The purpose of this study was to characterize the molecular abnormalities in patients with infantile or later-onset forms of the disease. The complete sequencing of the 14 exons and flanking regions of the HEXA gene was performed with a unique technical condition in 10 unrelated TSD patients. Eleven mutations were identified, including five splice mutations, one insertion, two deletions and three single-base substitutions. Four mutations were novel: two splice mutations (IVS8+5G>A, IVS2+4delAGTA), one missense mutation in exon 6 (c.621T>G (p.D207E)) and one small deletion (c.1211-1212delTG) in exon 11 resulting in a premature stop codon at residue 429. The c.621T>G missense mutation was found in a patient presenting an infantile form. Its putative role in the pathogenesis of TSD is suspected as residue 207 is highly conserved in human, mouse and rat. Moreover, structural modelling predicted changes likely to affect substrate binding and catalytic activity of the enzyme. The time-saving procedure reported here could be useful for the characterization of Tay-Sachs-causing mutations, in particular in non-Ashkenazi patients mainly exhibiting rare mutations. Copyright (c) 2010 Elsevier Inc. All rights reserved.

  6. Tay-Sachs disease with conspicuous cranial computerized tomographic appearances

    International Nuclear Information System (INIS)

    Watanabe, Kishichiro; Mukawa, Akio; Muto, Kazuhiko; Nishikawa, Jiro; Takahashi, Shigeko.

    1985-01-01

    An autopsy case of a 3-year-old female infant with Tay-Sachs disease was presented. A cherry red spot in the fundus and a deficiency of N-acetyl-β-hexosaminidase A in the white blood cells were revealed soon after admission at the age of one year. Her parents and sister were found to be healthy carriers. The patient showed a typical clinical course with marked cranial swelling. In addition to the marked ballooning of neurons on light microscope, membranous cytoplasmic body (MCB) on electron microscope and abnormal accumulation of GM 2 ganglioside in the cerebral cortex by thin layer chromatography were confirmed in the autopsy specimens. In the late stage of her clinical course, the cranial computerized tomography (CT) demonstrated symmetric and deep-wavy hyperdense cerebral cortical zones, diffuse hypodensity and diminished volume of cerebral white matter, mild to moderate ventricular dilatation, and a small cerebellum and brainstem. These conspicuous appearances of the cranial CT seem to be characteristic of Tay-Sachs disease in the late stage, and they are derived from abnormal accumulation of GM 2 ganglioside in the cerebral cortex, and diffuse intense demyelination (dysmyelinating demyelination) of the cerebral white matter. (author)

  7. Gravitational, shear and matter waves in Kantowski-Sachs cosmologies

    Energy Technology Data Exchange (ETDEWEB)

    Keresztes, Zoltán; Gergely, László Á. [Department of Theoretical Physics, University of Szeged, Tisza Lajos krt 84-86, Szeged 6720 (Hungary); Forsberg, Mats; Bradley, Michael [Department of Physics, UmeåUniversity (Sweden); Dunsby, Peter K.S., E-mail: zkeresztes@titan.physx.u-szeged.hu, E-mail: forsberg.mats.a.b@gmail.com, E-mail: michael.bradley@physics.umu.se, E-mail: peter.dunsby@uct.ac.za, E-mail: gergely@physx.u-szeged.hu [Astrophysics, Cosmology and Gravity Centre (ACGC), University of Cape Town, Rondebosch 7701, Cape Town (South Africa)

    2015-11-01

    A general treatment of vorticity-free, perfect fluid perturbations of Kantowski-Sachs models with a positive cosmological constant are considered within the framework of the 1+1+2 covariant decomposition of spacetime. The dynamics is encompassed in six evolution equations for six harmonic coefficients, describing gravito-magnetic, kinematic and matter perturbations, while a set of algebraic expressions determine the rest of the variables. The six equations further decouple into a set of four equations sourced by the perfect fluid, representing forced oscillations and two uncoupled damped oscillator equations. The two gravitational degrees of freedom are represented by pairs of gravito-magnetic perturbations. In contrast with the Friedmann case one of them is coupled to the matter density perturbations, becoming decoupled only in the geometrical optics limit. In this approximation, the even and odd tensorial perturbations of the Weyl tensor evolve as gravitational waves on the anisotropic Kantowski-Sachs background, while the modes describing the shear and the matter density gradient are out of phase dephased by π /2 and share the same speed of sound.

  8. [George Nicholas Papanicolaou].

    Science.gov (United States)

    Broso, P R; Buffetti, G

    1993-10-01

    G. N. Papanicolaou was born on May the 13, 1883 in the city of Kymi on the Greek island of Euboea. He received his MD degree from the University of Athens in 1904 and a PhD from the University of Munich in 1910. After service as a medical officer in the Balkan War of 1912-1913, he came to New York with Mary (for over 50 years Dr Pap's life companion). George's violin playing at restaurants and coffee-shops supplied them with a few extra cents. Papanicolaou was appointed assistant in the Pathology Laboratory at the New York Hospital. In 1928 he presented his work "New Cancer Diagnosis" to the third race betterment conference (Battle Creek, Michigan). But the work was met with scepticism. The now famous monograph "The Diagnostic Value of Vaginal Smears in Carcinoma of the Uterus" was published in 1941 in the Am J Obst Gyn. During this time, he developed his method of preservation of these cells by wet fixation and precise staining. Papanicolaou persisted with his ideas, and finally cytologic examination of the cervix was accepted. The power of Papanicolaou screening for uterine cancer was remarkable. The first National Cytology Congress, held in 1948, hailed this new diagnostic tool for carcinoma of the cervix as unique because it could detect cancer before it was visible. He described the importance of a distinct cellular pattern corresponding to cervical intraepithelial neoplastic lesions. The value of this pattern, expressing evolutionary steps in the development of cancer at individual cell levels, was not appreciated.(ABSTRACT TRUNCATED AT 250 WORDS)

  9. Structural consequences of amino acid substitutions causing Tay-Sachs disease.

    Science.gov (United States)

    Ohno, Kazuki; Saito, Seiji; Sugawara, Kanako; Sakuraba, Hitoshi

    2008-08-01

    To determine the structural changes in the alpha-subunit of beta-hexosaminidase due to amino acid substitutions causing Tay-Sachs disease, we built structural models of mutant alpha-subunits resulting from 33 missense mutations (24 infantile and 9 late-onset), and analyzed the influence of each amino acid replacement on the structure by calculating the number of atoms affected and determining the solvent-accessible surface area of the corresponding amino acid residue in the wild-type alpha-subunit. In the infantile Tay-Sachs group, the number of atoms influenced by a mutation was generally larger than that in the late-onset Tay-Sachs group in both the main chain and the side chain, and residues associated with the mutations found in the infantile Tay-Sachs group tended to be less solvent-accessible than those in the late-onset Tay-Sachs group. Furthermore, color imaging determined the distribution and degree of the structural changes caused by representative amino acid substitutions, and that there were also differences between the infantile and late-onset Tay-Sachs disease groups. Structural study is useful for elucidating the basis of Tay-Sachs disease.

  10. George and the big bang

    CERN Document Server

    Hawking, Lucy; Parsons, Gary

    2012-01-01

    George has problems. He has twin baby sisters at home who demand his parents’ attention. His beloved pig Freddy has been exiled to a farm, where he’s miserable. And worst of all, his best friend, Annie, has made a new friend whom she seems to like more than George. So George jumps at the chance to help Eric with his plans to run a big experiment in Switzerland that seeks to explore the earliest moment of the universe. But there is a conspiracy afoot, and a group of evildoers is planning to sabotage the experiment. Can George repair his friendship with Annie and piece together the clues before Eric’s experiment is destroyed forever? This engaging adventure features essays by Professor Stephen Hawking and other eminent physicists about the origins of the universe and ends with a twenty-page graphic novel that explains how the Big Bang happened—in reverse!

  11. 'Cherry red spot' in a patient with Tay-Sachs disease: case report.

    Science.gov (United States)

    Aragão, Ricardo Evangelista Marrocos de; Ramos, Régia Maria Gondim; Pereira, Felipe Bezerra Alves; Bezerra, Andreya Ferreira Rodrigues; Fernandes, Daniel Nogueira

    2009-01-01

    Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzyme hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called 'cherry red spot' is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.

  12. George Washington: A Grounded Leader

    Science.gov (United States)

    2011-04-08

    placed in this book two years after the death of his father likely indicating he was now beginning to take possession of key documents that he...these truths would become part of the strong foundation upon which George would continue to build after the death ofhis father. As George moved into...men and women admired. He was both an I impressive horseman at a time when these talents truly counted, and became an extraordinarily graceful

  13. Kantowski-Sachs multidimensional cosmological models and dynamical dimensional reduction

    International Nuclear Information System (INIS)

    Demianski, M.; Rome Univ.; Golda, Z.A.; Heller, M.; Szydlowski, M.

    1988-01-01

    Einstein's field equations are solved for a multidimensional spacetime (KS) x Tsup(m), where (KS) is a four-dimensional Kantowski-Sachs spacetime and Tsup(m) is an m-dimensional torus. Among all possible vacuum solutions there is a large class of spacetimes in which the macroscopic space expands and the microscopic space contracts to a finite volume. We also consider a non-vacuum case and we explicitly solve the field equations for the matter satisfying the Zel'dovich equation of state. In non-vacuum models, with matter satisfying an equation of state p = γρ, O ≤ γ < 1, at a sufficiently late stage of evolution the microspace always expands and the dynamical dimensional reduction does not occur. (author)

  14. Atypical presentation of late-onset Tay-Sachs disease.

    Science.gov (United States)

    Deik, Andres; Saunders-Pullman, Rachel

    2014-05-01

    Late-onset Tay-Sachs disease (LOTS) is a lysosomal storage disease caused by deficient Beta-hexosaminidase A activity. We describe a 53-year-old woman who presented with adult-onset leg weakness, and whose initial diagnosis was progressive muscular atrophy without identifiable etiology. Development of cerebellar ataxia in mid-life prompted reassessment. Beta-hexosaminidase A quantification assay demonstrated absence of the isozyme. Genetic testing identified compound heterozygous mutations in the HEXA gene, confirming the diagnosis of LOTS. The phenotypic spectrum of LOTS includes motor neuronopathy, ataxia, choreoathetosis, neuropathy, and psychiatric symptoms in various combinations. This patient highlights the emergence of different clinical features over many years and emphasizes the need to consider LOTS in the differential diagnosis of progressive muscular atrophy. Copyright © 2013 Wiley Periodicals, Inc.

  15. Tay-Sachs disease: current perspectives from Australia.

    Science.gov (United States)

    Lew, Raelia M; Burnett, Leslie; Proos, Anné L; Delatycki, Martin B

    2015-01-01

    Tay-Sachs disease (TSD) is a fatal, recessively inherited neurodegenerative condition of infancy and early childhood. Although rare in most other populations, the carrier frequency is one in 25 in Ashkenazi Jews. Australian high-school-based TSD preconception genetic screening programs aim to screen, educate, and optimize reproductive choice for participants. These programs have demonstrated high uptake, low psychological morbidity, and have been shown to result in fewer than expected Jewish TSD-affected births over 18 years of operation. The majority of Jewish individuals of reproductive age outside of the high school screening program setting in Australia have not accessed screening. Recent recommendations advocate supplementing the community high school screening programs with general practitioner- and obstetrician-led genetic screening of Ashkenazi Jewish individuals for TSD and other severe recessive diseases for which this group is at risk. Massively parallel DNA sequencing is expected to become the testing modality of choice over the coming years.

  16. Tay-Sachs disease: current perspectives from Australia

    Directory of Open Access Journals (Sweden)

    Lew RM

    2015-01-01

    Full Text Available Raelia M Lew,1,7 Leslie Burnett,2,3,4 Anné L Proos,2 Martin B Delatycki5,6 1Department of Obstetrics and Gynecology, QEII Research Institute for Mothers and Infants, The University of Sydney, Australia; 2NSW Health Pathology North, Royal North Shore Hospital, St Leonards, Australia; 3SEALS, Prince of Wales Hospital, Randwick, Australia; 4Sydney Medical School-Northern, Royal North Shore Hospital E25, University of Sydney, Sydney, Australia; 5Department of Clinical Genetics, Austin Health, Heidelberg, Australia; 6Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Australia; 7Department of Obstetrics and Gynaecology, Dentistry and Health Sciences, Faculty of Medicine, The University of Melbourne, Melbourne, Australia Abstract: Tay-Sachs disease (TSD is a fatal, recessively inherited neurodegenerative condition of infancy and early childhood. Although rare in most other populations, the carrier frequency is one in 25 in Ashkenazi Jews. Australian high-school-based TSD preconception genetic screening programs aim to screen, educate, and optimize reproductive choice for participants. These programs have demonstrated high uptake, low psychological morbidity, and have been shown to result in fewer than expected Jewish TSD-affected births over 18 years of operation. The majority of Jewish individuals of reproductive age outside of the high school screening program setting in Australia have not accessed screening. Recent recommendations advocate supplementing the community high school screening programs with general practitioner- and obstetrician-led genetic screening of Ashkenazi Jewish individuals for TSD and other severe recessive diseases for which this group is at risk. Massively parallel DNA sequencing is expected to become the testing modality of choice over the coming years. Keywords: Tay-Sachs disease, genetic screening, Australia

  17. A tribute to George Plafker

    Science.gov (United States)

    Fuis, Gary S.; Haeussler, Peter J.; Atwater, Brian F.

    2015-01-01

    In a long and distinguished career, George Plafker made fundamental advances in understanding of megathrust tectonics, tsunami generation, paleoseismology, crustal neotectonics, and Alaskan geology, chiefly by means of geological field observations. George discovered that giant earthquakes result from tens of meters of seismic slip on subduction megathrusts, and he did this before the theory of plate tectonics had become a paradigm. The discovery was founded on George's comprehensive mapping of land-level changes in the aftermath of the 1964 earthquake in Alaska, and on his follow-up mapping, in 1968, in the region of the 1960 earthquakes in Chile. The mapping showed paired, parallel belts of coseismic uplift largely offshore and coseismic subsidence mostly onshore – a pattern now familiar as the initial condition assumed in simulations of subduction-zone tsunamis. George recognized, moreover, that splay faulting can play a major role in tsunami generation, and he also distinguished carefully between tectonic and landslide sources for the multiple tsunamis that accounted for nearly all the fatalities associated with the 1964 Alaska earthquake. George's classic monographs on the 1964 earthquake include findings on subduction-zone paleoseismology that he soon extended to include stratigraphic evidence for cyclic vertical deformation at the Copper River Delta, as well as recurrent uplift evidenced by flights of marine terraces at Middleton Island. As a geologist of earthquakes, George also clarified the tectonics and hazards of crustal faulting in Alaska, California, and other areas worldwide. All the while, George was mapping bedrock geology in Alaska, where he contributed importantly to today's understanding of how terranes were accreted and modified. Especially important was his documentation of the origin, movement, subduction, and collision of the Yakutat terrane in southern Alaska.

  18. Digital imaging of autoradiographs from paintings by Georges de La Tour (1593-1652)

    CERN Document Server

    Fischer, C O; Laurenze, C; Schmidt, C; Slusallek, K

    1999-01-01

    The artistic work of the painter Georges de La Tour has been studied very intensively in the last few years, mainly by French and US-American art historians and natural scientists. To support the in-depth analysis of two paintings from the Kimbell Art Museum in Fort Worth, Texas, USA, two similar paintings from the Gemaeldegalerie Berlin have been investigated. The method of neutron activation autoradiography has been applied using imaging plates with digital image processing.

  19. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    OpenAIRE

    Roldan, Omar

    2017-01-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instan...

  20. Segregation of Tay-Sachs and Sandhoff alleles in a non-Jewish family.

    OpenAIRE

    Lane, A B; Young, E; Jenkins, T

    1980-01-01

    A non-Jewish family is presented in which the genes for Tay-Sachs disease and Sandhoff disease are segregating. Individuals heterozygous for both alleles have low serum and white cell total hexosaminidase levels together with a proportion of heat-labile hexosaminidase A (HEX A) which falls in the normal range. The individuals would not be detected as carriers of Tay-Sachs disease or Sandhoff disease in a population screening program.

  1. Segregation of Tay-Sachs and Sandhoff alleles in a non-Jewish family.

    Science.gov (United States)

    Lane, A B; Young, E; Jenkins, T

    1980-01-01

    A non-Jewish family is presented in which the genes for Tay-Sachs disease and Sandhoff disease are segregating. Individuals heterozygous for both alleles have low serum and white cell total hexosaminidase levels together with a proportion of heat-labile hexosaminidase A (HEX A) which falls in the normal range. The individuals would not be detected as carriers of Tay-Sachs disease or Sandhoff disease in a population screening program. PMID:7446530

  2. Tay-Sach disease with "cherry-red spot"--first reported case in Malaysia.

    Science.gov (United States)

    Chan, L Y; Balasubramaniam, S; Sunder, R; Jamalia, R; Karunakar, T V N; Alagaratnam, J

    2011-12-01

    We present a rare case of Tay-Sachs disease with retinal 'cherry-red spots' in a 19-month-old Malay child. Molecular genetic studies confirmed the diagnosis. The case highlights that 'cherry-red spot' is a useful clinical clue in Tay-Sachs disease and several other lysosomal storage disorders. It serves as an ideal illustration of the eye as a window to inborn error of metabolism.

  3. In honour of Georges Charpak

    CERN Multimedia

    More than 40 years ago, Georges Charpak invented the multi-wire proportional chamber, which revolutionized the detection of particles and which found applications in medicine. He was awarded the Nobel Prize in physics for this invention in 1992. On March 9, there will be a conference here at CERN to celebrate Georges Charpak’s 85th birthday and to pay tribute to his groundbreaking work. After an introduction by Rolf Heuer, CERN Director-General, Georges Charpak will give a short speech by videoconference. This will be followed by a talk on the invention of the multi-wire proportional chamber by Ioanis Giomataris of CEA-Saclay. You are invited to attend this special conference in the Main Auditorium, from 4.00 pm to 6.00 pm: http://indico.cern.ch/conferenceDisplay.py?confId=53188

  4. George and the unbreakable code

    CERN Document Server

    Hawking, Lucy; Parsons, Gary

    2014-01-01

    George and his best friend Annie haven't had any space adventures for a while and they're missing the excitement. But not for long . . . Seriously strange things start happening. Banks are handing out free money; supermarkets can't charge for their produce so people are getting free food; and aircraft are refusing to fly. It looks like the world's biggest and best computers have all been hacked. George and Annie will travel further into space than ever before in order to find out who is behind it.

  5. George Sandi "Consuelo" / Ott Ojamaa

    Index Scriptorium Estoniae

    Ojamaa, Ott, 1926-1996

    2010-01-01

    Arvustus: Sand, George, pseud. Consuelo. 1-2. : romaan / [prantsuse keelest tõlkinud Leili-Maria Kask järelsõna ja märkused: Ott Ojamaa]. Tallinn : Eesti Riiklik Kirjastus, 1961. (Suuri sõnameistreid). 2. tr. ilmus 1988. a.

  6. A Post-Modern George.

    Science.gov (United States)

    Hunter, Robert S.

    2000-01-01

    Presents an art project in which students create postmodern portraits of George Washington in the style of Andy Warhol's pop-art portraits. Each portrait incorporates a fact and six symbols associated with Washington. Describes the project in detail and lists the materials and project objectives. (CMK)

  7. George Hampton (1920-2004)

    CERN Multimedia

    2004-01-01

    George Hampton, who died recently, was CERN's Director of Administration in the 1960s and an important member of the team who managed the growth of CERN as it left the construction period and became a world-class physics laboratory. George came to CERN in the 1963, when the laboratory was just starting its main research activities after the intense period of construction for the Proton Synchrotron (PS). At that time the laboratory was passing through a major budget crisis, and the new Director-general, Viki Weisskopf, was faced with a completely new structure set up by his predecessor John Adams, with 12 divisions for running the laboratory reporting directly to him, and with four Directors. With the renewal of the position of Director of Administration in 1963, Weisskopf selected George Hampton from candidates from the Member States. He came from the UK Atomic Energy Authority, but had worked earlier as a delegate to the international Civil Aviation Organization. George's position at the start was to hel...

  8. George Morrison: Anishinaabe Expressionist Artist

    Science.gov (United States)

    Vizenor, Gerald

    2006-01-01

    In this article, the author discusses the life and works of an Anishinaabe expressionist artist George Morrison. Morrison was an eminent expressionist painter with a singular romantic vision and an erudite sense of natural reason and liberty. He created an elusive shimmer of "endless space," the color and eternal motion of nature. The…

  9. Kantowski-Sachs Einstein-æther perfect fluid models

    Energy Technology Data Exchange (ETDEWEB)

    Latta, Joey [Department of Mathematics and Statistics, Dalhousie University, Halifax, Nova Scotia, B3H 3J5 (Canada); Leon, Genly [Instituto de Física, Pontificia Universidad de Católica de Valparaíso, Casilla 4950, Valparaíso (Chile); Paliathanasis, Andronikos, E-mail: lattaj@mathstat.dal.ca, E-mail: genly.leon@pucv.cl, E-mail: anpaliat@phys.uoa.gr [Instituto de Ciencias Físicas y Matemáticas, Universidad Austral de Chile, Valdivia (Chile)

    2016-11-01

    We investigate Kantowski-Sachs models in Einstein-æ ther theory with a perfect fluid source using the singularity analysis to prove the integrability of the field equations and dynamical system tools to study the evolution. We find an inflationary source at early times, and an inflationary sink at late times, for a wide region in the parameter space. The results by A.A. Coley, G. Leon, P. Sandin and J. Latta ( JCAP 12 (2015) 010), are then re-obtained as particular cases. Additionally, we select other values for the non-GR parameters which are consistent with current constraints, getting a very rich phenomenology. In particular, we find solutions with infinite shear, zero curvature, and infinite matter energy density in comparison with the Hubble scalar. We also have stiff-like future attractors, anisotropic late-time attractors, or both, in some special cases. Such results are developed analytically, and then verified by numerics. Finally, the physical interpretation of the new critical points is discussed.

  10. Fort Carson Wind Resource Assessment

    Energy Technology Data Exchange (ETDEWEB)

    Robichaud, R.

    2012-10-01

    This report focuses on the wind resource assessment, the estimated energy production of wind turbines, and economic potential of a wind turbine project on a ridge in the southeastern portion of the Fort Carson Army base.

  11. Effective gene therapy in an authentic model of Tay-Sachs-related diseases.

    Science.gov (United States)

    Cachón-González, M Begoña; Wang, Susan Z; Lynch, Andrew; Ziegler, Robin; Cheng, Seng H; Cox, Timothy M

    2006-07-05

    Tay-Sachs disease is a prototypic neurodegenerative disease. Lysosomal storage of GM2 ganglioside in Tay-Sachs and the related disorder, Sandhoff disease, is caused by deficiency of beta-hexosaminidase A, a heterodimeric protein. Tay-Sachs-related diseases (GM2 gangliosidoses) are incurable, but gene therapy has the potential for widespread correction of the underlying lysosomal defect by means of the secretion-recapture cellular pathway for enzymatic complementation. Sandhoff mice, lacking the beta-subunit of hexosaminidase, manifest many signs of classical human Tay-Sachs disease and, with an acute course, die before 20 weeks of age. We treated Sandhoff mice by stereotaxic intracranial inoculation of recombinant adeno-associated viral vectors encoding the complementing human beta-hexosaminidase alpha and beta subunit genes and elements, including an HIV tat sequence, to enhance protein expression and distribution. Animals survived for >1 year with sustained, widespread, and abundant enzyme delivery in the nervous system. Onset of the disease was delayed with preservation of motor function; inflammation and GM2 ganglioside storage in the brain and spinal cord was reduced. Gene delivery of beta-hexosaminidase A by using adeno-associated viral vectors has realistic potential for treating the human Tay-Sachs-related diseases.

  12. [Tay-Sachs disease in non-Jewish infant in Israel].

    Science.gov (United States)

    Nadim, Nasser

    2012-01-01

    Tay-Sachs disease, also known as GM2 gangliosidosis or Hexosaminidase A deficiency is an autosomal recessive genetic fatal disorder. The disease is known to appear in East European Ashkenazi Jews, North African Jews, and Quebec French Canadians exclusively, but, with different frequency and type of mutation. Its most common variant is the infantile type Tay-Sachs disease. Juvenile and late-onset forms of the disease are infrequent and slowly progressive. At nearly 3 to 6 months old, a baby with Tay-Sachs progressively loses his motor skills and attentiveness. Startle responses and hyperreflexia become prominent, especially on eliciting deep patellar and Achilles reflexes, as a consequence of neurodegeneration of the upper motor neuron. Other systemic damage ensues gradually; seizures, blindness, spasticity of limbs, inability to swallow and breathe, and eventually the baby dies at 1-4 years of age. All Tay-Sachs patients have a "cherry red spot", easily seen in the macula area of the retina, using an ophthalmoscope. The "cherry red spot" is the only normal part of the retina in these sick babies. The case presented here emphasizes that Tay-Sachs disease is sometimes misdiagnosed at first visits even by an experienced clinician, because of his lack of awareness that this disease is not exclusively a Jewish disease.

  13. Basic versus applied research: Julius Sachs (1832-1897) and the experimental physiology of plants.

    Science.gov (United States)

    Kutschera, Ulrich

    2015-01-01

    The German biologist Julius Sachs was the first to introduce controlled, accurate, quantitative experimentation into the botanical sciences, and is regarded as the founder of modern plant physiology. His seminal monograph Experimental-Physiologie der Pflanzen (Experimental Physiology of Plants) was published 150 y ago (1865), when Sachs was employed as a lecturer at the Agricultural Academy in Poppelsdorf/Bonn (now part of the University). This book marks the beginning of a new era of basic and applied plant science. In this contribution, I summarize the achievements of Sachs and outline his lasting legacy. In addition, I show that Sachs was one of the first biologists who integrated bacteria, which he considered to be descendants of fungi, into the botanical sciences and discussed their interaction with land plants (degradation of wood etc.). This "plant-microbe-view" of green organisms was extended and elaborated by the laboratory botanist Wilhelm Pfeffer (1845-1920), so that the term "Sachs-Pfeffer-Principle of Experimental Plant Research" appears to be appropriate to characterize this novel way of performing scientific studies on green, photoautotrophic organisms (embryophytes, algae, cyanobacteria).

  14. Nutritional advice from George Orwell

    DEFF Research Database (Denmark)

    Larsen, Morten Hedegaard

    2015-01-01

    is historical descriptions (in the form of excerpts) from George Orwell's 1937 book “The Road to Wigan Pier” on the living conditions of the British working classes. These descriptions will be aligned with results from contemporary research into nutritional behaviour. Strong similarities are identified between...... George Orwell's historical descriptions of the working-class's unhealthy diet and the findings from contemporary research into nutritional behaviour of people with a low socio-economic status. The article, on this basis, argues that certain social mechanisms influencing nutritional choices are readily...... identifiable across disciplines, and even partly reproduced in different historical, social and spatial contexts, with stronger negative (ill health) consequences for people with low socio-economic status especially. Finally the article discusses how social mechanisms affecting our nutritional choices could...

  15. Georg Cantor: habitar lo eterno

    OpenAIRE

    Penagos, Juan Manuel Rodríguez

    2002-01-01

    Este artículo pone en discusión la relación entre estructura psíquica y la producción lógica del delirio; para ello, parte de un matemático alemán llamado Georg Cantor. Aquí se discute la relación entre la producción matemática y la instalación de su psicosis. Este artigo coloca em discussão a relação entre estrutura psíquica e a produção lógica do delírio, partindo de um matemático alemão chamado Georg Cantor. Discutese ainda a relação entre a produção matemática e a instalação de sua psi...

  16. George and the blue moon

    CERN Document Server

    Hawking, Lucy

    2016-01-01

    George and his best friend, Annie have been selected as junior astronauts - part of a programme that trains up young people for a trip to Mars in the future. This is everything they've ever wanted - they get to be a part of up-to-the minute space discoveries and meet a bunch of new friends who are as fascinated by the universe as they are. But when they arrive at space camp, George and Annie quickly learn that strange things are happening - on Earth as well as up in our skies. Mysterious space missions are happening in secret, and the astronaut training they're undertaking gets scarier and scarier . . . The fifth adventure in this series by Lucy and Stephen Hawking - also containing up-to-the-minute scientific facts and information by the world's leading scientists.

  17. Georg Cantor: habitar lo eterno

    Directory of Open Access Journals (Sweden)

    Juan Manuel Rodríguez Penagos

    Full Text Available Este artículo pone en discusión la relación entre estructura psíquica y la producción lógica del delirio; para ello, parte de un matemático alemán llamado Georg Cantor. Aquí se discute la relación entre la producción matemática y la instalación de su psicosis.

  18. Georges Charpak street sign unveiled

    CERN Multimedia

    Paola Catapano

    2011-01-01

    While it might not be the only French street named in honour of the late Georges Charpak, who passed away in September 2010 at the age of 87, the street chosen by the mayor of Saint-Genis-Pouilly is certainly the only one located directly opposite the CERN "campus". The road overlooks buildings on the CERN Meyrin site, where Georges Charpak spent most of his career as a physicist, conducting the research that won him the Nobel Prize in Physics in 1992.   From left to right: Sigurd Lettow, Dominique Charpak and the mayor of Saint-Genis-Pouilly. The unveiling took place on 17 October and was organised by the mayor of Saint-Genis-Pouilly. George Charpak’s wife, Dominique, and Sigurd Lettow, CERN Director of Administration and General Infrastructure, attended what was an intimate and touching ceremony. The mayor’s speech at the event praised Georges’ commitment to scientific education. The highlight of the event, however, was a witty and humorous ...

  19. Georges et le big bang

    CERN Document Server

    Hawking, Lucy; Parsons, Gary

    2011-01-01

    Georges et Annie, sa meilleure amie, sont sur le point d'assister à l'une des plus importantes expériences scientifiques de tous les temps : explorer les premiers instants de l'Univers, le Big Bang ! Grâce à Cosmos, leur super ordinateur, et au Grand Collisionneur de hadrons créé par Éric, le père d'Annie, ils vont enfin pouvoir répondre à cette question essentielle : pourquoi existons nous ? Mais Georges et Annie découvrent qu'un complot diabolique se trame. Pire, c'est toute la recherche scientifique qui est en péril ! Entraîné dans d'incroyables aventures, Georges ira jusqu'aux confins de la galaxie pour sauver ses amis...Une plongée passionnante au coeur du Big Bang. Les toutes dernières théories de Stephen Hawking et des plus grands scientifiques actuels.

  20. Proton MR spectroscopy in three children with Tay-Sachs disease.

    Science.gov (United States)

    Aydin, Kubilay; Bakir, Baris; Tatli, Burak; Terzibasioglu, Ege; Ozmen, Meral

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM(2) gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease.

  1. Proton MR spectroscopy in three children with Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege [Istanbul University, Neuroradiology Division, Department of Radiology, Istanbul (Turkey); Tatli, Burak; Ozmen, Meral [Istanbul University, Department of Paediatric Neurology, Istanbul (Turkey)

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM{sub 2} gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  2. Proton MR spectroscopy in three children with Tay-Sachs disease

    International Nuclear Information System (INIS)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege; Tatli, Burak; Ozmen, Meral

    2005-01-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM 2 gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  3. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.

    Science.gov (United States)

    Kodama, Takashi; Togawa, Tadayasu; Tsukimura, Takahiro; Kawashima, Ikuo; Matsuoka, Kazuhiko; Kitakaze, Keisuke; Tsuji, Daisuke; Itoh, Kohji; Ishida, Yo-Ichi; Suzuki, Minoru; Suzuki, Toshihiro; Sakuraba, Hitoshi

    2011-01-01

    To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.

  4. [Sandhoff's and Tay-Sachs disease--based on our own cases].

    Science.gov (United States)

    Kokot, Witold; Raczyńska, Krystyna; Krajka-Lauer, Jarosława; Iwaszkiewicz-Bilikiewicz, Barbara; Wierzba, Jolanta

    2004-01-01

    The authors described two infant with Sandhoff's and Tay-Sachs disease. Tay-Sachs disease is well-known inherited disease leading to an accumulation of gangliosides in the brain and retina. Sandhoffs disease (GM2 gangliosidosis type 0) was diagnosed in an infant, in whom a progressive neurological disorder and cherry-red foveal spots were developed. In addition, to the general clinical examination, indirect ophthalnoscopy, blood white cells enzymatic examination are used to make definitive diagnosis. In this cases, the early eye fundus examination allowed us to make the proper diagnosis. The fundus change is characterized by the "cherry-red spot" in the central area.

  5. Planck 2015 results: XXI. The integrated Sachs-Wolfe effect

    International Nuclear Information System (INIS)

    Ade, P. A. R.; Aghanim, N.; Arnaud, M.; Ashdown, M.; Aumont, J.

    2016-01-01

    Here, this paper presents a study of the integrated Sachs-Wolfe (ISW) effect from the Planck 2015 temperature and polarization data release. This secondary cosmic microwave background (CMB) anisotropy caused by the large-scale time-evolving gravitational potential is probed from different perspectives. The CMB is cross-correlated with different large-scale structure (LSS) tracers: radio sources from the NVSS catalogue; galaxies from the optical SDSS and the infrared WISE surveys; and the Planck 2015 convergence lensing map. The joint cross-correlation of the CMB with the tracers yields a detection at 4σ where most of the signal-to-noise is due to the Planck lensing and the NVSS radio catalogue. In fact, the ISW effect is detected from the Planck data only at ≈3σ (through the ISW-lensing bispectrum), which is similar to the detection level achieved by combining the cross-correlation signal coming from all the galaxy catalogues mentioned above. We study the ability of the ISW effect to place constraints on the dark-energy parameters; in particular, we show that Ω Λ is detected at more than 3σ. This cross-correlation analysis is performed only with the Planck temperature data, since the polarization scales available in the 2015 release do not permit significant improvement of the CMB-LSS cross-correlation detectability. Nevertheless, the Planck polarization data are used to study the anomalously large ISW signal previously reported through the aperture photometry on stacked CMB features at the locations of known superclusters and supervoids, which is in conflict with ΛCDM expectations. We find that the current Planck polarization data do not exclude that this signal could be caused by the ISW effect. In addition, the stacking of the Planck lensing map on the locations of superstructures exhibits a positive cross-correlation with these large-scale structures. Finally, we have improved our previous reconstruction of the ISW temperature fluctuations by combining

  6. NSU Art Museum Fort Lauderdale | Art Museum in Fort Lauderdale

    Science.gov (United States)

    NSU Art Museum Fort Lauderdale Visit Admissions Hours & Admission Policies & Accessibility Airports Shop & Dine About the Café & Store Store Café Menu Art Exhibitions Currently on View Thursday 2-for-1 specials on wine and craft beer in the Museum Café, and hands-on art projects for all

  7. Black Hole Entropy from Bondi-Metzner-Sachs Symmetry at the Horizon.

    Science.gov (United States)

    Carlip, S

    2018-03-09

    Near the horizon, the obvious symmetries of a black hole spacetime-the horizon-preserving diffeomorphisms-are enhanced to a larger symmetry group with a three-dimensional Bondi-Metzner-Sachs algebra. Using dimensional reduction and covariant phase space techniques, I investigate this augmented symmetry and show that it is strong enough to determine the black hole entropy in any dimension.

  8. Shock absorption of below-knee prostheses : A comparison between the SACH and the Multiflex foot

    NARCIS (Netherlands)

    van Leeuwen, J. L.; Speth, L. A W M; Daanen, H. A M

    1990-01-01

    Shock waves were measured during walking on a treadmill on the metal tube of a below-knee KBM prosthesis, provided either with a SACH foot or with a Multiflex foot. Accelerations were measured in the axial direction and the dorso-ventral direction, about 160 mm proximal to the sole of the shoe. The

  9. 78 FR 24447 - Goldman Sachs Trust, et al.; Notice of Application

    Science.gov (United States)

    2013-04-25

    ... SECURITIES AND EXCHANGE COMMISSION [Investment Company Act Release No. 30471; 812-14075] Goldman Sachs Trust, et al.; Notice of Application April 19, 2013. AGENCY: Securities and Exchange Commission..., or cause more than 10% of the acquired company's voting stock to be owned by investment companies and...

  10. Tay-Sachs disease: high gene frequency in a non-Jewish population.

    Science.gov (United States)

    Kelly, T E; Chase, G A; Kaback, M M; Kumor, K; McKusick, V A

    1975-01-01

    A non-Amish "Pennsylvania Dutch" semi-isolate was found to have a high frequency of Tay-Sachs gene. This high frequency could be ascribed to founder effect and may represent, in microcosm, how this mechanism could have produced the high gene frequency among Ashkenazi Jews. PMID:803011

  11. Late-onset Tay-Sachs disease: adverse effects of medications and implications for treatment.

    Science.gov (United States)

    Shapiro, B E; Hatters-Friedman, S; Fernandes-Filho, J A; Anthony, K; Natowicz, M R

    2006-09-12

    The authors conducted a retrospective and brief prospective study of adverse effects of approximately 350 medications in 44 adults with late-onset Tay-Sachs disease (LOTS). Some medications were relatively safe, whereas others, particularly haloperidol, risperidone, and chlorpromazine, were associated with neurologic worsening.

  12. A pseudodeficiency allele common in non-Jewish Tay-Sachs carriers: Implications for carrier screening

    Energy Technology Data Exchange (ETDEWEB)

    Triggs-Raine, B.L.; Akerman, B.R.; Gravel, R.A. (McGill Univ.-Montreal Children' s Hospital Research Institute, Montreal, Quebec (Canada)); Mules, E.H.; Thomas, G.H.; Dowling, C.E. (Johns Hopkins School of Medicine, Baltimore, MD (United States)); Kaback, M.M.; Lim-Steele, J.S.T. (Univ. of California, San Diego, CA (United States)); Natowicz, M.R. (Eunice Kennedy Shriver Center for Mental Retardation, Waltham, MA (United States)); Grebner, E.E. (Thomas Jefferson Univ., Philadelphia, PA (United States)); Navon, R.R. (Tel-Aviv Univ., Kfar-Sava (Israel)); Welch, J.P. (Dalhousie Univ., Halifax, Nova, Scotia (Canada)); Greenberg, C.R. (Univ. of Manitoba, Winnipeg (Canada))

    1992-10-01

    Deficiency of [beta]-hexosaminidase A (Hex A) activity typically results in Tay-Sachs disease. However, healthy subjects found to be deficient in Hex A activity (i.e., pseudodeficient) by means of in vitro biochemical tests have been described. The authors analyzed the HEXA gene of one pseudodeficient subject and identified both a C[sub 739]-to-T substitution that changes Arg[sub 247][yields]Trp on one allele and a previously identified Tay-Sachs disease mutation of the second allele. Six additional pseudodeficient subjects were found to have the C[sub 739]-to-T but for none of 36 Jewish enzyme-defined carries who did not have one of three known mutations common to this group. The C[sub 739]-to-T allele, together with a [open quotes]true[close quotes] Tay-Sachs disease allele, causes Hex A pseudodeficiency. Given both the large proportion of non-Jewish carriers with this allele and that standard biochemical screening cannot differentiate between heterozygotes for the C[sub 739]-to-T mutations and Tay-Sachs disease carriers, DNA testing for this mutation in at-risk couples is essential. This could prevent unnecessary or incorrect prenatal diagnoses. 40 refs., 3 figs., 4 tabs.

  13. Energy in the Kantowski–Sachs space-time using teleparallel ...

    Indian Academy of Sciences (India)

    Inflation is again considered to be one of the early phases of the Universe [10]. For testing gravitational energy–momentum tensor of the teleparallel theory in quasilocal approach and also in the. Hamiltonian structure of TEGR, it is intended to calculate the total energy of inflationary. Kantowski–Sachs-type Universe. Again ...

  14. Outcomes of arthroscopic "Remplissage": capsulotenodesis of the engaging large Hill-Sachs lesion

    Directory of Open Access Journals (Sweden)

    Mayo Lee

    2011-06-01

    Full Text Available Abstract Background A Hill-Sachs lesion of the humeral head after a shoulder dislocation is clinically insignificant in most cases. However, a sizable defect will engage with the anterior rim of the glenoid and cause instability even after anterior glenoid reconstruction. The purpose of this study was to evaluate the outcome of arthroscopic capsulotenodesis of the posterior capsule and infraspinatus tendon ("remplissage" to seal a large engaging Hill-Sachs lesion in an unstable shoulder. Methods This was a prospective follow-up study of patients who underwent arthroscopic surgery for recurrent shoulder instability with a large engaging Hill-Sachs lesion from 2007 to 2009. The clinical results were measured preoperatively and postoperatively with the Simple Shoulder test (SST and the Rowe score for instability. Results Eleven patients met the inclusion criteria of this study. The mean follow-up time was 30 months (range 24 to 35 months. At the last follow-up, significant improvement was observed in both scores with no recurrent dislocations. The mean SST improved from 6.6 to 11 (p Conclusions Arthroscopic remplissage for shoulder instability is an effective soft tissue technique to seal a large engaging Hill-Sachs lesion with respect to recurrence rate, range of motion and shoulder function.

  15. Fort Saint Vrain operational experience

    International Nuclear Information System (INIS)

    Fuller, C.H.

    1989-01-01

    Fort St. Vrain (FSV), on the system of the Public Service Company of Colorado, is the only high temperature gas-cooled (HTGR) power reactor in the United States. The plant features a helium-cooled reactor with a uranium-thorium fuel cycle. The paper describes the experience made during its operation. (author). 2 refs, 4 figs, 2 tabs

  16. Three-dimensional computed tomography measurement accuracy of varying Hill-Sachs lesion size.

    Science.gov (United States)

    Ho, Anthony; Kurdziel, Michael D; Koueiter, Denise M; Wiater, J Michael

    2018-02-01

    The glenoid track concept has been proposed to correlate shoulder stability with bone loss. Accurate assessment of Hill-Sachs lesion size preoperatively may affect surgical planning and postoperative outcomes; however, no measurement method has been universally accepted. This study aimed to assess the accuracy and reliability of measuring Hill-Sachs lesion sizes using 3-dimensional (3D) computed tomography (CT). Nine polyurethane humerus bone substitutes were used to create Hill-Sachs lesions of varying sizes with a combination of lesion depth (shallow, intermediate, and deep) and width (small, medium, and large). Specimens were scanned with a clinical CT scanner for size measurements and a micro-CT scanner for measurement of true lesion size. Six evaluators repeated measurements twice in a 2-week interval. Scans were measured by use of 3D CT reconstructions for length, width, and Hill-Sachs interval and with use of 2D CT for depth. The interclass correlation coefficient evaluated interobserver and intraobserver variability and percentage error, and Student t-tests assessed measurement accuracy. Interclass correlation coefficient reliability demonstrated strong agreement for all variables measured (0.856-0.975). Percentage error between measured length and measured depth and the true measurement significantly varied with respect to both lesion depth (P = .003 and P = .005, respectively) and lesion size (P = .049 and P = .004, respectively). The 3D CT imaging is effective and reproducible in determining lesion size. Determination of Hill-Sachs interval width is also reliable when it is applied to the glenoid track concept. Measured values on 3D and 2-dimensional imaging using a conventional CT scanner may slightly underestimate true measurements. Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  17. Sachês antimicrobianos em pós-colheita de manga

    Directory of Open Access Journals (Sweden)

    Eber Antonio Alves Medeiros

    2011-10-01

    Full Text Available Objetivou-se com este trabalho desenvolver sachês incorporados com óleos essenciais avaliando as propriedades físico-químicas e microbiológicas de mangas armazenadas em saco de papel contendo estes sachês em seu interior. Os óleos essenciais de orégano (Origanum vulgaris e capim-limão (Cymbopogon citratus foram incorporados em saches, e sua atividade antimicrobiana foi testada nos fungos Colletotrichum gloeosporides, Lasiodiplodia theobromae, Xanthomonas campestris pv. mangiferae indica, Alternaria alternata. Frutos de manga 'Tommy Atkins' foram acondicionados individualmente em sacos de papel contendo em seu interior um sachê antimicrobiano e mantidos a 25 °C ± 2 °C e UR 80% ± 5% por nove dias. Os parâmetros cor da epiderme, firmeza de polpa, sólidos solúveis, acidez titulável e pH sofreram pouca influência dos óleos essenciais de orégano e capim limão, indicando que a presença do óleo essencial não altera as características físico-químicas da polpa de manga. Os sachês ativos incorporados com óleos essenciais de orégano e capim limão apresentaram controle no crescimento dos microrganismos testados, sendo o capim-limão mais eficiente, reduzindo em aproximadamente 2 ciclos Log a contagem de mesófilos aeróbios e fungos filamentosos e leveduras em relação ao tratamento controle empregado.

  18. Georges et le code secret

    CERN Document Server

    Hawking, Lucy; Parsons, Gary

    2015-01-01

    À Foxbridge, Georges et Annie s'ennuient, ils rêvent de repartir dans l'espace pour de nouvelles aventures intergalactiques. Pourtant, sur la terre ferme, il se passe des choses étranges... L'argent s'envole des distributeurs de billets, les avions refusent de décoller et les caisses enregistreuses des supermarchés ne fonctionnent plus... Quel chaos ! Il semblerait qu'un bug planétaire ait déréglé tous les systèmes informatiques. S'ils veulent découvrir ce qui se cache derrière tout ça, les deux amis devront voyager plus loin que jamais dans l'espace...

  19. Appendices for Fort George G. Meade Active Sanitary Landfill and Clean Fill Dump Remedial Investigation Report

    Science.gov (United States)

    1992-12-01

    3O VLYqq Time: P7 /0 Well Condition.: Well Diameter (inches): L4 U Odor (describe): Sounding Method: tW L= . . Measurement Reference: ’TOC Pv Stick... acca (f:):~ (=in)-__ --- 13.10 L AP Siium i=; ’are: _______________ Commane n ds Cbserwvuic-n: ______________________ 507 31 ,;IL 3: iL8.15 ’a~~l !o

  20. Environmental Impact Statement Addressing Campus Development at Fort George Meade, Maryland

    Science.gov (United States)

    2010-09-01

    Dumetella carolinensis), mockingbird (Mimus polyglyottos), Carolina chickadee (Poecile carolinensis), Carolina wren (Thryothorus ludovicianus), house wren...regulations, the MBTA makes it unlawful to pursue, hunt, take, capture, or kill ; attempt to take, capture or kill ; possess, offer to sell, barter...foreign country, any bird, part, nest, or egg that was captured, killed , taken, shipped, transported, or carried contrary to the laws from where it was

  1. Fort George G. Meade Active Sanitary Landfill and Clean Fill Dump, Remedial Investigation Report

    National Research Council Canada - National Science Library

    1992-01-01

    ...) and a Site Inspection at the inactive Clean Fill Dump (CFD) sites. The objective of the ASL study involved obtaining additional data to evaluate the potential environmental impacts associated with the landfill operations...

  2. Georges Charpak, Nobel Physics Prize 1992

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    1992-12-15

    Wednesday 14 October looked like being a day like any other for detector specialist Georges Charpak. Except he had an unwelcome appointment with the dentist early that afternoon. Late that morning he was able to telephone to cancel the appointment. 'I have a small problem...', he explained. The problem was the announcement that Georges Charpak receives 1992's most prestigious award for physics.

  3. George A. Olah, Carbocation and Hydrocarbon Chemistry

    Science.gov (United States)

    dropdown arrow Site Map A-Z Index Menu Synopsis George A. Olah, Carbocation and Hydrocarbon Chemistry George Olah received the 1994 Nobel Prize in Chemistry "for his contribution to carbocation chemistry" and his 'role in the chemistry of hydrocarbons. In particular, he developed superacids

  4. Von Braun Rocket Team at Fort Bliss, Texas

    Science.gov (United States)

    1940-01-01

    The German Rocket Team, also known as the Von Braun Rocket Team, poses for a group photograph at Fort Bliss, Texas. After World War II ended in 1945, Dr. Wernher von Braun led some 120 of his Peenemuende Colleagues, who developed the V-2 rocket for the German military during the War, to the United Sttes under a contract to the U.S. Army Corps as part of Operation Paperclip. During the following five years the team worked on high altitude firings of the captured V-2 rockets at the White Sands Missile Range in New Mexico, and a guided missile development unit at Fort Bliss, Texas. In April 1950, the group was transferred to the Army Ballistic Missile Agency (ABMA) at Redstone Arsenal in Huntsville, Alabama, and continued to work on the development of the guided missiles for the U.S. Army until transferring to a newly established field center of the National Aeronautic and Space Administration (NASA), George C. Marshall Space Flight Center (MSFC).

  5. The frequency of Tay-Sachs disease causing mutations in the Brazilian Jewish population justifies a carrier screening program

    Directory of Open Access Journals (Sweden)

    Roberto Rozenberg

    Full Text Available CONTEXT: Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. OBJECTIVE: To evaluate the importance of a Tay-Sachs disease carrier screening program in the Brazilian Jewish population by determining the frequency of heterozygotes and the acceptance of the program by the community. SETTING: Laboratory of Molecular Genetics - Institute of Biosciences - Universidade de São Paulo. PARTICIPANTS: 581 senior students from selected Jewish high schools. PROCEDURE: Molecular analysis of Tay-Sachs disease causing mutations by PCR amplification of genomic DNA, followed by restriction enzyme digestion. RESULTS: Among 581 students that attended educational classes, 404 (70% elected to be tested for Tay-Sachs disease mutations. Of these, approximately 65% were of Ashkenazi Jewish origin. Eight carriers were detected corresponding to a carrier frequency of 1 in every 33 individuals in the Ashkenazi Jewish fraction of the sample. CONCLUSION: The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.

  6. The frequency of Tay-Sachs disease causing mutations in the Brazilian Jewish population justifies a carrier screening program.

    Science.gov (United States)

    Rozenberg, R; Pereira, L da V

    2001-07-05

    Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. To evaluate the importance of a Tay-Sachs disease carrier screening program in the Brazilian Jewish population by determining the frequency of heterozygotes and the acceptance of the program by the community. Laboratory of Molecular Genetics--Institute of Biosciences--Universidade de São Paulo. 581 senior students from selected Jewish high schools. Molecular analysis of Tay-Sachs disease causing mutations by PCR amplification of genomic DNA, followed by restriction enzyme digestion. Among 581 students that attended educational classes, 404 (70%) elected to be tested for Tay-Sachs disease mutations. Of these, approximately 65% were of Ashkenazi Jewish origin. Eight carriers were detected corresponding to a carrier frequency of 1 in every 33 individuals in the Ashkenazi Jewish fraction of the sample. The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.

  7. Ecological Baseline, Fort Hood, Texas

    Science.gov (United States)

    1980-08-01

    cedar eTm (Uiimus crassifolia), Texas ash (Fraxinus texansis), and Texas persimmon ( Diospyros texana). Conversely, the two predominant tree species...Ilex decidua), Mex- ican buckeye (Ungnadia spjeciosa), and Texas persimmon ( Diospyros texana). Vines included greenbrier (Smilax bona-nox) and white...Hedgehey Cactus (Echinocereus sp.) has been observed on Fort Hood. Due to the brief period of flowering for this genus , the individual species were not

  8. Inventory of Forts in Indonesia

    Science.gov (United States)

    Rinandi, N.; Suryaningsih, F.

    2015-08-01

    The great archipelago in Indonesia with its wealthy and various nature, the products and commodities of tropic agriculture and the rich soil, was through the centuries a region of interest for other countries all over the world. For several reasons some of these countries came to Indonesia to establish their existence and tried to monopolize the trading. These countries such as the Portuguese, the Spanish, the Dutch and the British built strengthened trade stations which later became forts all over Indonesia to defend their interest. The archipelago of Indonesia possesses a great number of fortification-works as legacies of native rulers and those which were built by European trading companies and later became colonial powers in the 16th to the 19th centuries. These legacies include those specific structures built as a defence system during pre and within the period of World War II. These fortresses are nowadaysvaluable subjects, because they might be considered as shared heritage among these countries and Indonesia. It's important to develop a vision to preserve these particular subjects of heritage, because they are an interesting part of the Indonesian history and its cultural treasures. The Government of the Republic of Indonesia has national program to compile a comprehensive documentation of the existing condition of these various types of forts as cultural heritage. The result of the 3 years project was a comprehensive 442 forts database in Indonesia, which will be very valuable to the implementation of legal protection, preservation matters and adaptive re-use in the future.

  9. George Mandler (1924-2016).

    Science.gov (United States)

    Craik, Fergus

    2017-10-01

    Presents an obituary for George Mandler, who died in London on May 6, 2016 at the age of 91. Mandler was one of the pioneers of the cognitive revolution in psychology. He was instrumental in moving the study of human learning from notions based largely on associations to a view of memory as an organized, nested hierarchical structure. Mandler was also a major proponent of the dual-process theory of recognition memory, in which general feelings of familiarity are distinguished from the context-rich experience of recollection. He brought the study of emotion into prominence, suggesting how emotion and cognition are related. Finally, he repatriated the concept of consciousness from its intellectual exile under behaviorism, stating boldly in 1975 that the construct was respectable, useful, and probably necessary. Mandler edited the Psychological Review from 1970 to 1976, chaired the Governing Board of the Psychonomic Society, and was president of APA Divisions 1 (General Psychology) and 3 (Experimental Psychology and Cognitive Science). (PsycINFO Database Record (c) 2017 APA, all rights reserved).

  10. Georges Charpak: 1924-2010

    CERN Multimedia

    2010-01-01

    All of CERN was deeply saddened to learn the news that our friend and colleague, Georges Charpak, passed away on 29 September 2010.   There are few people who can honestly be said to have changed the world, but Charpak was one of them. Born in Dabrovika, Poland on 8 March 1924, Charpak fled the Nazi regime under a false identity and moved to France where he joined the resistance. Arrested and deported to Dachau, he returned to France after the war and took French nationality in 1946. A student of Frédéric Joliot-Curie at the Collège de France, he joined CERN in 1959, just five years after the Organization’s foundation, and he certainly left his mark. From the start, Charpak applied himself to the development of new particle detector techniques. His outstanding and pioneering efforts revolutionised particle physics, taking the field into the electronic age. It is fair to say that without the developments pioneered by him, particularly the invention of t...

  11. Georges Bank review studies list

    International Nuclear Information System (INIS)

    1999-01-01

    Federal and provincial legislation placed a moratorium on petroleum exploration and drilling on Georges Bank off southwestern Nova Scotia in 1988. Since that time, a public review was conducted to determine the environmental and socio-economic impacts of petroleum exploration on the area. Several consultant groups have been involved and prepared reports for the consideration of the Minister of Natural Resources Canada and the Minister for the Nova Scotia Department of Natural Resources. One of the consultant groups, CEF Consultants Ltd. of Halifax, Nova Scotia conducted a study which resulted in a series of papers on energy and the offshore, dealing with : (1) hydrocarbon resources, (2) exploring for offshore oil and gas, (3) offshore production, storage and transportation, (4) offshore regulation, (5) the energy context, (6) (study not available), (7) the global environmental implications of new hydrocarbon developments, and (8) communities and change. Other studies were conducted by other groups of consultants involving winds, larvae, ecosystems, an economic profile, and a historical overview of offshore and and gas development. The Ministers of Natural Resources Canada and Nova Scotia Department of Natural Resources are required to make a decision regarding the public review and moratorium by January 1, 2000

  12. GEORG CANTOR’UN SONSUZLARI

    OpenAIRE

    GÜNEY, Zekeriya; KORKMAZ, Nebiye

    2014-01-01

     Özet Georg Cantor, kümeler kuramı ve matematiksel biçimciliğin öncüsüdür. Cantor’un özellikle sonsuzluklarla ilgili çalışmaları matematikte devrim niteliğindedir ve başta topoloji olmak üzere matematiğin çeşitli alanlarında önemli gelişmelere yol açmıştır. Cantor Kuramı’nın pür matematiksel karakteri, matematiğe ve matematik eğitimine yeni bir bakış açısı getirmiş ve matematiğin asıl eğitimsel öneminin, bir zihin jimnastiği olmasından geldiği daha iyi anlaşılır olmuştur. Matematiğin, herhang...

  13. George Combe and common sense.

    Science.gov (United States)

    Dyde, Sean

    2015-06-01

    This article examines the history of two fields of enquiry in late eighteenth- and early nineteenth-century Scotland: the rise and fall of the common sense school of philosophy and phrenology as presented in the works of George Combe. Although many previous historians have construed these histories as separate, indeed sometimes incommensurate, I propose that their paths were intertwined to a greater extent than has previously been given credit. The philosophy of common sense was a response to problems raised by Enlightenment thinkers, particularly David Hume, and spurred a theory of the mind and its mode of study. In order to succeed, or even to be considered a rival of these established understandings, phrenologists adapted their arguments for the sake of engaging in philosophical dispute. I argue that this debate contributed to the relative success of these groups: phrenology as a well-known historical subject, common sense now largely forgotten. Moreover, this history seeks to question the place of phrenology within the sciences of mind in nineteenth-century Britain.

  14. Investigation on Adaptive Re-use of Heritage Building in George Town, Penang

    Directory of Open Access Journals (Sweden)

    Marhamah Abdul Hadi

    2013-09-01

    Full Text Available Adaptive re-use of heritage buildings in George Town has gained attention from their owners. Their owners either private owners or government, want to adapt their respective buildings to new usage for instance to become a gallery, museum, restaurant, boutique hotel and many more. Every heritage building that is being adapted to paper main objective is to identify the changes made in terms of structure, space and material when adapting the heritage buildings to a new usage specifically into a gallery. Two heritage buildings are chosen as case studies for this paper which are Rumah Teh Bunga and Fort Cornwallis; both buildings located in Penang, Malaysia. The changes made to these two buildings were analyzed using the guidelines provided which are Guideline for Conservation areas and Heritage Buildings and National Heritage Acts 2005. Both buildings will be analyzed using National Heritage Acts, while only Fort Cornwallis will be analyzed using Guideline for Conservation areas and Heritage Buildings. Adaptation of these two heritage buildings requires changes in structure, space and material. The changes in Rumah Teh Bunga focuses more on materials and space while changes in Fort Cornwallis emphasize more to space and addition of other structures. Analysis on the changes are made by using the guideline provided, most of the changes made to both of these heritage buildings comply the rules and regulations stated in the guideline. It was found from the data that some of the reasons on why the owner change Rumah Teh Bunga to gallery are because of its complicated procedure that involves in privatization of this building to other owner and the need to promote the heritage significance of this building to the public. As for Fort Cornwallis, the adaptation is more on strengthening its value as a fort and becoming a tourist attraction.

  15. CONARC Training Workshop, Fort Gordon, Georgia, 5-7 October 1971. Volume I. General: Opening, Closing, and Dinner Sessions.

    Science.gov (United States)

    1971-10-01

    Jack and Bobby Kennedy, Jim Plunkett, George Jackson, the Beatles , Janice Joplin, and the growing number of men who have walked on the moon. It...Executive Summary as an Interim Re- port on the CONARC Training Workshop, which was held 5-7 October 1971 at Fort Gordon, Georgia. 3. The report will be...volume will be sent to each addressee to form two complete sets of the report. Requests for additional copies of the separate volumes will be filled

  16. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    Energy Technology Data Exchange (ETDEWEB)

    Roldan, Omar, E-mail: oaroldan@if.ufrj.br [Instituto de Física, Universidade Federal do Rio de Janeiro, 21941-972, Rio de Janeiro, RJ (Brazil)

    2017-08-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instance, facilitate the search for primordial non-Gaussianity in future works. It also disentangles the non-linear ISW from other effects. Finally, we provide a method which can iteratively be used to obtain the lensing solution at the desired order.

  17. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    International Nuclear Information System (INIS)

    Roldan, Omar

    2017-01-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instance, facilitate the search for primordial non-Gaussianity in future works. It also disentangles the non-linear ISW from other effects. Finally, we provide a method which can iteratively be used to obtain the lensing solution at the desired order.

  18. Role of the physician in screening for carriers of Tay-Sachs disease.

    Science.gov (United States)

    Lowden, J. A.

    1978-01-01

    A screening test for carriers of Tay-Sachs disease has been available in Toronto for more than 6 years. In that time more than 11 000 Jewish residents have been tested. Most had requested testing after hearing about the screening program from friends or the media; few had been advised by their physicians to be tested. To sample the attitudes of physicians in Toronto towards carrier screening, we studied questionnaire responses of 42 physicians whose practices were composed largely of Jewish patients. Only 31% regularly advised their young adult Jewish patients to have a carrier screening test but 76% said they had patients who asked if they should be tested. Of the 14 (33%) who had had one or more patients with Tay-Sachs disease 6 did not advise carrier testing. There was a positive correlation between specialty training and support for the screening program. Methods for increasing physician advocacy of these programs are discussed. PMID:709448

  19. Neurocognitive testing in late-onset Tay-Sachs disease: a pilot study.

    Science.gov (United States)

    Elstein, D; Doniger, G M; Simon, E; Korn-Lubetzki, I; Navon, R; Zimran, A

    2008-08-01

    To test neurocognitive function in patients with late-onset Tay-Sachs disease (LOTS) using a computerized system to assess whether cognition is a clinically relevant outcome measure of possible therapeutic intervention in LOTS. Ten adults with Tay-Sachs disease were administered at least one battery of the Mindstreams Neurotrax system for evaluation of cognitive function. Six sub-scores and a Global Cognitive Score (GCS) were tabulated. A disease specific severity score was also devised with six domains. Despite identical genotypes, all patients but the two oldest had > or = 3/6 sub-scores one standard deviation below normal mean (100); verbal and executive functions were most affected. The severity score measured other functions. Because of provocative findings on re-testing in patients exposed to miglustat, and despite the very small cohort, cognitive function may be an appropriate and clinically relevant outcome measure for future therapeutic interventions in LOTS.

  20. Quantum field theory of the universe in the Kantowski-Sachs space-time

    International Nuclear Information System (INIS)

    Shen, Y.; Tan, Z.

    1996-01-01

    In this paper, the quantum field theory of the universe in the Kantowski-Sachs space-time is studied. An analogue of proceedings in quantum field theory is applied in curved space-time to the Kantowski-Sachs space-time, obtaining the wave function of the universe satisfied the Wheeler-DeWitt equation. Regarding the wave function as a universe field in the minisuperspace, the authors can not only overcome the difficulty of the probabilistic interpretation in quantum cosmology, but also come to the conclusion that there is multiple production of universes. The average number of the produced universes from nothing is calculated. The distribution of created universe is given. It is the Planckian distribution

  1. Diagnosis and molecular characterization of non-classic forms of Tay-Sachs disease in Brazil.

    Science.gov (United States)

    Rozenberg, R; Kok, F; Burin, M G; Sá Miranda, M C; Vasques, C; Henriques-Souza, A M M; Giugliani, R; Vainzof, Mariz; Pereira, L V

    2006-06-01

    Molecular analysis of five Brazilian families, including eight patients presenting with nonclassic Tay-Sachs disease, was performed to identify frequent causative mutations and their correlation with clinical course. Three patients were affected by the B1 subacute variant and were shown to carry the R178H mutation (the DN allele), which is also common among Portuguese patients. Two of them were compound heterozygotes, whereas the third presented with the mutation in both alleles. Since Brazil was a Portuguese colony for over two centuries, common ancestry might be the probable explanation. The fourth patient presented with a juvenile phenotype and carries the R499H mutation, which has been reported only once worldwide and is associated with residual enzyme activity, responsible for a slower clinical course. The fifth family, of an Ashkenazi Jewish background, showed an extensive intrafamilial clinical variability among three affected sibs presenting with muscle atrophy, ataxia, and psychiatric symptoms. They were first diagnosed as having atypical spinal muscular atrophy and, subsequently, spinocerebellar ataxia, but, recently, the diagnosis of late-onset Tay-Sachs disease was confirmed based on reduced plasma hexosaminidase A activity and the G269S/InsTATC1278 genotype. It is therefore highly recommended to test patients with a similar clinical history for Tay-Sachs disease. In the same family, one first cousin committed suicide at the age of 24 years, presenting with a clinical phenotype that suggested an undiagnosed case and highlighting the effect of the intrafamilial clinical variability in delaying a prompt diagnosis. It is now recognized that his parents are, in fact, a carrier couple. Additionally, another relative had been previously identified as a heterozygote in a Tay-Sachs disease screening program, but the information was not shared among the family. Since this information might anticipate diagnosis and genetic counseling, it is advisable that

  2. Substrate reduction therapy in the infantile form of Tay-Sachs disease.

    Science.gov (United States)

    Bembi, B; Marchetti, F; Guerci, V I; Ciana, G; Addobbati, R; Grasso, D; Barone, R; Cariati, R; Fernandez-Guillen, L; Butters, T; Pittis, M G

    2006-01-24

    Substrate reduction therapy (SRT) with miglustat has been proposed for treatment of some lysosomal storage disorders. Based on the positive experience in Gaucher disease and experimental data in Tay-Sachs (TSD) and Sandhoff animal models, the authors investigated the clinical efficacy of SRT in two patients with infantile TSD. SRT could not arrest the patients' neurologic deterioration. However, a significant drug concentration in CSF as well as macrocephaly prevention were observed.

  3. ACOG committee opinion. Number 318, October 2005. Screening for Tay-Sachs disease.

    Science.gov (United States)

    2005-10-01

    Tay-Sachs disease (TSD) is a severe progressive neurologic disease that causes death in early childhood. Carrier screening, should be offered before pregnancy to individuals and couples at high-risk, including those of Ashkenazi Jewish, French-Canadian, or Cajun descent and those with a family history consistent with TSD. If both partners are determined to be carriers of TSD, genetic counseling and prenatal diagnosis should be offered.

  4. George Chester Stone (1924-2013).

    Science.gov (United States)

    Adler, Nancy

    2014-01-01

    George Chester Stone was born February 21, 1924, and died on July 13, 2013. A quiet revolutionary, George was a founder of the field of health psychology. George played critical roles conceptualizing the field of health psychology, charting its bounds and potential, promulgating guidelines for training, founding the first doctoral program in health psychology, editing influential volumes defining the new field, launching the flagship journal for the field, and establishing a home for the field within APA. He was able to accomplish all this through his talent for working collaboratively. 2014 APA, all rights reserved

  5. Correlation of augmented startle reflex with brainstem electrophysiological responses in Tay-Sachs disease.

    Science.gov (United States)

    Nakamura, Sadao; Saito, Yoshiaki; Ishiyama, Akihiko; Sugai, Kenji; Iso, Takashi; Inagaki, Masumi; Sasaki, Masayuki

    2015-01-01

    To clarify the evolution of an augmented startle reflex in Tay-Sachs disease and compare the temporal relationship between this reflex and brainstem evoked potentials. Clinical and electrophysiological data from 3 patients with Tay-Sachs disease were retrospectively collected. The augmented startle reflex appeared between the age of 3 and 17 months and disappeared between the age of 4 and 6 years. Analysis of brainstem auditory evoked potentials revealed that poor segregation of peak I, but not peak III, coincided with the disappearance of the augmented startle reflex. A blink reflex with markedly high amplitude was observed in a patient with an augmented startle reflex. The correlation between the augmented startle reflex and the preservation of peak I but not peak III supports the theory that the superior olivary nucleus is dispensable for this reflex. The blink reflex with high amplitudes may represent augmented excitability of reticular formation at the pontine tegmentum in Tay-Sachs disease, where the pattern generators for the augmented startle and blink reflexes may functionally overlap. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  6. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.

    Directory of Open Access Journals (Sweden)

    Takashi Kodama

    Full Text Available To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2 levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.

  7. George W. Bushi "intellektuaalne armuromaan" / Urmas Kiil

    Index Scriptorium Estoniae

    Kiil, Urmas

    2005-01-01

    Paljude poliitikute arvates kinnitab USA presidendi George Bushi vaimustus Natan Sharanski raamatust "The Case of Democracy: The Power of Freedom to Overcome Tyranny and Terror " fakti, et midagi pole tema poliitikas muutunud ka teisel ametiajal

  8. Johann Georg Eisen / Indrek Jürjo

    Index Scriptorium Estoniae

    Jürjo, Indrek, 1956-2009

    1999-01-01

    Rets. rmt.: Johann Georg Eisen (1717-1779). Ausgewählte Schriften. Deutsche Volksaufklärung und Leibeigenschaft im Russischen Reich. Hrsg. von Roger Bartlett und Erich Donnert. Marburg : IHerder-Institut, 1998

  9. Georges Charpak, Nobel Physics Prize 1992

    International Nuclear Information System (INIS)

    Anon.

    1992-01-01

    Wednesday 14 October looked like being a day like any other for detector specialist Georges Charpak. Except he had an unwelcome appointment with the dentist early that afternoon. Late that morning he was able to telephone to cancel the appointment. 'I have a small problem...', he explained. The problem was the announcement that Georges Charpak receives 1992's most prestigious award for physics

  10. George's secret key to the universe

    CERN Document Server

    Hawking, Lucy; Galfard, Christophe; Parsons, Gary

    2007-01-01

    In their bestselling book for young readers, noted physicist Stephen Hawking and his daughter, Lucy, provide a grand and funny adventure that explains fascinating information about our universe, including Dr. Hawking's latest ideas about black holes. It's the story of George, who's taken through the vastness of space by a scientist, his daughter, and their super-computer named Cosmos. George's Secret Key to the Universe was a New York Times bestseller and a selection of Al's Book Club on the Today show.

  11. Georges Charpak and his multiwire chamber

    CERN Multimedia

    1970-01-01

    In 1968, Georges Charpak developed the 'multiwire proportional chamber', a gas-filled box with a large number of parallel detector wires, each connected to individual amplifiers. Linked to a computer, it could achieve a counting rate a thousand times better than existing techniques - without a camera in sight. From left to right, Georges Charpak, Fabio Sauli and Jean-Claude Santiard working on a multiwire chamber in 1970.

  12. Fort St. Vrain core performance

    International Nuclear Information System (INIS)

    McEachern, D.W.; Brown, J.R.; Heller, R.A.; Franek, W.J.

    1977-07-01

    The Fort St. Vrain High Temperature Gas Cooled Reactor core performance has been evaluated during the startup testing phase of the reactor operation. The reactor is graphite moderated, helium cooled, and uses coated particle fuel and on-line flow control to each of the 37 refueling regions. Principal objectives of startup testing were to determine: core and control system reactivity, radial power distribution, flow control capability, and initial fission product release. Information from the core demonstrates that Technical Specifications are being met, performance of the core and fuel is as expected, flow and reactivity control are predictable and simple for the operator to carry out

  13. Address of U.S. President George W. Bush / George W. Bush

    Index Scriptorium Estoniae

    Bush, George W., 1946-

    2005-01-01

    Ilmunud ka: Eesti Päevaleht : Mõte 10. juuni lk. 9-11. Ameerika Ühendriikide presidendi George Bushi Läti visiidi ajal 7. mail peetud kõne. Ajal. Eesti Päevaleht : Möte toodud ka George W. Bushi CV. Ajal. The Baltic Times lüh.

  14. Doença de Tay-Sachs em criança de raça negra Tay-Sachs disease in a Negro child

    Directory of Open Access Journals (Sweden)

    Sérgio Rosemberg

    1970-12-01

    Full Text Available É relatado um caso de doença de Tay-Sachs em criança da raça negra. O encontro desta entidade em indivíduos não judeus é bastante raro, sendo este o quinto caso descrito em criança negra. Os autores estudam alguns aspectos genéticos, chegando à conclusão de que o cálculo da freqüência do gene mutante é impossível devido ao pequeno número de casos e ao fato de que não há estimativas seguras quanto à taxa de nascimento de indivíduos negros na população, Em que pese o fato de o diagnóstico se assentar em bases puramente clínicas e anatômicas, prescindindo os autores de exames bioquímicos específicos, os dados apresentados são congruentes com os casos clássicos descritos na literatura.A case of Tay-Sachs diseases in a Negro child is reported. The occurrence of this disease in non-Jewish individuals is very rare and this is the fifth case of a Negro-child reported in the litterature. Some genetical aspects are studied and the authors conclude that the frequency calculation of the mutant gene is impossible due to the small number of cases and to the fact that there is reliable information regarding the rate of birth of Negro individuals in the general population. The diagnosis was based on ophtalmological and clinical data. No specific biochemical tests were performed but the data obtained are congruent with other cases reported in the litterature.

  15. Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease.

    Science.gov (United States)

    Hussein, Norita; Weng, Stephen F; Kai, Joe; Kleijnen, Jos; Qureshi, Nadeem

    2018-03-14

    Globally, about five per cent of children are born with congenital or genetic disorders. The most common autosomal recessive conditions are thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease, with higher carrier rates in specific patient populations. Identifying and counselling couples at genetic risk of the conditions before pregnancy enables them to make fully informed reproductive decisions, with some of these choices not being available if genetic counselling is only offered in an antenatal setting. This is an update of a previously published review. To assess the effectiveness of systematic preconception genetic risk assessment to improve reproductive outcomes in women and their partners who are identified as carriers of thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease in healthcare settings when compared to usual care. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Registers. In addition, we searched for all relevant trials from 1970 (or the date at which the database was first available if after 1970) to date using electronic databases (MEDLINE, Embase, CINAHL, PsycINFO), clinical trial databases (National Institutes of Health, Clinical Trials Search portal of the World Health Organization, metaRegister of controlled clinical trials), and hand searching of key journals and conference abstract books from 1998 to date (European Journal of Human Genetics, Genetics in Medicine, Journal of Community Genetics). We also searched the reference lists of relevant articles, reviews and guidelines and also contacted subject experts in the field to request any unpublished or other published trials.Date of latest search of the registers: 20 June 2017.Date of latest search of all other sources: 16 November 2017. Any randomised or quasi-randomised controlled trials (published or unpublished) comparing reproductive outcomes of systematic preconception genetic risk assessment for thalassaemia, sickle

  16. Fort St. Vrain decommissioning project

    International Nuclear Information System (INIS)

    Fisher, M.

    1998-01-01

    Public Service Company of Colorado (PSCo), owner of the Fort St. Vrain nuclear generating station, achieved its final decommissioning goal on August 5, 1997 when the Nuclear Regulatory Commission terminated the Part 50 reactor license. PSCo pioneered and completed the world's first successful decommissioning of a commercial nuclear power plant after many years of operation. In August 1989, PSCo decided to permanently shutdown the reactor and proceed with its decommissioning. The decision to proceed with early dismantlement as the appropriate decommissioning method proved wise for all stake holders - present and future - by mitigating potential environmental impacts and reducing financial risks to company shareholders, customers, employees, neighboring communities and regulators. We believe that PSCo's decommissioning process set an exemplary standard for the world's nuclear industry and provided leadership, innovation, advancement and distinguished contributions to other decommissioning efforts throughout the world. (author)

  17. Expanding the spectrum of HEXA mutations in Indian patients with Tay–Sachs disease

    Directory of Open Access Journals (Sweden)

    Jayesh Sheth

    2014-01-01

    Full Text Available Tay–Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52, resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay–Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A and c.805G>C (p.G269R in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57 and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i c.532C>T (p.R178C, (ii c.964G>T (p.D322Y, and (iii c.1385A>T (p.E462V; two nonsense mutations (i c.709C>T (p.Q237X and (ii c.1528C>T (p.R510X, one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5 and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay–Sachs disease with clustering of ~73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  18. Expanding the spectrum of HEXA mutations in Indian patients with Tay-Sachs disease.

    Science.gov (United States)

    Sheth, Jayesh; Mistri, Mehul; Datar, Chaitanya; Kalane, Umesh; Patil, Shekhar; Kamate, Mahesh; Shah, Harshuti; Nampoothiri, Sheela; Gupta, Sarita; Sheth, Frenny

    2014-01-01

    Tay-Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52), resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay-Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A) and c.805G>C (p.G269R) in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57) and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i) c.532C>T (p.R178C), (ii) c.964G>T (p.D322Y), and (iii) c.1385A>T (p.E462V); two nonsense mutations (i) c.709C>T (p.Q237X) and (ii) c.1528C>T (p.R510X), one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5) and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay-Sachs disease with clustering of ~ 73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  19. [Dr Mavro Sachs (1817-1888): the first lecturer of Zagreb University].

    Science.gov (United States)

    Dugacki, Vladimir

    2010-01-01

    Mavro Sachs (Jánosháza, Hungary, 1817 % emdash; Rijeka, 1888.) was a Zagreb student since 1828. In 1846 he graduated in medicine from the University of Vienna and returned to Zagreb to be the city physician. In 1849, he started to teach forensic medicine at the School of Law of the Royal Academy of Sciences in Zagreb and continued teaching the same subject at the Zagreb University Faculty of Law in the capacity of docent (corresponds to lecturer in the British system). He also taught medical law at pharmaceutical studies of Zagreb University. From 1855 to 1860, he presided over the Jewish Community of Zagreb.

  20. Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease.

    Science.gov (United States)

    Optican, Lance M; Rucker, Janet C; Keller, Edward L; Leigh, R John

    2008-01-01

    In late-onset Tay-Sachs disease (LOTS), saccades are interrupted by one or more transient decelerations. Some saccades reaccelerate and continue on before eye velocity reaches zero, even in darkness. Intervals between successive decelerations are not regularly spaced. Peak decelerations of horizontal and vertical components of oblique saccades in LOTS is more synchronous than those in control subjects. We hypothesize that these decelerations are caused by dysregulation of the fastigial nuclei (FN) of the cerebellum, which fire brain stem inhibitory burst neurons (IBNs).

  1. Submission to the Georges Bank Review Panel

    Energy Technology Data Exchange (ETDEWEB)

    Rankin, E.; Butler, M.; Church, T.

    1999-01-01

    The Georges Bank, off southwestern Nova Scotia, is one of the most biologically productive continental shelf ecosystems in the world. It supports a wide diversity of species, including many seabirds. In 1988, federal and provincial legislation placed a moratorium on petroleum exploration and drilling on the Georges Bank, directing the ministers of Natural Resources Canada, and of the Nova Scotia Department of Natural Resources to make a decision regarding the moratorium by January 1, 2000. This report by the Ecology Action Centre (EAC) asks the Georges Bank Review Panel to recommend extending the moratorium on Georges Bank and adjacent areas until 2012, in part to match the American moratorium. Other reasons why the panel made this recommendation were explored. The principal reason is the EAC concern that when the moratorium is lifted, several oil and gas developments will proceed in this ecologically significant offshore area that could result in the loss of the ecological integrity of Georges Bank. The EAC contends that before oil and gas development in the region could begin, the following issues would have to be resolved: (1) disposal of drill muds and cuttings, (2) volume and disposal of produced water, (3) effects of small and large spills, (4) impact of seismic activities on whales and turtles, and (5) carbon dioxide emission through venting and flaring. Also, the EAC does not believe that developing Georges Bank will create as many jobs in the affected communities as some people suggest. Lifting the moratorium by Canada would also frustrate American environmental goals, as well as compromise Canada`s position in international environmental partnerships such as the Gulf of Maine Council. 19 refs., tabs., figs.

  2. Submission to the Georges Bank Review Panel

    International Nuclear Information System (INIS)

    Rankin, E.; Butler, M.; Church, T.

    1999-01-01

    The Georges Bank, off southwestern Nova Scotia, is one of the most biologically productive continental shelf ecosystems in the world. It supports a wide diversity of species, including many seabirds. In 1988, federal and provincial legislation placed a moratorium on petroleum exploration and drilling on the Georges Bank, directing the ministers of Natural Resources Canada, and of the Nova Scotia Department of Natural Resources to make a decision regarding the moratorium by January 1, 2000. This report by the Ecology Action Centre (EAC) asks the Georges Bank Review Panel to recommend extending the moratorium on Georges Bank and adjacent areas until 2012, in part to match the American moratorium. Other reasons why the panel made this recommendation were explored. The principal reason is the EAC concern that when the moratorium is lifted, several oil and gas developments will proceed in this ecologically significant offshore area that could result in the loss of the ecological integrity of Georges Bank. The EAC contends that before oil and gas development in the region could begin, the following issues would have to be resolved: (1) disposal of drill muds and cuttings, (2) volume and disposal of produced water, (3) effects of small and large spills, (4) impact of seismic activities on whales and turtles, and (5) carbon dioxide emission through venting and flaring. Also, the EAC does not believe that developing Georges Bank will create as many jobs in the affected communities as some people suggest. Lifting the moratorium by Canada would also frustrate American environmental goals, as well as compromise Canada's position in international environmental partnerships such as the Gulf of Maine Council. 19 refs., tabs., figs

  3. Language, literature and theory in Georges Perec

    Directory of Open Access Journals (Sweden)

    Jacques Fux

    2013-07-01

    Full Text Available This article approaches some possible relations between space and literature within the work of the French writer Georges Perec. The book Espèces d’espaces, published in 1974, is taken as a major reference for a reflection about how its narrative structure produces displacements in its fictional status.

  4. Final Comments from Professors George and Beane.

    Science.gov (United States)

    Beane, James; George, Paul S.

    1996-01-01

    Concludes this journal focus section on curriculum integration with transcripts of questions asked by conference attendees and answers by Professors Beane and George. Areas addressed included experience levels with children and teachers, studies that point to the failure of curriculum integration, and how teachers can continue curriculum…

  5. Georg Franz Buquoy und "La famosissima Notte"

    Czech Academy of Sciences Publication Activity Database

    Konečný, Lubomír

    -, 0016 (2011), s. 1-9 ISSN 2190-3328 Institutional research plan: CEZ:AV0Z80330511 Keywords : Georg Franz August de Longueval, Count Buquoy * Antonio Allegri * Il Correggio Subject RIV: AL - Art, Architecture, Cultural Heritage http://www.riha-journal.org/articles/2011/2011-jan-mar/konecny-buquoy-famosissima-notte

  6. George MacDonald's Estimate of Childhood

    Science.gov (United States)

    Pridmore, John

    2007-01-01

    The nineteenth-century fantasy writer George MacDonald believed that "it is better to be a child in a green field than a knight of many orders." In this paper, I shall explore the bearing of this high estimate of childhood on spiritual education. MacDonald explores the spirituality of the child in his essay "A Sketch of Individual Development" and…

  7. Kdo byl Georg Placzek (1905-1955)

    Czech Academy of Sciences Publication Activity Database

    Gottvald, Aleš

    2005-01-01

    Roč. 55, č. 3 (2005), s. 275-287 ISSN 0009-0700 Institutional research plan: CEZ:AV0Z20650511 Keywords : Georg Placzek * the oretical physics * history of physics * nuclear physics * Manhattan project * spectroscopy Subject RIV: BG - Nuclear, Atomic and Molecular Physics, Colliders

  8. George A. Towns Elementary School. Atlanta, Georgia

    Science.gov (United States)

    Burt, Ralph H.

    1976-01-01

    A project testing solar heating and cooling in an existing building, the George A. Towns Elementary School, is intended to provide information on system design and performance, allow the identification and correction of problems encountered in installing large units, and gauge community/user reaction to solar equipment. (Author/MLF)

  9. Fort Davis National Historic Site : acoustical monitoring

    Science.gov (United States)

    2013-06-01

    During the summer of 2010 (September - October 2010), the Volpe Center collected baseline acoustical data at Fort Davis National Historic Site (FODA)at two sites deployed for approximately 30 days each. The baseline data collected during this period ...

  10. Renewable Energy Opportunities at Fort Hood, Texas

    Energy Technology Data Exchange (ETDEWEB)

    Solana, Amy E.; Warwick, William M.; Orrell, Alice C.; Russo, Bryan J.; Parker, Kyle R.; Weimar, Mark R.; Horner, Jacob A.; Manning, Anathea

    2011-11-14

    This report presents the results of Pacific Northwest National Laboratory's (PNNL) follow-on renewable energy (RE) assessment of Fort Hood. Fort Hood receives many solicitations from renewable energy vendors who are interested in doing projects on site. Based on specific requests from Fort Hood staff so they can better understand these proposals, and the results of PNNL's 2008 RE assessment of Fort Hood, the following resources were examined in this assessment: (1) Municipal solid waste (MSW) for waste-to-energy (WTE); (2) Wind; (3) Landfill gas; (4) Solar photovoltaics (PV); and (5) Shale gas. This report also examines the regulatory issues, development options, and environmental impacts for the promising RE resources, and includes a review of the RE market in Texas.

  11. Generalized Bondi-Sachs equations for characteristic formalism of numerical relativity

    Science.gov (United States)

    Cao, Zhoujian; He, Xiaokai

    2013-11-01

    The Cauchy formalism of numerical relativity has been successfully applied to simulate various dynamical spacetimes without any symmetry assumption. But discovering how to set a mathematically consistent and physically realistic boundary condition is still an open problem for Cauchy formalism. In addition, the numerical truncation error and finite region ambiguity affect the accuracy of gravitational wave form calculation. As to the finite region ambiguity issue, the characteristic extraction method helps much. But it does not solve all of the above issues. Besides the above problems for Cauchy formalism, the computational efficiency is another problem. Although characteristic formalism of numerical relativity suffers the difficulty from caustics in the inner near zone, it has advantages in relation to all of the issues listed above. Cauchy-characteristic matching (CCM) is a possible way to take advantage of characteristic formalism regarding these issues and treat the inner caustics at the same time. CCM has difficulty treating the gauge difference between the Cauchy part and the characteristic part. We propose generalized Bondi-Sachs equations for characteristic formalism for the Cauchy-characteristic matching end. Our proposal gives out a possible same numerical evolution scheme for both the Cauchy part and the characteristic part. And our generalized Bondi-Sachs equations have one adjustable gauge freedom which can be used to relate the gauge used in the Cauchy part. Then these equations can make the Cauchy part and the characteristic part share a consistent gauge condition. So our proposal gives a possible new starting point for Cauchy-characteristic matching.

  12. Integrated Sachs-Wolfe effect in a quintessence cosmological model: Including anisotropic stress of dark energy

    International Nuclear Information System (INIS)

    Wang, Y. T.; Xu, L. X.; Gui, Y. X.

    2010-01-01

    In this paper, we investigate the integrated Sachs-Wolfe effect in the quintessence cold dark matter model with constant equation of state and constant speed of sound in dark energy rest frame, including dark energy perturbation and its anisotropic stress. Comparing with the ΛCDM model, we find that the integrated Sachs-Wolfe (ISW)-power spectrums are affected by different background evolutions and dark energy perturbation. As we change the speed of sound from 1 to 0 in the quintessence cold dark matter model with given state parameters, it is found that the inclusion of dark energy anisotropic stress makes the variation of magnitude of the ISW source uncertain due to the anticorrelation between the speed of sound and the ratio of dark energy density perturbation contrast to dark matter density perturbation contrast in the ISW-source term. Thus, the magnitude of the ISW-source term is governed by the competition between the alterant multiple of (1+3/2xc-circumflex s 2 ) and that of δ de /δ m with the variation of c-circumflex s 2 .

  13. Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease.

    Science.gov (United States)

    Ozkara, Hatice Asuman; Sandhoff, Konrad

    2003-04-01

    Two homoallelic mutations have recently been identified in the alpha-subunit of hexosaminidase A (EC 3.2.1.52) causing the infantile form of Tay-Sachs disease in Turkish patients. Both of these mutations, a 12 bp deletion (1096-1107 or 1098-1108 or 1099-1109) in exon 10 and a point mutation (G1362 to A, Gly454 to Asp) in exon 12, are located in the catalytic domain of the hexosaminidase alpha-chain. In order to determine whether these mutations affect the function of the catalytic domain or result in an instable protein, both mutant cDNAs were overexpressed in COS-1 cells. As judged by Western blotting, transfections of wild-type cDNA produced pro-alpha-chain and mature alpha-chain in parallel with a fivefold increase in cellular hexosaminidase activity using the synthetic substrate 4-methylumbelliferyl beta-N-acetylglucosamine 6-sulfate (MUGS). However, both mutants produced only pro-alpha-chains, although no mature form or detectable hexosaminidase activity towards two different synthetic substrates was observed. These data are consistent with the biochemical phenotype of infantile Tay-Sachs disease. We conclude that the overexpressed mutant pro-alpha-chains were misfolded and could not undergo further proteolytic processing to the active form of the enzyme in the lysosome.

  14. Hydrologic Analysis of Fort Leonard Wood, Missouri

    Science.gov (United States)

    2015-08-01

    drainage areas are different, hydrological analysis will be conducted on the two basins individually. The results of the two analyses will be combined to...ER D C TR -1 5- 4 Environmental Quality and Installations Hydrologic Analysis of Fort Leonard Wood, Missouri En gi ne er R es ea rc h...Environmental Quality and Installations ERDC TR-15-4 August 2015 Hydrologic Analysis of Fort Leonard Wood, Missouri Michael L. Follum, Darla C. McVan

  15. Bent's Old Fort: Amphibians and Reptiles

    Science.gov (United States)

    Muths, E.

    2008-01-01

    Bent's Old Fort National Historic Site sits along the Arkansas River in the semi-desert prairie of southeastern Colorado. The USGS provided assistance in designing surveys to assess the variety of herpetofauna (amphibians and reptiles) resident at this site. This brochure is the results of those efforts and provides visitors with information on what frogs, toads, snakes and salamanders might be seen and heard at Bent's Old Fort.

  16. "Cherry red spot" in a patient with Tay-Sachs disease: case report "Mácula em cereja" em paciente com doença de Tay-Sachs: relato de caso

    Directory of Open Access Journals (Sweden)

    Ricardo Evangelista Marrocos de Aragão

    2009-08-01

    Full Text Available Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzime hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called "cherry red spot" is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.Tay-Sachs é uma doença autossômica recessiva, caracterizada pela deficiência da enzima hexosaminidase A levando ao acúmulo de esfingolipídios (GM2 em células neuronais que resulta em uma perda progressiva da função neurológica. O acúmulo de lipídios em células ganglionais da retina leva a uma aparência de mácula em cereja, característica do fundo de olho de pessoas acometidas. "Mácula em cereja" também pode ser vista em outras doenças neurometabólicas e em oclusão da artéria central da retina. Este trabalho relata o caso de um paciente com doença de Tay-Sachs em uma família com história de quatro óbitos por causas semelhantes sem diagnóstico.

  17. Regional neuro axonal injury detected by 1H 3 Tesla spectroscopic imaging in late onset Tay sachs

    International Nuclear Information System (INIS)

    Gagoski, Borjan Aleksandar; Eichler, Florian S.

    2010-01-01

    Late-onset Tay Sachs (LOTS) is a rare lysosomal storage disorder resulting from mutations of the subunit of the lysosomal enzyme β-hexosaminidase A, which catalyzes the degradation of GM2 ganglioside. We have applied the fast encoding spectroscopic imaging technique to LOTS patients to further investigate the neuro degenerative consequences of this disease.(Author)

  18. Exact Kantowski-Sachs and Bianchi types I and III cosmological models with a conformally invariant scalar field

    International Nuclear Information System (INIS)

    Accioly, A.J.

    1985-01-01

    Exact solutions of the Einstein-Conformally Invariant Scalar Field Equations are obtained for Kantowski-Sachs and Bianchi types I and III cosmologies. The presence of the conformally invariant scalar field is responsible for some interesting features of the solutions. In particular it is found that the Bianchi I model is consistent with the big-bang theory of cosmology. (Author) [pt

  19. The philosophy of Hans-Georg Gadamer / Wolfgang Drechsler

    Index Scriptorium Estoniae

    Drechsler, Wolfgang, 1963-

    1998-01-01

    Rets.rmt.: The philosophy of Hans-Georg Gadamer : a review of the philosophy of Hans-Georg Gadamer / Lewis Edwin Hahn, ed. Chicago and La Salle, IL: Open Court, 1997. (The Library of Living Philosophers, vol. XXIV)

  20. The 1995 Georges Bank Stratification Study and Moored Array Measurements

    National Research Council Canada - National Science Library

    Alessi, C

    2001-01-01

    .... GLOBEC Northwest Atlantic/Georges Bank field program. The GBSS was designed to investigate the physical processes which control the seasonal development of stratification along the southern flank of Georges Bank during spring and summer...

  1. EDITORIAL: George W Series Memorial Essays

    Science.gov (United States)

    Dodd, J. N.

    1997-01-01

    I would like to recall my experiences when I joined George Series when on study leave from the University of Otago in 1959. Like George, we had decided, some years previously, to pursue the line of research opened up by the double-resonance experiment of Brossel and Bitter. Up to that time both groups had been applying the new technique to traditional spectroscopic problems, those of measuring the fine structure, hyperhe structure, g-factors and nuclear moments. But here, in Oxford, was something new and exciting - experiments that studied the nature of light itself, questioning some long-held views. George had come to the view that, in a double-resonance experiment, one could demonstrate the inner coherence that could exist between superposition states of an atom. He, and his students Wilf Fox and Mike Taylor, had already obtained promising evidence; but he needed more definite results if he was going to allay the doubts and suspicions of some others in the Clarendon. At the time, people generally thought that light, being a superposition of radiations from many atoms with no obvious phase relation to each other, was an incoherent wave phenomenon. One could observe small scale coherence (for example, in the Young interference experiment) but it would be largely concealed by the incoherent nature of the radiation from many atoms in a source. In the experiments that had been performed the coherence was introduced between a pair of excited states (Zeeman sub-states) by the application of an oscillating magnetic field - was it not likely, according to the doubters, that the modulation observed in the fluorescence was due to "pick-up" in the amplifiers from the source of oscillations? George had to prove that this was not the cause. I well remember him saying at the time "I have a hunch that I am correct". That typified his approach to physics. The work of 1959 demonstrated beyond all doubt that substantial coherence, manifested as strong beats in the intensity of the

  2. Late-onset Tay-Sachs disease: phenotypic characterization and genotypic correlations in 21 affected patients.

    Science.gov (United States)

    Neudorfer, Orit; Pastores, Gregory M; Zeng, Bai J; Gianutsos, John; Zaroff, Charles M; Kolodny, Edwin H

    2005-02-01

    The purpose of this study was to describe the phenotype (and corresponding genotype) of adult patients with late-onset Tay-Sachs disease, a clinical variant of the GM2-gangliosidoses. A comprehensive physical examination, including neurological assessments, was performed to establish the current disease pattern and severity. In addition, the patients' past medical histories were reviewed. The patients' alpha-subunit mutations (beta-Hexosaminidase A genotype) were determined and correlated with their corresponding clinical findings and disease course. Twenty-one patients (current mean age: 27.0 years; range: 14-47 years) were identified. The pedigree revealed a relative with the "classic" infantile or late-onset form of Tay-Sachs disease in four (out of 18) unrelated families. The patients were predominantly male (15/21 individuals) and of Ashkenazi Jewish ancestry (15/18 families). Mean age at onset was 18.1 years; balance problems and difficulty climbing stairs were the most frequent presenting complaints. In several cases, the diagnosis was delayed (mean age at diagnosis: 27.0 years). Analysis of the beta-hex A gene revealed the G269S mutation as the most common disease allele; found in homozygosity (N = 1) or heterozygosity (N = 18; including 2 sib pairs). Disease onset (age 36 years) was delayed and progression relatively slower in the homozygous G269S patient. Two siblings (ages 28 and 31 years), of non-Jewish ancestry, were compound heterozygotes (TATC1278/W474C); their clinical course is dominated by psychiatric problems. Brain imaging studies revealed marked cerebellar atrophy in all patients (N = 18) tested, regardless of disease stage. Late-onset Tay-Sachs disease is an infrequent disorder and the diagnosis is often missed or delayed (by approximately 8 years). Early on, the majority of patients develop signs of either cerebellar or anterior motor neuron involvement. Affected individuals may also develop psychotic episodes. In most cases, the later

  3. The Georg Jensen and Alessi Design

    DEFF Research Database (Denmark)

    Tanderup, Sisse

    2009-01-01

    in the design process. Alessi's motivation for involving the concept of memory is that it adds a cultural dimension to the design objects, enabling the objects to make an identity-forming impact. Compared to this, Danish and Scandinavian design practice has traditionally focused on form and function......  The Georg Jensen and Alessi Design A comparative analysis focusing on the use of memory   The dimension of memory has become a central theme within several fields of research, including architecture, philosophy, psychology and medicine. However, memory has not yet been explored more substantially...... as an independent identity category in design research. This article deals with the use of the memory theme in design examples from the two companies Georg Jensen and Alessi. Italian design practice is characterized by a tradition of involving memory categories from Italian history, philosophy, art and tradition...

  4. George A. Miller (1920–2012)

    OpenAIRE

    Pinker, Steven

    2013-01-01

    Presents an obituary for George A. Miller (1920—2012). Miller ranks among the most important psychologists of the 20th century. In addition to writing one of the best known papers in the history of psychology (“The Magical Number Seven, Plus or Minus Two: Some Limits on Our Capacity for Processing Information,” published in Psychological Review in 1956), Miller also fomented the cognitive revolution, invented psycholinguistics and cognitive psychology, imported powerful ideas from the theorie...

  5. Autobiography of Sir George Biddell Airy

    Science.gov (United States)

    Airy, George Biddell; Airy, Wilfred

    2010-06-01

    Preface; 1. Personal sketch of George Biddell Airy; 2. From his birth to his taking his B.A. degree; 3. At Trinity College, Cambridge; 4. At Cambridge Observatory; 5. At Greenwich Observatory, 1836-1846; 6. At Greenwich Observatory, 1846-1856; 7. At Greenwich Observatory, 1856-1866; 8. At Greenwich Observatory, 1866-1876; 9. At Greenwich Observatory to his resignation in 1881; 10. At the White House, Greewich, to his death; Appendix: List of printed papers; Index.

  6. Unverrichteter Sache

    Directory of Open Access Journals (Sweden)

    Jani Kirov

    2007-01-01

    Full Text Available Rezensiertes Werk: Adriaan Lanni, Law and Justice in the Courts of Classical Athens, Cambridge: Cambridge University Press 2006, 210 S., ISBN-13 978-0-521-85759-8; ISBN-10 0-521-85759-7

  7. Phenomenological dynamics of loop quantum cosmology in Kantowski-Sachs spacetime

    International Nuclear Information System (INIS)

    Chiou, D.-W.

    2008-01-01

    The fundamental theory and the semiclassical description of loop quantum cosmology (LQC) have been studied in the Friedmann-Robertson-Walker and Bianchi I models. As an extension to include both anisotropy and intrinsic curvature, this paper investigates the cosmological model of Kantowski-Sachs spacetime with a free massless scalar field at the level of phenomenological dynamics with the LQC discreteness corrections. The LQC corrections are implemented in two different improved quantization schemes. In both schemes, the big bang and big crunch singularities of the classical solution are resolved and replaced by the big bounces when the area or volume scale factor approaches the critical values in the Planck regime measured by the reference of the scalar field momentum. Symmetries of scaling are also noted and suggest that the fundamental spatial scale (area gap) may give rise to a temporal scale. The bouncing scenarios are in an analogous fashion of the Bianchi I model, naturally extending the observations obtained previously.

  8. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    Energy Technology Data Exchange (ETDEWEB)

    Fukumizu, M; Yoshikawa, H; Kurokawa, T [National Center Hospital for Mental, Nervous, and Muscular Disorders, Tokyo (Japan). Div. of Child Neurology; Takashima, S [National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Mental Retardation and Birth Defect Research; Sakuragawa, N [National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Inherited Metabolic Disease

    1992-11-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.).

  9. Optical structures, algebraically special spacetimes, and the Goldberg-Sachs theorem in five dimensions

    International Nuclear Information System (INIS)

    Taghavi-Chabert, Arman

    2011-01-01

    Optical (or Robinson) structures are one generalization of four-dimensional shearfree congruences of null geodesics to higher dimensions. They are Lorentzian analogues of complex and CR structures. In this context, we extend the Goldberg-Sachs theorem to five dimensions. To be precise, we find a new algebraic condition on the Weyl tensor, which generalizes the Petrov type II condition, in the sense that it ensures the existence of such congruences on a five-dimensional spacetime, vacuum or under weaker assumptions on the Ricci tensor. This results in a significant simplification of the field equations. We discuss possible degenerate cases, including a five-dimensional generalization of the Petrov type D condition. We also show that the vacuum black ring solution is endowed with optical structures, yet fails to be algebraically special with respect to them. We finally explain the generalization of these ideas to higher dimensions, which has been checked in six and seven dimensions.

  10. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    International Nuclear Information System (INIS)

    Fukumizu, M.; Yoshikawa, H.; Kurokawa, T.; Takashima, S.; Sakuragawa, N.

    1992-01-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.)

  11. Late-onset Tay-Sachs disease: the spectrum of peripheral neuropathy in 30 affected patients.

    Science.gov (United States)

    Shapiro, Barbara E; Logigian, Eric L; Kolodny, Edwin H; Pastores, Gregory M

    2008-08-01

    Late-onset Tay-Sachs (LOTS) disease is a chronic, progressive, lysosomal storage disorder caused by a partial deficiency of beta-hexosaminidase A (HEXA) activity. Deficient levels of HEXA result in the intracellular accumulation of GM2-ganglioside, resulting in toxicity to nerve cells. Clinical manifestations primarily involve the central nervous system (CNS) and lower motor neurons, and include ataxia, weakness, spasticity, dysarthria, dysphagia, dystonia, seizures, psychosis, mania, depression, and cognitive decline. The prevalence of peripheral nervous system (PNS) involvement in LOTS has not been well documented, but it has traditionally been thought to be very low. We examined a cohort of 30 patients with LOTS who underwent clinical and electrophysiologic examination, and found evidence of a predominantly axon loss polyneuropathy affecting distal nerve segments in the lower and upper extremities in eight patients (27%).

  12. Hill-Sachs lesion location: does it play a role in engagement?

    Energy Technology Data Exchange (ETDEWEB)

    Gyftopoulos, Soterios; Wang, Annie; Babb, James [NYU Langone Medical Center, Department of Radiology, New York, NY (United States)

    2015-08-15

    To see if there is an association between engagement on physical examination (PE) and the location of the Hill-Sachs lesion (HSL) as assessed by the modified biceps angle. Sixty-two patients with a history of anterior shoulder dislocation, who underwent preoperative MRI and arthroscopy at our institution and were tested for engagement on PE, were collected. Two musculoskeletal radiologists reviewed the MR studies, noting the presence of an HSL and documenting the location of the HSL with the modified biceps angle. Statistical analysis included the Mann-Whitney (MW) test and ROC (receiver-operating characteristic) curve. Of 62 patients, there were 58 males and 4 females with a mean age of 30 (range 18-59 years). Twenty patients demonstrated engagement on PE, while 42 did not. All patients had evidence of an HSL on MRI and arthroscopy. The mean biceps angle for the engaging group was 151.5 ± 13.9 , and 142.4 ± 17.3 for the non-engaging group. The biceps angle was statistically significantly higher among patients who had engagement compared to those who did not (p = 0.027). Overall, diagnostic accuracy was highest for a biceps angle >149 , which resulted in a sensitivity of 70 % and specificity of 67 %. The modified biceps angle, as measured on MRI, was significantly higher in patients who demonstrated engagement on physical examination than in those who did not. This supports the theory that the location of the Hill-Sachs lesion may play a role in engagement and may be its most important characteristic when determining its significance. (orig.)

  13. Neuro-ophthalmology of late-onset Tay-Sachs disease (LOTS).

    Science.gov (United States)

    Rucker, J C; Shapiro, B E; Han, Y H; Kumar, A N; Garbutt, S; Keller, E L; Leigh, R J

    2004-11-23

    Late-onset Tay-Sachs disease (LOTS) is an adult-onset, autosomal recessive, progressive variant of GM2 gangliosidosis, characterized by involvement of the cerebellum and anterior horn cells. To determine the range of visual and ocular motor abnormalities in LOTS, as a prelude to evaluating the effectiveness of novel therapies. Fourteen patients with biochemically confirmed LOTS (8 men; age range 24 to 53 years; disease duration 5 to 30 years) and 10 age-matched control subjects were studied. Snellen visual acuity, contrast sensitivity, color vision, stereopsis, and visual fields were measured, and optic fundi were photographed. Horizontal and vertical eye movements (search coil) were recorded, and saccades, pursuit, vestibulo-ocular reflex (VOR), vergence, and optokinetic (OK) responses were measured. All patients showed normal visual functions and optic fundi. The main eye movement abnormality concerned saccades, which were "multistep," consisting of a series of small saccades and larger movements that showed transient decelerations. Larger saccades ended earlier and more abruptly (greater peak deceleration) in LOTS patients than in control subjects; these changes can be attributed to premature termination of the saccadic pulse. Smooth-pursuit and slow-phase OK gains were reduced, but VOR, vergence, and gaze holding were normal. Patients with late-onset Tay-Sachs disease (LOTS) show characteristic abnormalities of saccades but normal afferent visual systems. Hypometria, transient decelerations, and premature termination of saccades suggest disruption of a "latch circuit" that normally inhibits pontine omnipause neurons, permitting burst neurons to discharge until the eye movement is completed. These measurable abnormalities of saccades provide a means to evaluate the effects of novel treatments for LOTS.

  14. FORTE spacecraft vibration mitigation. Final report

    International Nuclear Information System (INIS)

    Maly, J.R.

    1996-02-01

    This report documents work that was performed by CSA Engineering, Inc., for Los Alamos National Laboratory (LANL), to reduce vibrations of the FORTE spacecraft by retrofitting damped structural components into the spacecraft structure. The technical objective of the work was reduction of response at the location of payload components when the structure is subjected to the dynamic loading associated with launch and proto-qualification testing. FORTE is a small satellite that will be placed in orbit in 1996. The structure weighs approximately 425 lb, and is roughly 80 inches high and 40 inches in diameter. It was developed and built by LANL in conjunction with Sandia National Laboratories Albuquerque for the United States Department of Energy. The FORTE primary structure was fabricated primarily with graphite epoxy, using aluminum honeycomb core material for equipment decks and solar panel substrates. Equipment decks were bonded and bolted through aluminum mounting blocks to adjoining structure

  15. Bianchi - I, II, VIII, IX and Kantowski-Sachs-like cosmological models with perfect fluid and electromagnetic fields with conductivity current

    International Nuclear Information System (INIS)

    Portugal, R.

    1984-01-01

    Three processes of solutions of the Einstein-Maxwell equations for Bianchi - I, II, VIII, IX and Kantowski-Sachs-like cosmological models with perfect fluid in magnetohydrolodynamical regimem are presented. Diagonal Bianchi-like models are considered with two anisotropy direction in the maximum. Solutions are found for Bianchi-II and IX-like models with energy conditions to be analyzed. Solutions are found for Bianchi-IX and Kantowski-Sachs-Like models with positive electric conductivity and satisfering to the predominant energy conditions. Solutions are formed for isotropic Kantowski-Sachs-Like models satisfering to the equation of state p=λρ, 0 0, admiting, in addition to the perfect fluid, electric field only. It is shown that a class of Bertotti-Robinson-like solutions is unstable by perturbations and it is carried in Kantowski-Sachs-like models with non-null electric conductivity. (L.C.) [pt

  16. Renewable Energy Opportunities at Fort Polk, Louisiana

    Energy Technology Data Exchange (ETDEWEB)

    Solana, Amy E. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Boyd, Brian K. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Horner, Jacob A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Gorrissen, Willy J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Orrell, Alice C. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Weimar, Mark R. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Hand, James R. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Russo, Bryan J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Williamson, Jennifer L. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2010-11-17

    This document provides an overview of renewable resource potential at Fort Polk, based primarily upon analysis of secondary data sources supplemented with limited on-site evaluations. This effort focuses on grid-connected generation of electricity from renewable energy sources and also on ground source heat pumps for heating and cooling buildings. The effort was funded by the U.S. Army Installation Management Command (IMCOM) as follow-on to the 2005 Department of Defense (DoD) Renewables Assessment. The site visit to Fort Polk took place on February 16, 2010.

  17. Renewable Energy Opportunities at Fort Sill, Oklahoma

    Energy Technology Data Exchange (ETDEWEB)

    Boyd, Brian K.; Hand, James R.; Horner, Jacob A.; Orrell, Alice C.; Russo, Bryan J.; Weimar, Mark R.; Nesse, Ronald J.

    2011-03-31

    This document provides an overview of renewable resource potential at Fort Sill, based primarily upon analysis of secondary data sources supplemented with limited on-site evaluations. This effort focuses on grid-connected generation of electricity from renewable energy sources and on ground source heat pumps for heating and cooling buildings. The effort was funded by the U.S. Army Installation Management Command (IMCOM) as follow-on to the 2005 Department of Defense (DoD) Renewables Assessment. The site visit to Fort Sill took place on June 10, 2010.

  18. Prolífica George Sand

    Directory of Open Access Journals (Sweden)

    Àngels Santa

    2014-04-01

    Full Text Available A propósito de las obras de George Sand, Œuvres complètes. Sous la direction de Béatrice Didier. 1841-1842.Un hiver à Majorque. Édition critique par Angela Ryan. Horace.Édition critique par Jeanne Brunereau (París, Honoré Champion, 2013, 748 p. ISBN : 9782745319265 y Œuvres complètes. ́Sous la direction de Béatrice Didier. 1849.La petite Fadette. Édition critique par Andrée Mansau (París, Honoré Champion, 2013, 345p. ISBN : 9782745319203

  19. THE ONEIRIC AUTOBIOGRAPHY OF GEORGES PEREC.

    Science.gov (United States)

    Schwartz, Henry P

    2016-01-01

    Georges Perec's book La Boutique Obscure (1973; translated into English in 2012) serves as the basis for this paper. The book is a collection of dreams that its author dreamed from May 1968 to August 1972. The present author treats these dreams as chapters in a bizarre autobiography, elaborating Perec's life through a discussion of those dreams and using them as a starting point with which to discuss his views of dream interpretation and the role of dreams in psychoanalysis. © 2016 The Psychoanalytic Quarterly, Inc.

  20. Areva's challenge for ''Georges Besse 2''

    International Nuclear Information System (INIS)

    Jemain, A.

    2003-01-01

    For its future uranium enrichment plant of its Tricastin site (Drome, France), the world nuclear leader Areva has abandoned the gaseous diffusion technique (of French origin) for the centrifugation technique, more economical and modular. This future plant, named 'Georges Besse 2' will require 3 billions of euros of investment and will supply a world market also estimated to 3 billions of euros and shared between Areva, Urenco (UK), Usec (US), Minatom (Russia), JNC (Japan) and CNNC (China). The first batches of enriched uranium will be produced using a thousand of centrifuges by 2007. (J.S.)

  1. Defense.gov Special Report: Fort Hood Shooting

    Science.gov (United States)

    identify possible insider threats, Army Secretary John M. McHugh told lawmakers. Story Obama: Soldiers ," Army Secretary John M. McHugh told lawmakers. Story President Praises Swift Response to Fort Hood Remarks on Fort Hood Shooting at White House McHugh, Odierno Address Fort Hood Shooting Before Congress

  2. Unilaterally and rapidly progressing white matter lesion and elevated cytokines in a patient with Tay-Sachs disease.

    Science.gov (United States)

    Hayase, Tomomi; Shimizu, Jun; Goto, Tamako; Nozaki, Yasuyuki; Mori, Masato; Takahashi, Naoto; Namba, Eiji; Yamagata, Takanori; Momoi, Mariko Y

    2010-03-01

    We report the case of a girl with Tay-Sachs disease who had convulsions and deteriorated rapidly after an upper respiratory infection at the age of 11 months. At the age of 16 months, her seizures became intractable and magnetic resonance imaging of the brain showed high signal intensity on T2-weighted images and marked swelling in the white matter and basal nucelei of the right hemisphere. Her seizures and right hemisphere lesion improved with glycerol and dexamethasone treatment. When dexamethasone was discontinued, her symptoms worsened and lesions later appeared in the left hemisphere. Her cerebrospinal fluid showed elevated levels of the cytokines TNF-alpha and IL-5. It is considered that inflammation contributes to disease progression in Tay-Sachs disease.

  3. İki Kardeşte Görülen Tay-Sachs Hastalığı

    OpenAIRE

    KÜÇÜKÖDÜK, Ş.

    2010-01-01

    In view of two cases of autosomal recessive Tay-Sachs disease which affected two siblings, genetic consultation has been once more emphasized. The clinical features of this entity has been reviewed under the information obtained from recent literature. Otozomal resesif geçiş gösteren ve iki kardeşide etkiliyen Tay-Sachs hastalığında, genetik danışmanın önemi bir kez daha vurgulandı. Bu hastalığın klinik özellikleri literatür bilgilerinin ışığı altında gözden geçirildi....

  4. Smithsonian Marine Station (SMS) at Fort Pierce

    Science.gov (United States)

    share current Smithsonian research on the plants and animals of the Indian River Lagoon and marine Smithsonian Marine Station at Fort Pierce Website Search Box Search Field: SMS Website Search Twitter SMS Home › Welcome to the Smithsonian Marine Station Homepage slideshow Who We Are... The

  5. FORT NAMUTONI: FROM MILITARY STRONGHOLD TO TOURIST ...

    African Journals Online (AJOL)

    STRONGHOLD TO TOURIST CAMP. Col Dr Jan Ploeger*. "... this fortress was not just a white elephant, it was actually occupied and played a major role in the settlement of Germans in the far North." (own translation) - D.W. Krynauw Die Verhaal van. Namutoni, p 3. Introduction. Fort Namutoni, the last White outpost east of ...

  6. Den tabte fortælling

    DEFF Research Database (Denmark)

    Jørgensen, Kenneth Mølbjerg

    2008-01-01

    Ledelse er et af nøgleordene i fornyelsen af den offentlige sektor. Vi har imidlertid glemt et væsentligt aspekt af ledelse. Dette skyldes ikke mindst, at omgangsformen i dag er reguleret af information, mens den tidligere var reguleret af fortælleevnen. Evnen til dialog, indlevelse og nærvær er...

  7. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Proia, R.L.; Yamanaka, S.; Johnson, M.D. [and others

    1994-09-01

    Tay-Sachs disease, the prototype of the G{sub M2} gangliosidoses, is a catastrophic neurodegenerative disorder of infancy. The disease is caused by mutations in the HEXA gene resulting in an absence of the lysosomal enzyme, {beta}-hexosaminidase A. As consequence of the enzyme deficiency, G{sub M2} ganglioside accumulates progressively, beginning early in fetal life, to excessive amounts in the central nervous system (CNS). Rapid mental and motor deterioration starting in the first year of life leads to death by 2 to 4 years of age. Through the targeted disruption of the Hexa gene in embryonic stem cells, we have produced mice with biochemical and neuropathologic features of Tay-Sachs disease. The mutant mice exhibited less than 1% of normal {beta}-hexosaminidase A activity and accumulated G{sub M2} ganglioside in their CNS in an age-dependent manner. The accumulated ganglioside was stored in neurons as membranous cytoplasmic bodies characteristically found in the neurons of Tay-Sachs disease patients. At three to five months of age the mutant mice showed no apparent defects in motor or memory function. These {beta}-hexosaminidase A deficient mice should be useful for devising strategies to introduce functional enzymes and genes into the CNS. This model may also be valuable for studying the biochemical and pathologic changes occurring during the course of the disease.

  8. GM2-Gangliosidosis (Sandhoff and Tay Sachs disease): Diagnosis and Neuroimaging Findings (An Iranian Pediatric Case Series).

    Science.gov (United States)

    Karimzadeh, Parvaneh; Jafari, Narjes; Nejad Biglari, Habibeh; Jabbeh Dari, Sayena; Ahmad Abadi, Farzad; Alaee, Mohammad-Reza; Nemati, Hamid; Saket, Sasan; Tonekaboni, Seyed Hasan; Taghdiri, Mohammad-Mahdi; Ghofrani, Mohammad

    2014-01-01

    GM2-Gangliosidosis disease is a rare autosomal recessive genetic disorder that includes two disorders (Tay-Sachs and Sandhoff disease).These disorders cause a progressive deterioration of nerve cells and inherited deficiency in creating hexosaminidases A, B, and AB. Patients who were diagnosed withGM2-Gangliosidosis in the Neurology Department of Mofid Children's Hospital in Tehran, Iran from October 2009 to February 2014were included in our study. The disorder was confirmed by neurometabolic and enzyme level detection of hexosaminidases A, B, and AB in reference to Wagnester Laboratory in Germany. We assessed age, gender, past medical history, developmental status, clinical manifestations, and neuroimaging findings of 9 patients with Sandhoff disease and 9 with Tay Sachs disease. 83% of our patients were the offspring of consanguineous marriages. All of them had a developmental disorder as a chief complaint. 38%of patients had a history of developmental delay or regression and 22% had seizures. The patients with Sandhoff and Tay Sachs disease were followed for approximately 5 years and the follow-up showed all patients were bedridden or had expired due to refractory seizures, pneumonia aspiration, or swallowing disorders. Neuro-imaging findings included bilateral thalamic involvement, brain atrophy, and hypo myelination in near half of our patients (48%). According to the results of this study, we suggest that cherry-red spots, hyperacusis, refractory seizures, and relative parents in children with developmental delay and/or regression should be considered for assessment of GM2-Gangliosidosis disease.

  9. On the structure and applications of the Bondi-Metzner-Sachs group

    Science.gov (United States)

    Alessio, Francesco; Esposito, Giampiero

    This work is a pedagogical review dedicated to a modern description of the Bondi-Metzner-Sachs (BMS) group. Minkowski space-time has an interesting and useful group of isometries, but, for a generic space-time, the isometry group is simply the identity and hence provides no significant informations. Yet symmetry groups have important role to play in physics; in particular, the Poincaré group describing the isometries of Minkowski space-time plays a role in the standard definitions of energy-momentum and angular-momentum. For this reason alone it would seem to be important to look for a generalization of the concept of isometry group that can apply in a useful way to suitable curved space-times. The curved space-times that will be taken into account are the ones that suitably approach, at infinity, Minkowski space-time. In particular we will focus on asymptotically flat space-times. In this work, the concept of asymptotic symmetry group of those space-times will be studied. In the first two sections we derive the asymptotic group following the classical approach which was basically developed by Bondi, van den Burg, Metzner and Sachs. This is essentially the group of transformations between coordinate systems of a certain type in asymptotically flat space-times. In the third section the conformal method and the notion of “asymptotic simplicity” are introduced, following mainly the works of Penrose. This section prepares us for another derivation of the BMS group which will involve the conformal structure, and is thus more geometrical and fundamental. In the subsequent sections we discuss the properties of the BMS group, e.g. its algebra and the possibility to obtain as its subgroup the Poincaré group, as we may expect. The paper ends with a review of the BMS invariance properties of classical gravitational scattering discovered by Strominger, that are finding application to black hole physics and quantum gravity in the literature.

  10. Identification of novel mutations in HEXA gene in children affected with Tay Sachs disease from India.

    Directory of Open Access Journals (Sweden)

    Mehul Mistri

    Full Text Available Tay Sachs disease (TSD is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. The mutations leading to Tay Sachs disease in India are yet unknown. We aimed to determine mutations leading to TSD in India by complete sequencing of the HEXA gene. The clinical inclusion criteria included neuroregression, seizures, exaggerated startle reflex, macrocephaly, cherry red spot on fundus examination and spasticity. Neuroimaging criteria included thalamic hyperdensities on CT scan/T1W images of MRI of the brain. Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients. Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. The exons and exon-intron boundaries of the HEXA gene were bidirectionally sequenced using an automated sequencer. Mutations were confirmed in parents and looked up in public databases. In silico analysis for mutations was carried out using SIFT, Polyphen2, MutationT@ster and Accelrys Discovery Studio softwares. Fifteen families were included in the study. We identified six novel missense mutations, c.340 G>A (p.E114K, c.964 G>A (p.D322N, c.964 G>T (p.D322Y, c.1178C>G (p.R393P and c.1385A>T (p.E462V, c.1432 G>A (p.G478R and two previously reported mutations. c.1277_1278insTATC and c.508C>T (p.R170W. The mutation p.E462V was found in six unrelated families from Gujarat indicating a founder effect. A previously known splice site mutation c.805+1 G>C and another intronic mutation c.672+30 T>G of unknown significance were also identified. Mutations could not be identified in one family. We conclude that TSD patients from Gujarat should be screened for the common mutation p.E462V.

  11. Identification of novel mutations in HEXA gene in children affected with Tay Sachs disease from India.

    Science.gov (United States)

    Mistri, Mehul; Tamhankar, Parag M; Sheth, Frenny; Sanghavi, Daksha; Kondurkar, Pratima; Patil, Swapnil; Idicula-Thomas, Susan; Gupta, Sarita; Sheth, Jayesh

    2012-01-01

    Tay Sachs disease (TSD) is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. The mutations leading to Tay Sachs disease in India are yet unknown. We aimed to determine mutations leading to TSD in India by complete sequencing of the HEXA gene. The clinical inclusion criteria included neuroregression, seizures, exaggerated startle reflex, macrocephaly, cherry red spot on fundus examination and spasticity. Neuroimaging criteria included thalamic hyperdensities on CT scan/T1W images of MRI of the brain. Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients). Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. The exons and exon-intron boundaries of the HEXA gene were bidirectionally sequenced using an automated sequencer. Mutations were confirmed in parents and looked up in public databases. In silico analysis for mutations was carried out using SIFT, Polyphen2, MutationT@ster and Accelrys Discovery Studio softwares. Fifteen families were included in the study. We identified six novel missense mutations, c.340 G>A (p.E114K), c.964 G>A (p.D322N), c.964 G>T (p.D322Y), c.1178C>G (p.R393P) and c.1385A>T (p.E462V), c.1432 G>A (p.G478R) and two previously reported mutations. c.1277_1278insTATC and c.508C>T (p.R170W). The mutation p.E462V was found in six unrelated families from Gujarat indicating a founder effect. A previously known splice site mutation c.805+1 G>C and another intronic mutation c.672+30 T>G of unknown significance were also identified. Mutations could not be identified in one family. We conclude that TSD patients from Gujarat should be screened for the common mutation p.E462V.

  12. George Orwell and 1984: a personal view

    Directory of Open Access Journals (Sweden)

    ALFREDO SANDOVAL GÓMEZ

    2013-11-01

    Full Text Available George Orwell´s 1984 is considered a great negative utopia, in the sense it depicts the nightmare of what life might become in an oligarchic collectivism pursued to its logical conclusion. Under a social setup which is nothing but totalitarian barbarism, eternal warfare is the price one pays for an elusive peace. The Party with capital P keeps a total control over all of man´s actions as well as thoughts . The novel is a great satire and it attempts to diagnose man´s alienation in all its aspects, but with special emphasis on the social organization recommended by Marx and practiced by Stalin.  

  13. [Georg Friedrich Nicolai: war physician against war].

    Science.gov (United States)

    van Bergen, L

    2017-01-01

    Georg Friedrich Nicolai was a German professor and heart specialist who was one of the few who protested against the war at the beginning of World War I. As a result, he lost his job and was convicted. After the war, right-wing nationalist students and lack of support from his university superiors made it impossible for him to teach. He left Germany in 1922, never to return. In his book, Die Biologie des Krieges (The Biology of War), which was published in neutral Switzerland in 1917, he contradicted the social Darwinist idea - supported by many physicians as well - that war strengthened humanity, people and races, physically and mentally. On the contrary, he argued, war is biologically counterproductive.

  14. George Jeffreys: Pentecostal and Contemporary Implications

    Directory of Open Access Journals (Sweden)

    William K Kay

    2018-02-01

    Full Text Available The life and work of the Welsh evangelist George Jeffreys resulted in the planting of two denominations in the UK between 1915 and 1962, when he died. The Elim churches continue to this day to be one of the larger classical Pentecostal denominations in the UK, while the Bible Pattern Fellowship dispersed on Jeffreys’ death. The disputes that led to Jeffreys’ departure from Elim were said to have arisen from his adherence to British Israel doctrine, though his supporters believed they arose from his championing of local church ownership and democracy. This paper considers sociological and other reasons for Jeffreys’ remarkable success in the interwar years and his eventual departure from a denomination he founded. It concludes by reflecting on topics (such as the importance of debate and law that have relevance for contemporary Pentecostalism.

  15. Georges Sagnac: A life for optics

    Science.gov (United States)

    Darrigol, Olivier

    2014-12-01

    Georges Sagnac is mostly known for the optical effect in rotating frames that he demonstrated in 1913. His scientific interests were quite diverse: they included photography, optical illusions, X-ray physics, radioactivity, the blue of the sky, anomalous wave propagation, interferometry, strioscopy, and acoustics. An optical theme nonetheless pervaded his entire œuvre. Within optics, an original theory of the propagation of light motivated most of his investigations, from an ingenious explanation of the Fresnel drag, through the discovery of the Sagnac effect, to his quixotic defense of an alternative to relativity theory. Optical analogies efficiently guided his work in other domains. Optics indeed was his true passion. He saw himself as carrying the torch of the two great masters of French optics, Augustin Fresnel and Hippolyte Fizeau. In this mission he overcame his poor health and labored against the modernist tide, with much success originally and bitter isolation in the end. xml:lang="fr"

  16. GEORG BRANDES AND THE MODERN PROJECT

    Directory of Open Access Journals (Sweden)

    Jørgen Veisland

    2011-01-01

    Full Text Available Georg Brandes’ lecture series on The Main Tendencies in 19th Century Literature was initiated in 1871 and consists of a number of innovative interpretations of European literature. Brandes’ interpretations exemplify his interest in the ideals of the Enlightenment: knowledge, liberation, progress and free thought. The lecture series traces a dialectical movement in European literature, described by Brandes as a dialectics between revolution and liberalism on one hand and conservatism and reactionary tendencies on the other. Brandes includes a dialectics between home, i.e. Danishness, and the ‘foreign’; he opens up a dialogue between nationalism and internationalism with the intention of almost literally placing Europe in Denmark and vice versa. The future was going to be realized in the present. Georg Brandes tried to persuade the young writers of Scandinavia to adopt liberalism and naturalism in their novels and dramas, which some of them proceeded to do, convinced as they were that Brandes’ ideas regarding a new ‘debate’, moral and political, in literature, were right. However, they failed to implement these ideas as we can see by a reading of, for example, Jens Peter Jacobsen’s Niels Lyhne (1880; Henrik Ibsen’s Rosmersholm (1886 and Henrik Pontoppidan’s Lykke-Per (1898–1904. Emanicipating the mind and liberating oneself from the reactionary forces of society turned out to be too difficult for the women and men of what Brandes himself referred to as The Modern Breakthrough. Looking at Brandes today we may conclude that he was ahead of his own time as well as ahead of ours. His ideas regarding a new internationalism and liberalism are far from being realized.

  17. Three novel mutations in Iranian patients with Tay-Sachs disease.

    Science.gov (United States)

    Jamali, Solmaz; Eskandari, Nasim; Aryani, Omid; Salehpour, Shadab; Zaman, Talieh; Kamalidehghan, Behnam; Houshmand, Massoud

    2014-01-01

    Tay-Sachs disease (TSD), or GM2 gangliosidosis, is a lethal autosomal recessive neurodegenerative disorder, which is caused by a deficiency of beta-hexosaminidase A (HEXA), resulting in lysosomal accumulation of GM2 ganglioside. The aim of this study was to identify the TSD-causing mutations in an Iranian population. In this study, we examined 31 patients for TSD-causing mutations using PCR, followed by restriction enzyme digestion. Molecular genetics analysis of DNA from 23 patients of TSD revealed mutations that has been previously reported, including four-base duplications c.1274_1277dupTATC in exon 11 and IVS2+1G>A, deletion TTAGGCAAGGGC in exon 10 as well as a few novel mutations, including C331G, which altered Gln>Glu in HEXB, A>G, T>C, and p.R510X in exon 14, which predicted a termination codon or nonsense mutation. In conclusion, with the discovery of these novel mutations, the genotypic spectrum of Iranian patients with TSD disease has been extended and could facilitate definition of disease-related mutations.

  18. Adeno-Associated Virus Gene Therapy in a Sheep Model of Tay-Sachs Disease.

    Science.gov (United States)

    Gray-Edwards, Heather L; Randle, Ashley N; Maitland, Stacy A; Benatti, Hector R; Hubbard, Spencer M; Canning, Peter F; Vogel, Matthew B; Brunson, Brandon L; Hwang, Misako; Ellis, Lauren E; Bradbury, Allison M; Gentry, Atoska S; Taylor, Amanda R; Wooldridge, Anne A; Wilhite, Dewey R; Winter, Randolph L; Whitlock, Brian K; Johnson, Jacob A; Holland, Merilee; Salibi, Nouha; Beyers, Ronald J; Sartin, James L; Denney, Thomas S; Cox, Nancy R; Sena-Esteves, Miguel; Martin, Douglas R

    2018-03-01

    Tay-Sachs disease (TSD) is a fatal neurodegenerative disorder caused by a deficiency of the enzyme hexosaminidase A (HexA). TSD also occurs in sheep, the only experimental model of TSD that has clinical signs of disease. The natural history of sheep TSD was characterized using serial neurological evaluations, 7 Tesla magnetic resonance imaging, echocardiograms, electrodiagnostics, and cerebrospinal fluid biomarkers. Intracranial gene therapy was also tested using AAVrh8 monocistronic vectors encoding the α-subunit of Hex (TSD α) or a mixture of two vectors encoding both the α and β subunits separately (TSD α + β) injected at high (1.3 × 10 13 vector genomes) or low (4.2 × 10 12 vector genomes) dose. Delay of symptom onset and/or reduction of acquired symptoms were noted in all adeno-associated virus-treated sheep. Postmortem evaluation showed superior HexA and vector genome distribution in the brain of TSD α + β sheep compared to TSD α sheep, but spinal cord distribution was low in all groups. Isozyme analysis showed superior HexA formation after treatment with both vectors (TSD α + β), and ganglioside clearance was most widespread in the TSD α + β high-dose sheep. Microglial activation and proliferation in TSD sheep-most prominent in the cerebrum-were attenuated after gene therapy. This report demonstrates therapeutic efficacy for TSD in the sheep brain, which is on the same order of magnitude as a child's brain.

  19. Simultaneous preimplantation genetic diagnosis for Tay-Sachs and Gaucher disease.

    Science.gov (United States)

    Altarescu, Gheona; Brooks, Barry; Margalioth, Ehud; Eldar Geva, Talia; Levy-Lahad, Ephrat; Renbaum, Paul

    2007-07-01

    Preimplantation genetic diagnosis (PGD) for single gene defects is described for a family in which each parent is a carrier of both Tay-Sachs (TS) and Gaucher disease (GD). A multiplex fluorescent polymerase chain reaction protocol was developed that simultaneously amplified all four familial mutations and 10 informative microsatellite markers. In one PGD cycle, seven blastomeres were analysed, reaching a conclusive diagnosis in six out of seven embryos for TS and in five out of seven embryos for GD. Of the six diagnosed embryos, one was wild type for both TS and GD, and three were wild type for GD and carriers of TS. Two remaining embryos were compound heterozygotes for TS. Two transferable embryos developed into blastocysts (wt/wt and wt GD/carrier TS) and both were transferred on day 5. This single cycle of PGD resulted in a healthy live child. Allele drop-out (ADO) was observed in three of 34 reactions, yielding an 8% ADO rate. The occurrence of ADO in single cell analysis and undetected recombination events are primary causes of misdiagnosis in PGD and emphasize the need to use multiple polymorphic markers. So far as is known, this is the first report of concomitant PGD for two frequent Ashkenazi Jewish recessive disorders.

  20. Radioimmunoassay and heat denaturation enzyme assay for the detection of Tay-Sachs heterozygotes during pregnancy

    International Nuclear Information System (INIS)

    Nguyen, C.; Gold, R.J.M.; Mahuran, D.; Lowden, J.A.

    1981-01-01

    Tay-Sachs disease results from a loss of activity of hexosaminidase A (HEX A) in body tissues and fluids. Heterozygotes for the disease are usually identified by their relatively low ratio of heat-labile HEX A to total hexosaminidase. During pregnancy an intermediate isoenzyme (HEX I) increases in activity in serum and obscures the heterozygote status. HEX I does not increase in leucocytes, tears and other body tissues but because of technical difficulties in these assays the authors examined the feasibility of using a radioimmunoassay for HEX A. By univariate analysis, the heat denaturation assay gave a lower cost of misclassification for non-pregnant normals while RIA did so for pregnant normals. A combination of both tests led to reduced cost of misclassification compared to either alone. Bayesian analysis of bivariate gaussian density functions for heat denaturation and for radioimmunoassays of HEX isoenzymes was employed to calculate misclassification frequencies. Among the parameters examined, HEX A measured by RIA and % HEX A by heat-denaturation assay were the two having the best discriminatory power. (Auth.)

  1. High school Tay-Sachs disease carrier screening: 5 to 11-year follow-up.

    Science.gov (United States)

    Curd, Helen; Lewis, Sharon; Macciocca, Ivan; Sahhar, Margaret; Petrou, Vicki; Bankier, Agnes; Lieberman, Sari; Levy-Lahad, Ephrat; Delatycki, Martin B

    2014-04-01

    The Melbourne high school Tay-Sachs disease (TSD) carrier screening program began in 1997. The aim of this study was to assess the outcomes of this screening program among those who had testing more than 5 years ago, to evaluate the long-term impact of screening. A questionnaire was used for data collection and consisted of validated scales and purposively designed questions. Questionnaires were sent to all carriers and two non-carriers for each carrier who were screened in the program between 1999 and 2005. Twenty-four out of 69 (34.8 %) carriers and 30/138 (21.7 %) non-carriers completed the questionnaire. Most participants (82 %) retained good knowledge of TSD and there was no evidence of a difference in knowledge between carriers and non-carriers. Most participants (83 %) were happy with the timing and setting of screening and thought that education and screening for TSD should be offered during high school. There was no difference between carriers and non-carriers in mean scores for the State Trait Anxiety Inventory and Decision Regret Scale. This evaluation indicated that 5-11 years post high school screening, those who were screened are supportive of the program and that negative consequences are rare.

  2. The presence of two different infantile Tay-Sachs disease mutations in a Cajun population

    Energy Technology Data Exchange (ETDEWEB)

    McDowell, G.A.; Blitzer, M.G. (Univ. of Maryland School of Medicine, Baltimore, MD (United States)); Mules, E.H. (Kennedy Krieger Institute, Baltimore, MD (United States)); Fabacher, P. (Office of Public Health, Shreveport, LA (United States)); Shapira, E. (Tulane Univ. of School of Medicine, New Orleans, LA (United States))

    1992-11-01

    A study was undertaken to characterize the mutation(s) responsible for Tay-Sachs disease (TSD) in a Cajun population in southwest Louisiana and to identify the origins of these mutations. Eleven of 12 infantile TSD alleles examined in six families had the [beta]-hexosaminidase A (Hex A) [alpha]-subunit exon 11 insertion mutation that is present in approximately 70% of Ashkenazi Jewish TSD heterozygotes. The mutation in the remaining allele was a single-base transition in the donor splice site of the [alpha]-subunit intron 9. To determine the origins of these two mutations in the Cajun population, the TSD carrier status was enzymatically determined for 90 members of four of the six families, and extensive pedigrees were constructed for all carriers. A single ancestral couple from France was found to be common to most of the carriers of the exon 11 insertion. Pedigree data suggest that this mutation has been in the Cajun population since its founding over 2 centuries ago and that it may be widely distributed within the population. In contrast, the intron 9 mutation apparently was introduced within the last century and probably is limited to a few Louisiana families. 29 refs., 4 figs.

  3. Ashkenazi Jewish population screening for Tay-Sachs disease: the international and Australian experience.

    Science.gov (United States)

    Lew, Raelia M; Burnett, Leslie; Proos, Anné L; Barlow-Stewart, Kristine; Delatycki, Martin B; Bankier, Agnes; Aizenberg, Harry; Field, Michael J; Berman, Yemima; Fleischer, Ronald; Fietz, Michael

    2015-03-01

    Internationally, Tay-Sachs disease (TSD) preconception screening of Ashkenazi Jewish (AJ) individuals and couples has led to effective primary prevention of TSD. In Australia, adolescent preconception genetic screening programs operate mainly in Jewish community high schools. These existing programs offer an effective means of primary prevention of TSD, are cost effective and safe. However, in the broader Australian community TSD screening is not systematically performed and cases still occur in unscreened AJ individuals. In order to improve the effectiveness of Australian screening, there is a need for definitive guidelines for healthcare professionals to facilitate extension of the proven benefits of preconception TSD screening to all AJ individuals at risk. We performed a systematic review of the relevant literature relating to AJ pre-conception and antenatal screening for TSD. The evidence was assessed using an established National Health and Medical Research Council evidence grading system. Evaluations of efficacy of TSD screening programs design and execution, cost-benefit and cost-utility health economic evaluation, and population outcomes were undertaken. The results have been used to propose a model for universal AJ TSD preconception and antenatal screening for the primary care setting. © 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  4. Spontaneous appearance of Tay-Sachs disease in an animal model.

    Science.gov (United States)

    Zeng, B J; Torres, P A; Viner, T C; Wang, Z H; Raghavan, S S; Alroy, J; Pastores, G M; Kolodny, E H

    2008-01-01

    Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder due to an autosomal recessively inherited deficiency of beta-hexosaminidase A (Hex A). Deficiency of Hex A in TSD is caused by a defect of the alpha-subunit resulting from mutations of the HEXA gene. To date, there is no effective treatment for TSD. Animal models of genetic diseases, similar to those known to exist in humans, are valuable and essential research tools for the study of potentially effective therapies. However, there is no ideal animal model of TSD available for use in therapeutic trials. In the present study, we report an animal model (American flamingo; Phoenicopterus ruber) of TSD with Hex A deficiency occurring spontaneously in nature, with accumulation of G(M2)-ganglioside, deficiency of Hex A enzymatic activity, and a homozygous P469L mutation in exon 12 of the hexa gene. In addition, we have isolated the full-length cDNA sequence of the flamingo, which consists of 1581 nucleotides encoding a protein of 527 amino acids. Its coding sequence indicates approximately 71% identity at the nucleotide level and about 72.5% identity at the amino acid level with the encoding region of the human HEXA gene. This animal model, with many of the same features as TSD in humans, could represent a valuable resource for investigating therapy of TSD.

  5. Integrated Sachs-Wolfe effect versus redshift test for the cosmological parameters

    Science.gov (United States)

    Kantowski, R.; Chen, B.; Dai, X.

    2015-04-01

    We describe a method using the integrated Sachs-Wolfe (ISW) effect caused by individual inhomogeneities to determine the cosmological parameters H0, Ωm , and ΩΛ, etc. This ISW-redshift test requires detailed knowledge of the internal kinematics of a set of individual density perturbations, e.g., galaxy clusters and/or cosmic voids, in particular their density and velocity profiles, and their mass accretion rates. It assumes the density perturbations are isolated and embedded (equivalently compensated) and makes use of the newly found relation between the ISW temperature perturbation of the cosmic microwave background (CMB) and the Fermat potential of the lens. Given measurements of the amplitudes of the temperature variations in the CMB caused by such clusters or voids at various redshifts and estimates of their angular sizes or masses, one can constrain the cosmological parameters. More realistically, the converse is more likely, i.e., if the background cosmology is sufficiently constrained, measurement of ISW profiles of clusters and voids (e.g., hot and cold spots and rings) can constrain dynamical properties of the dark matter, including accretion, associated with such lenses and thus constrain the evolution of these objects with redshift.

  6. Fortælleværksteder

    DEFF Research Database (Denmark)

    Krøjer, Jo; Hutters, Camilla

    2009-01-01

    Unges valg af videregående uddannelse er omgærdet af forventninger. Forventninger til hvad man skal vælge. Forventninger til hvor lang tid, man skal være om at tage en uddannelse. Og forventninger til, hvad uddannelsen skal føre til. Artiklen præsenterer fortælleværkstedet, en metode til kollekti...... refleksioner over egne og adres forventninger til og tanker om uddannelsesvalg....

  7. Fort St. Vrain circulator operating experience

    Energy Technology Data Exchange (ETDEWEB)

    Brey, H. L.

    1988-08-15

    Fort St. Vrain, on the system of Public Service Company of Colorado, is the only high-temperature gas-cooled power reactor in the United States. Four helium circulators are utilized in this plant to transfer heat from the reactor to the steam generators. These unique machines have a single stage axial flow helium compressor driven by a single stage steam turbine. A single stage water driven (pelton wheel) turbine is the back-up drive utilizing either feed water, condensate, or fire water as the driving fluid. Developmental testing of the circulators was accomplished prior to installation into Fort St. Vrain. A combined machine operating history of approximately 250,000 hours has shown these machines to be of conservative design and proven mechanical integrity. However, many problems have been encountered in operating the complex auxiliaries which are necessary for successful circulator and plant operation. It has been 15 years since initial installation of the circulators occurred at Fort St. Vrain. During this time, a number of significant issues had to be resolved dealing specifically with machine performance. These events include cavitation damage of the pelton wheels during the initial plant hot functional testing, cracks in the water turbine buckets and cervic coupling, static shutdown seal bellows failure, and, most recently, degradation of components within the steam drive assembly. Unreliable operation particularly with the circulator auxiliaries has been a focus of attention by Public Service Company of Colorado. Actions to replace or significantly modify the existing circulators and their auxiliaries are currently awaiting decisions concerning the long-term future of the Fort St. Vrain plant. (author). 10 refs, 7 figs, 2 tabs.

  8. Rail Outloading Capability Study, Fort Polk, Louisiana,

    Science.gov (United States)

    1977-06-01

    regardless of experience, to avoid wasted man -hours. The main problem at Fort Polk is that no blocking and bracing material stockpile exists and no...ti1 hottul only thtrough the 0111crinost hole; to defect within 20 days after it is determined to -tuit Owt ttrtk in tuse. III thle caste of classes 3...wheels, slipping, or similar trak (meh causes. 1 -------------------- (12) " Shelly spots" means a condition 2 ------------------------ % where a thin

  9. Fort St. Vrain circulator operating experience

    International Nuclear Information System (INIS)

    Brey, H.L.

    1988-01-01

    Fort St. Vrain, on the system of Public Service Company of Colorado, is the only high-temperature gas-cooled power reactor in the United States. Four helium circulators are utilized in this plant to transfer heat from the reactor to the steam generators. These unique machines have a single stage axial flow helium compressor driven by a single stage steam turbine. A single stage water driven (pelton wheel) turbine is the back-up drive utilizing either feed water, condensate, or fire water as the driving fluid. Developmental testing of the circulators was accomplished prior to installation into Fort St. Vrain. A combined machine operating history of approximately 250,000 hours has shown these machines to be of conservative design and proven mechanical integrity. However, many problems have been encountered in operating the complex auxiliaries which are necessary for successful circulator and plant operation. It has been 15 years since initial installation of the circulators occurred at Fort St. Vrain. During this time, a number of significant issues had to be resolved dealing specifically with machine performance. These events include cavitation damage of the pelton wheels during the initial plant hot functional testing, cracks in the water turbine buckets and cervic coupling, static shutdown seal bellows failure, and, most recently, degradation of components within the steam drive assembly. Unreliable operation particularly with the circulator auxiliaries has been a focus of attention by Public Service Company of Colorado. Actions to replace or significantly modify the existing circulators and their auxiliaries are currently awaiting decisions concerning the long-term future of the Fort St. Vrain plant. (author). 10 refs, 7 figs, 2 tabs

  10. Renewable Energy Opportunities at Fort Hood, Texas

    Energy Technology Data Exchange (ETDEWEB)

    Chvala, William D.; Warwick, William M.; Dixon, Douglas R.; Solana, Amy E.; Weimar, Mark R.; States, Jennifer C.; Reilly, Raymond W.

    2008-06-30

    The document provides an overview of renewable resource potential at Fort Hood based primarily upon analysis of secondary data sources supplemented with limited on-site evaluations. The effort was funded by the U.S. Army Installation Management Command (IMCOM) as follow-on to the 2005 DoD Renewables Assessment. This effort focuses on grid-connected generation of electricity from renewable energy sources and also ground source heat pumps for heating and cooling buildings, as directed by IMCOM.

  11. FIND: Fort Calhoun Station, Unit 2

    International Nuclear Information System (INIS)

    Williams, W.H.

    1976-07-01

    This index is presented for the microfiche material of Docket 50548 which concerns the application of Omaha Public Power District to build and operate Fort Calhoun Station, Unit 2. The information includes both application and review material dated from September 1975 through March 1976. There are five amendments to the PSAR and one supplement to the ER which have been incorporated by reference into the respective reports. Docket RESAR-3 is used as a reference for portions of the PSAR

  12. Aktjorõ filma "Georg" vpervõje sobralis vmestje

    Index Scriptorium Estoniae

    2006-01-01

    Vene näitlejanna Anastasia Makejeva ja eesti laulev näitleja Marko Matvere, kes mängivad Peeter Simmi töös olevas mängufilmis "Georg" Georg Otsa ja tema teist abikaasat Asta Otsa, alustavad homme filmivõtteid

  13. Gilbert ja George lasevad oma kunstiteose alla laadida

    Index Scriptorium Estoniae

    2007-01-01

    9.05.2007 öösel on kõigil soovijail võimalik tasuta interneti vahendusel omandada Londonis tegutseva kunstnikepaari Gilbert & George (Gilbert Proesch ja George Passmore) teos "Planed". Aadressid: www.bbc.co.uk/imagine ja www.guardian.co.uk/art

  14. George Hoyt Whipple and the Impact of Liver on Anemia

    Science.gov (United States)

    dropdown arrow Site Map A-Z Index Menu Synopsis George Hoyt Whipple and the Impact of Liver on Anemia cases of anemia". George Hoyt Whipple Courtesy of University of Rochester Medical Center Whipple's main research was concerned with anemia and with the physiology and pathology of the liver. He won the

  15. Tay-Sachs and Sandhoff diseases: enzymatic diagnosis in dried blood spots on filter paper: retrospective diagnoses in newborn-screening cards.

    Science.gov (United States)

    Chamoles, Néstor A; Blanco, Mariana; Gaggioli, Daniela; Casentini, Carina

    2002-04-01

    Tay-Sachs disease (TSD), Sandhoff disease (SD) and variants are caused by deficient activity of the lysosomal enzymes hexosaminidase A (HA) and total hexosaminidase (TH) (hexosaminidase A plus B), respectively. For diagnosis, these enzymes are usually measured in plasma or extracts of leukocytes. We describe methods for the assay of hexosaminidase A and total hexosaminidase activities in dried blood spots (DBSs) on filter paper. We studied 163 healthy controls, 9 Tay-Sachs patients, 4 Sandhoff patients, 18 obligate carriers and the newborn-screening cards from two patients with Tay-Sachs and one patient with Sandhoff disease. To tubes containing a 3-mm-diameter blood spot, we added elution liquid and substrate solution. After incubation at 37 degrees C, the amount of hydrolyzed product was compared with a calibrator to allow the quantification of enzyme activity. The described methodology is useful to distinguish patients with Tay-Sachs disease or Sandhoff disease from carriers and controls using samples that are sufficiently stable to be transported to the testing laboratory by mail. The diagnosis of both diseases from a newborn-screening card (NSC) was clearly demonstrated, even after storage for up to 38 months at room temperature. The newborn-screening card has been added to the biological materials that allow the identification of patients with Tay-Sachs disease and Sandhoff disease.

  16. 78 FR 17087 - Special Local Regulation; New River Raft Race, New River; Fort Lauderdale, FL

    Science.gov (United States)

    2013-03-20

    ...-AA08 Special Local Regulation; New River Raft Race, New River; Fort Lauderdale, FL AGENCY: Coast Guard... on the New River in Fort Lauderdale, Florida during the Rotary Club of Fort Lauderdale New River Raft... States during the Rotary Club of Fort Lauderdale New River Raft Race. On March 23, 2013, Fort Lauderdale...

  17. The WIMS-E module W-FORTE

    International Nuclear Information System (INIS)

    Roth, M.J.

    1983-09-01

    There are three distinct versions of the WIMS lattice cell program. WIMS-E is the most general, WIMSD4 is restricted to clusters or to one dimensional slab or annular geometry, and LWRWIMS is designed principally for light water reactor geometries. W-FORTE is used to transfer data from WIMSD4 or LWRWIMS to WIMS-E. A description of the W-FORTE module is given, and includes the relevant data for WIMSD4, LWRWIMS and W-FORTE. (UK)

  18. George Gamow: Scientific Amateur and Polymath

    Science.gov (United States)

    Harper, Eamon

    George Gamow (1904-1968) was among the first of the many brilliant scientists who forsook Europe for the United States in the early 1930s. Although most were fleeing the fascist imperium of Hitler and Mussolini, Gamow was one of a few who managed to escape the burgeoning despotism of Stalin in the Soviet Union. His early application of quantum mechanics to the atomic nucleus and his subsequent insight into the role played by the physics of the atom and its nucleus in stars, galaxies, and the universe identifies him as a scientist of unusual genius. Gamow displayed a boisterous, infectious - almost Rutherfordian - interest in all aspects of pure science. His interests were broad and his industry prodigious. His scientific output covered areas as diverse as nuclear physics, astrophysics, cosmology, biological genetics, and the fascinating question of the relationship of the large-scale structure and development of the universe to the properties of elementary particles and fields. He also was an immensely imaginative and prolific author of popular expositions on scientific subjects. One who is as well-known for his authorship of the Mr. Tompkins series of science popularizations as for his contributions to the development of the physical consequences of the big-bang theory of the expanding universe and the prediction of the cosmic background radiation must be unique in the scientific pantheon.

  19. George Smoot talks to the Bulletin

    CERN Multimedia

    CERN Bulletin

    2011-01-01

    Antimatter, dark matter, dark energy, the nature of space and time… The Nobel Laureate George Smoot answers Paola Catapano’s questions about his career and the many issues about the Universe that are still open.   You started your scientific career as a particle physicist, but soon you moved to astrophysics and in particular the Big Bang theory and cosmology. What motivated your interest in the Big Bang theory? After I graduated from MIT, I went to Berkeley to work with particle physicist Luis Alvarez. He knew I was interested in many areas and said, “tell me what you would like to do and we’ll try and work on that”. I saw astrophysics as a new field, with a lot of new and exciting opportunities. I started doing experiments looking for antimatter, which eventually led to the idea of ASTROMAG and later to AMS. Studying antimatter, we found that it was in less than one part in 10,000. I thought: “There’s no antimatter around us ...

  20. Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population

    Directory of Open Access Journals (Sweden)

    Anaita Udwadia-Hegde MD, DCH, MRCPCH

    2017-01-01

    Full Text Available Background: Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. Patient: We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. Conclusion: This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

  1. Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population.

    Science.gov (United States)

    Udwadia-Hegde, Anaita; Hajirnis, Omkar

    2017-01-01

    Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

  2. The effect of a combined glenoid and Hill-Sachs defect on glenohumeral stability: a biomechanical cadaveric study using 3-dimensional modeling of 142 patients.

    Science.gov (United States)

    Arciero, Robert A; Parrino, Anthony; Bernhardson, Andrew S; Diaz-Doran, Vilmaris; Obopilwe, Elifho; Cote, Mark P; Golijanin, Petr; Mazzocca, Augustus D; Provencher, Matthew T

    2015-06-01

    Bone loss in anterior glenohumeral instability occurs on both the glenoid and the humerus; however, existing biomechanical studies have evaluated glenoid and humeral head defects in isolation. Thus, little is known about the combined effect of these bony lesions in a clinically relevant model on glenohumeral stability. The purpose of this study was to determine the biomechanical efficacy of a Bankart repair in the setting of bipolar (glenoid and humeral head) bone defects determined via computer-generated 3-dimensional (3D) modeling of 142 patients with recurrent anterior shoulder instability. The null hypothesis was that adding a bipolar bone defect will have no effect on glenohumeral stability after soft tissue Bankart repair. Controlled laboratory study. A total of 142 consecutive patients with recurrent anterior instability were analyzed with 3D computed tomography scans. Two Hill-Sachs lesions were selected on the basis of volumetric size representing the 25th percentile (0.87 cm(3); small) and 50th percentile (1.47 cm(3); medium) and printed in plastic resin with a 3D printer. A total of 21 cadaveric shoulders were evaluated on a custom shoulder-testing device permitting 6 degrees of freedom, and the force required to translate the humeral head anteriorly 10 mm at a rate of 2.0 mm/s with a compressive load of 50 N was determined at 60° of glenohumeral abduction and 60° of external rotation. All Bankart lesions were made sharply from the 2- to 6-o'clock positions for a right shoulder. Subsequent Bankart repair with transosseous tunnels using high-strength suture was performed. Hill-Sachs lesions were made in the cadaver utilizing a plastic mold from the exact replica off the 3D printer. Testing was conducted in the following sequence for each specimen: (1) intact, (2) posterior capsulotomy, (3) Bankart lesion, (4) Bankart repair, (5) Bankart lesion with 2-mm glenoid defect, (6) Bankart repair, (7) Bankart lesion with 2-mm glenoid defect and Hill-Sachs lesion

  3. Magnification-temperature correlation: The dark side of integrated Sachs-Wolfe measurements

    International Nuclear Information System (INIS)

    LoVerde, Marilena; Hui, Lam; Gaztanaga, Enrique

    2007-01-01

    Integrated Sachs-Wolfe (ISW) measurements, which involve cross-correlating the microwave background anisotropies with the foreground large-scale structure (e.g. traced by galaxies/quasars), have proven to be an interesting probe of dark energy. We show that magnification bias, which is the inevitable modulation of the foreground number counts by gravitational lensing, alters both the scale dependence and amplitude of the observed ISW signal. This is true especially at high redshifts because (1) the intrinsic galaxy-temperature signal diminishes greatly back in the matter-dominated era, (2) the lensing efficiency increases with redshift and (3) the number count slope generally steepens with redshift in a magnitude limited sample. At z > or approx. 2, the magnification-temperature correlation dominates over the intrinsic galaxy-temperature correlation and causes the observed ISW signal to increase with redshift, despite dark energy subdominance--a result of the fact that magnification probes structures all the way from the observer to the sources. Ignoring magnification bias therefore can lead to (significantly) erroneous conclusions about dark energy. While the lensing modulation opens up an interesting high z window for ISW measurements, high redshift measurements are not expected to add much new information to low redshift ones if dark energy is indeed the cosmological constant. This is because lensing introduces significant covariance across redshifts. The most compelling reasons for pursuing high redshift ISW measurements are to look for potential surprises such as early dark energy domination or signatures of modified gravity. We conclude with a discussion of existing measurements, the highest redshift of which is at the margin of being sensitive to the magnification effect. We also develop a formalism which might be of more general interest: to predict biases in estimating parameters when certain physical effects are ignored in interpreting observations

  4. Updated tomographic analysis of the integrated Sachs-Wolfe effect and implications for dark energy

    Science.gov (United States)

    Stölzner, Benjamin; Cuoco, Alessandro; Lesgourgues, Julien; Bilicki, Maciej

    2018-03-01

    We derive updated constraints on the integrated Sachs-Wolfe (ISW) effect through cross-correlation of the cosmic microwave background with galaxy surveys. We improve with respect to similar previous analyses in several ways. First, we use the most recent versions of extragalactic object catalogs, SDSS DR12 photometric redshift (photo-z ) and 2MASS Photo-z data sets, as well as those employed earlier for ISW, SDSS QSO photo-z and NVSS samples. Second, we use for the first time the WISE × SuperCOSMOS catalog, which allows us to perform an all-sky analysis of the ISW up to z ˜0.4 . Third, thanks to the use of photo-z s , we separate each data set into different redshift bins, deriving the cross-correlation in each bin. This last step leads to a significant improvement in sensitivity. We remove cross-correlation between catalogs using masks which mutually exclude common regions of the sky. We use two methods to quantify the significance of the ISW effect. In the first one, we fix the cosmological model, derive linear galaxy biases of the catalogs, and then evaluate the significance of the ISW using a single parameter. In the second approach we perform a global fit of the ISW and of the galaxy biases varying the cosmological model. We find significances of the ISW in the range 4.7 - 5.0 σ thus reaching, for the first time in such an analysis, the threshold of 5 σ . Without the redshift tomography we find a significance of ˜4.0 σ , which shows the importance of the binning method. Finally we use the ISW data to infer constraints on the dark energy redshift evolution and equation of state. We find that the redshift range covered by the catalogs is still not optimal to derive strong constraints, although this goal will be likely reached using future datasets such as from Euclid, LSST, and SKA.

  5. Serial CT scans and MRI scans of Tay-Sachs disease

    International Nuclear Information System (INIS)

    Shimoizumi, Hideo; Miyao, Masutomo; Ichihashi, Koh; Sawa, Rituko; Yamamoto, Yoshifumi; Tanaka, Osamu; Yanagisawa, Masayoshi; Kamoshita, Shigehiko.

    1986-01-01

    Serial X-ray CT and MRI were performed on 2 cases of Tay-Sachs disease. Case 1 : a 3-year-10-month-old girl. Her developmental milestones were normal until the age of 6 months. At the age of 10 months, hypotonia and unduly sensitiveness to sounds were noticed. She had cherry red spots in both fundi, and the serum hexosaminidase A activity was low. Significant clinical features during the next 3 years included regression of developmental milestones to the level of one month, and myoclonic and generalised seizures. After 3 years she showed megalencephaly, unstable body temperature, and respiratory disorders. Case 2 : a 2-year-6-month-old boy. His developmental milestones were normal until the age of 5 months. Then he manifested hyperreaction to noises and further developments ceased. He started to deteriorate from the age of 1 year and 2 months, and had no head control at the age of 1 year and 6 months. Cherry red spots were detected and the serum hexsosaminidase A activity was low, too. X-ray CT at the early stage of cases 1 and 2 showed mild cerebral atrophy and high density areas in bilateral thalami and basal ganglia. At the late stage of Case 1, high density areas appeared in occipital and frontal white matters. MRI in both patients were strikingly similar. In their grey matters, linear light areas were demonstrated on IR images. Their white matters showed extensive dark (long T 1 ) areas on IR images, and extensive light (long T 2 ) areas on SE images. Bilateral thalami and basal ganglia were light on IR images and dark on SE images. The findings of MRI in our patients seemed to correlate well with pathological and biochemical changes in their brains. (author)

  6. Characterization of inducible models of Tay-Sachs and related disease.

    Science.gov (United States)

    Sargeant, Timothy J; Drage, Deborah J; Wang, Susan; Apostolakis, Apostolos A; Cox, Timothy M; Cachón-González, M Begoña

    2012-09-01

    Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescues the acute neurodegenerative illness in Hexb-/- (Sandhoff) mice that lack β-hexosaminidases A and B. To define determinants of therapeutic efficacy and establish a dynamic experimental platform to systematically investigate cellular pathogenesis of GM2 gangliosidosis, we generated two inducible experimental models. Reversible transgenic expression of β-hexosaminidase directed by two promoters, mouse Hexb and human Synapsin 1 promoters, permitted progression of GM2 gangliosidosis in Sandhoff mice to be modified at pre-defined ages. A single auto-regulatory tetracycline-sensitive expression cassette controlled expression of transgenic Hexb in the brain of Hexb-/- mice and provided long-term rescue from the acute neuronopathic disorder, as well as the accompanying pathological storage of glycoconjugates and gliosis in most parts of the brain. Ultimately, late-onset brainstem and ventral spinal cord pathology occurred and was associated with increased tone in the limbs. Silencing transgenic Hexb expression in five-week-old mice induced stereotypic signs and progression of Sandhoff disease, including tremor, bradykinesia, and hind-limb paralysis. As in germline Hexb-/- mice, these neurodegenerative manifestations advanced rapidly, indicating that the pathogenesis and progression of GM2 gangliosidosis is not influenced by developmental events in the maturing nervous system.

  7. Transient HEXA expression in a transformed human fetal Tay-Sachs disease neuroglial cell line

    Energy Technology Data Exchange (ETDEWEB)

    Fernandes, M.J.; Hechtman, P.; Kaplan, F. [McGill Univ., Quebec (Canada)] [and others

    1994-09-01

    Tay-Sachs disease (TSD) is a severe neurodegenerative disorder characterized by the accumulation of GM{sub 2} ganglioside in the neurons of the central cortex. The recessively inherited disorder results from deficiency of hexosaminidase A (Hex A), a heterodimer of an {alpha} and {beta} subunit encoded by the HEXA and HEXB genes. Expression of HEXA mutations in COS cells has several disadvantages including high endogenous hexosaminidase activity. We report a new transient expression system with very low endogenous Hex A activity. An SV40-transformed fetal TSD neuroglial cell line was assessed for transient expression of the HEXA gene. pCMV{alpha}, a vector incorporating the cytomegalovirus promoter with the human {alpha}-subunit cDNA insert, proved to be the most efficient expression vector. Transfection of 4x10{sup 6} cells with 5-20 {mu}g of plasmid resulted in 100 to 500-fold Hex A activity (4MUGS hydrolysis) relative to mock-transfected cells. Use of pCMV{beta}-Gal as a control for transfection efficiency indicated that 10-20% of cells were transfected. Hex A specific activity increased for at least 72 h post-transfection. This new transient expression system should greatly improve the characterization of mutations in which low levels of HEXA expression result in milder clinical phenotypes and permit studies on enzymatic properties of mutant forms of Hex A. Since the cells used are of CNS origin and synthesize gangliosides, it should also be possible to study, in culture, the metabolic phenotype associated with TSD.

  8. Molecular characterization of both alleles in an unusual Tay-Sachs disease BI variant

    Energy Technology Data Exchange (ETDEWEB)

    Coulter-Mackie, M.B. (Univ. of Western Ontario, London (Canada) Child Health Research Institute, Children' s Hospital of Western Ontario, London (Canada) Child Parent Resource Institute, London, Ontario (Canada))

    1994-06-01

    In a recent report, the authors described an exon 6 mutation in a Tay-Sachs B1 variant patient, first reported by Gordon et al. (1988), who displayed a typical B1 variant biochemical phenotype - i.e., (a) significant levels of hexosaminidase A (Hex A) activity in an assay with a neutral synthetic substrate, 4-methylumbelliferyl-[beta]-N-acetylglucosamide, and (b) <2% of control Hex A in a test on the sulfated substrate, 4-methylumbelliferyl-[beta]-N-acetylglucosamide-6-sulfate. The patient was found to carry a double mutation (G[sub 574][yields]C [val[sub 192][yields]leu] and G[sub 598][yields]A [val[sub 200][yields]met]) inherited from her mother. Only the 574 mutation produced a deleterious effect on Hex A activity in transfected COS0-1 cells, producing a B1 variant biochemical phenotype. The paternal allele apparently caused decreased abundance of mRNA, since no candidate paternal mutations were found in cloned reverse transcription-PCR (RT-PCR) products in the reported study. The biochemical phenotype of the original patient and the properties of the cDNA carrying the G[sub 574] [yields] C mutation in transient expression studies were compatible with a B1 variant mutation. The possibility remained that there might be some contribution from the paternal allele to the patient's phenotype. However, the paternal allele produces relatively low yields of a largely mis-spliced mRNA whose product would not be functional. Therefore, the G[sub 574] [yields] C (val[yields]leu) mutation in the maternal allele is clearly confirmed as a B1 variant mutation with all the ramifications for the substrate binding site and/or catalytic center that this implies.

  9. Tay Sachs disease in Australia: reduced disease incidence despite stable carrier frequency in Australian Jews.

    Science.gov (United States)

    Lew, Raelia M; Proos, Anne L; Burnett, Leslie; Delatycki, Martin; Bankier, Agnes; Fietz, Michael J

    2012-12-10

    To evaluate the outcomes of preconception screening of Jewish Australians for Tay Sachs disease (TSD) carrier status on Jewish TSD-affected births. Epidemiological observational study involving a complete retrospective audit of infantile and intermediate TSD cases diagnosed in Sydney and Melbourne between 1 January 1995 and 31 December 2011 (Royal Children's Hospital Melbourne; Pacific Laboratory Medicine Services, Pathology North, NSW Health Pathology, Sydney; Victorian Clinical Genetics Services, Melbourne; and SA Pathology, Adelaide), and carrier frequency among Jewish high school students attending schools participating in TSD screening programs over the same period. Jewish TSD carrier frequency; and expected versus observed Jewish TSD-affected births. The 2006 Census indicated that most of the total 88,826 Jewish Australians live in Melbourne (46%) and Sydney (40%). The 7,756 Jewish high school students screened for TSD in Sydney and Melbourne during the study period had a carrier frequency of one in 31 (3.26%; 95% CI, 2.89%-3.68%).The estimated expected number of TSD-affected births in Melbourne and Sydney in 1995-2011 was 4.1 for Jewish births and 7.4 for other births (a ratio of Jewish to non-Jewish births of 1:2). The actual number was 12 (four in Sydney and eight in Melbourne), of which two were Jewish (a ratio of Jewish to non-Jewish births of 1:5). This finding of fewer than expected Jewish TSD cases coincided with a period during which screening programs were operating. There have been no Jewish TSD-affected children born to parents who were screened previously. Community education, appreciation of autosomal recessive inheritance and genetic carrier screening before pregnancy are the likely factors in our finding of fewer than expected Jewish babies with TSD. Ongoing outcome monitoring must continue.

  10. Characterization of inducible models of Tay-Sachs and related disease.

    Directory of Open Access Journals (Sweden)

    Timothy J Sargeant

    2012-09-01

    Full Text Available Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescues the acute neurodegenerative illness in Hexb-/- (Sandhoff mice that lack β-hexosaminidases A and B. To define determinants of therapeutic efficacy and establish a dynamic experimental platform to systematically investigate cellular pathogenesis of GM2 gangliosidosis, we generated two inducible experimental models. Reversible transgenic expression of β-hexosaminidase directed by two promoters, mouse Hexb and human Synapsin 1 promoters, permitted progression of GM2 gangliosidosis in Sandhoff mice to be modified at pre-defined ages. A single auto-regulatory tetracycline-sensitive expression cassette controlled expression of transgenic Hexb in the brain of Hexb-/- mice and provided long-term rescue from the acute neuronopathic disorder, as well as the accompanying pathological storage of glycoconjugates and gliosis in most parts of the brain. Ultimately, late-onset brainstem and ventral spinal cord pathology occurred and was associated with increased tone in the limbs. Silencing transgenic Hexb expression in five-week-old mice induced stereotypic signs and progression of Sandhoff disease, including tremor, bradykinesia, and hind-limb paralysis. As in germline Hexb-/- mice, these neurodegenerative manifestations advanced rapidly, indicating that the pathogenesis and progression of GM2 gangliosidosis is not influenced by developmental events in the maturing nervous system.

  11. Probing the integrated Sachs-Wolfe effect using embedded lens models

    Science.gov (United States)

    Chen, B.; Kantowski, R.

    2015-04-01

    The photometry profile of the integrated Sachs-Wolfe (ISW) effect, recently obtained by the Planck consortium by stacking patches of cosmic microwave background (CMB) sky maps around a large number of cosmic voids, contains a cold ring at about half the void's effective radius surrounded by a hot ring near the void's boundary. The source of the temperature structure is assumed to be the ISW effect but the exact cause of the ringed structure is not currently well understood, particularly the outer hot ring. Numerical simulations have suggested that hot/cold ring structures can be produced by motions associated with nonlinear growths of cosmic structures whose gravitational potentials produce the ISW effect. We have recently developed the embedded lens theory and the Fermat potential formalism which can be used to model the ISW effect caused by intervening individual lens inhomogeneities evolving arbitrarily. This theory only requires knowledge of the void's projected mass profile as a function of the passing CMB photons' impact radius and the rate of change of that mass distribution at passage. We present two simple embedded void lens models with evolving mass densities and investigate the ISW effect caused by these lenses. Both models possess expanding mass shells which produce hot rings around central cold regions, consistent with the recent observations. By adding a small overdensity at the void's center we can produce the slight positive temperature excess hinted at in Planck's photometric results. We conclude that the embedded lens theory and the Fermat potential formalism is well suited for modeling the ISW effect.

  12. Embedded Lensing Time Delays, the Fermat Potential, and the Integrated Sachs-Wolfe Effect

    Science.gov (United States)

    Chen, Bin; Kantowski, Ronald; Dai, Xinyu

    2015-05-01

    We derive the Fermat potential for a spherically symmetric lens embedded in a Friedman-Lemaître-Robertson-Walker cosmology and use it to investigate the late-time integrated Sachs-Wolfe (ISW) effect, i.e., secondary temperature fluctuations in the cosmic microwave background (CMB) caused by individual large-scale clusters and voids. We present a simple analytical expression for the temperature fluctuation in the CMB across such a lens as a derivative of the lens’ Fermat potential. This formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. It is much simpler to use and makes the same predictions as conventional approaches. In this approach the total temperature fluctuation can be split into a time-delay part and an evolutionary part. Both parts must be included for cosmic structures that evolve and both can be equally important. We present very simple ISW models for cosmic voids and galaxy clusters to illustrate the ease of use of our formalism. We use the Fermat potentials of simple cosmic void models to compare predicted ISW effects with those recently extracted from WMAP and Planck data by stacking large cosmic voids using the aperture photometry method. If voids in the local universe with large density contrasts are no longer evolving we find that the time delay contribution alone predicts values consistent with the measurements. However, we find that for voids still evolving linearly, the evolutionary contribution cancels a significant part of the time delay contribution and results in predicted signals that are much smaller than recently observed.

  13. Pathological alterations typical of human Tay-Sachs disease, in the retina of a deep-sea fish

    Science.gov (United States)

    Fishelson, L.; Delarea, Yacov; Galil, Bella S.

    Micrographs of retinas from the deep-sea fish Cataetyx laticeps revealed visual cells containing membranous whorls in the ellipsoids of the inner segments resulting from stretching and modifications of the mitochondria membranes and their cristae. These pathological structures seem to be homologous to the whorls observed in retinas of human carriers of Tay-Sachs disease. This disease, a genetic disorder, is found in humans and some mammals. Our findings in fish suggest that the gene responsible can be found throughout the vertebrate evolutionary tree, possibly dormant in most taxa.

  14. Identification of two HEXA mutations causing infantile-onset Tay-Sachs disease in the Persian population.

    Science.gov (United States)

    Haghighi, Alireza; Rezazadeh, Jamileh; Shadmehri, Azam Ahmadi; Haghighi, Amirreza; Kornreich, Ruth; Desnick, Robert J

    2011-09-01

    The β-hexosaminidase A (HEXA) mutations in the first reported cases of infantile Tay-Sachs disease in the Persian population were identified in two unrelated consanguineous families. The clinical diagnoses of the affected infants were confirmed by their markedly deficient levels of HEXA activity in plasma or peripheral leukocytes. The specific causative mutation in each family was determined by sequencing the HEXA alleles in both sets of related parents. Two mutations were identified: c.1A>G (p.MIV), which obliterated the initiating methionine in codon 1, and c.1177C>T (p.R393X), which predicted a termination codon or nonsense mutation.

  15. Limb anomalies in DiGeorge and CHARGE syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Prasad, C.; Quackenbush, E.J.; Whiteman, D.; Korf, B. [Harvard Medical School, Boston, MA (United States)

    1997-01-20

    Limb anomalies are not common in the DiGeorge or CHARGE syndromes. We describe limb anomalies in two children, one with DiGeorge and the other with CHARGE syndrome. Our first patient had a bifid left thumb, Tetralogy of Fallot, absent thymus, right facial palsy, and a reduced number of T-cells. A deletion of 22q11 was detected by fluorescence in situ hybridization (FISH). The second patient, with CHARGE syndrome, had asymmetric findings that included right fifth finger clinodactyly, camptodactyly, tibial hemimelia and dimpling, and severe club-foot. The expanded spectrum of the DiGeorge and CHARGE syndromes includes limb anomalies. 14 refs., 4 figs.

  16. Twisting and Writhing with George Ellery Hale

    Science.gov (United States)

    Canfield, Richard C.

    2013-06-01

    Early in his productive career in astronomy, George Ellery Hale developed innovative solar instrumentation that allowed him to make narrow-band images. Among the solar phenomena he discovered were sunspot vortices, which he attributed to storms akin to cyclones in our own atmosphere. Using the concept of magnetic helicity, physicists and mathematicians describe the topology of magnetic fields, including twisting and writhing. Our contemporary understanding of Hale's vortices as a consequence of large-scale twist in sunspot magnetic fields hinges on a key property of helicity: conservation. I will describe the critical role that this property plays, when applied to twist and writhe, in a fundamental aspect of global solar magnetism: the hemispheric and solar cycle dependences of active region electric currents with respect to magnetic fields. With the advent of unbroken sequences of high-resolution magnetic images, such as those presently available from the Helioseismic and Magnetic Imager on Solar Dynamics Observatory, the flux of magnetic helicity through the photosphere can be observed quantitatively. As magnetic flux tubes buoy up through the convection zone, buffeted and shredded by turbulence, they break up into fragments by repeated random bifurcation. We track these rising flux fragments in the photosphere, and calculate the flux of energy and magnetic helicity there. Using a quantitative model of coronal currents, we also track connections between these fragments to calculate the energy and magnetic helicity stored at topological interfaces that are in some ways analogous to the storage of stress at faults in the Earth's crust. Comparison of these values to solar flares and interplanetary coronal mass ejections implies that this is the primary storage mechanism for energy and magnetic helicity released in those phenomena, and suggests a useful tool for quantitative prediction of geomagnetic storms.

  17. Status of the Fort St. Vrain decommissioning

    International Nuclear Information System (INIS)

    Fisher, M.J.

    1990-01-01

    Fort St. Vrain is a high temperature gas cooled reactor. It has been shut down as a result of financial and technical difficulties. Fort St. Vrain has been planning for defueling and decommissioning for at least three years. The preliminary decommissioning plan, in accordance with the NRC's final rule, has been submitted and is being reviewed by the NRC. The basis of the preliminary decommissioning plan has been SAFSTOR. Public Service Company, who is the owner and operator of FSV, is scheduled to submit a proposed decommissioning plan to the NRC in the fourth quarter of 1990. PSC has gone out for bid on the decontamination and dismantlement of FSV. This paper includes the defueling schedule, the independent spent fuel storage installation status, the probability of shipping fuel to DOE, the status of the preliminary decommissioning plan submittal, the issuance of a possession only license and what are the results of obtaining this license amendment, preliminary decommissioning activities allowed prior to the approval of a proposed decommissioning plan, the preparation of a proposed decommissioning plan and the status of our decision to proceed with SAFSTOR or DECON as identified in the NRC's final decommissioning rule

  18. Fort Mason Center: Pier 2 Project

    Energy Technology Data Exchange (ETDEWEB)

    Nester, Patrick [Fort Mason Center, San Francisco, CA (United States)

    2014-08-30

    The rooftop Photovoltaic (PV) panels and radiant piping project was constructed by Fort Mason Center as part of its $21 million comprehensive rehabilitation of the Pier 2 shed which include the shed’s electrical, natural gas and water systems. Fort Mason Center improved performance while reducing energy and water usage and costs to demonstrate the efficiencies and opportunities available to large multi-function facilities. The scalable demand of these facilities required a layered approach to conservation, control and production. The project employed a comprehensive retrofit of electrical natural gas, and plumbing systems to maximize efficiency and lower carbon footprint specifically to demonstrate the effectiveness of these strategies in a public setting with varied and diverse use. The project was completed in July 2014 and met the expected outcomes regarding increased comfort and operational efficiency throughout the Pier 2 shed as well as on site electrical generation of current consumption. The entire Pier 2 shed project won a 2015 California Preservation Foundation design award for historic rehabilitation.

  19. Magnus Georg von Paucker (1787-1855) / Eckhard Spring

    Index Scriptorium Estoniae

    Spring, Eckhard

    2013-01-01

    Eestis sündinud ja Tartu Ülikoolis õppinud silmapaistvast baltisaksa teadlasest ja tema lapselapsest Alexandrine Pauckerist. 23. novembril 2012 Jelgavas/Mitaus toimunud Magnus Georg von Pauckerile pühendatud teaduskonverentsist

  20. Haldusreformist üldiselt / Georg Sootla, Kersten Kattai

    Index Scriptorium Estoniae

    Sootla, Georg, 1954-

    2016-01-01

    Haldusreformi ning Pärnu linna ja Are, Audru, Paikuse ja Tõstamaa valla ühinemise kohta jagavad selgitusi ühinemise konsultandid, Tallinna Ülikooli professor Georg Sootla ja lektor Kersten Kattai

  1. Serial MR imaging and 1H-MR spectroscopy in monozygotic twins with Tay-Sachs disease.

    Science.gov (United States)

    Imamura, A; Miyajima, H; Ito, R; Orii, K O

    2008-10-01

    Four-year-old monozygotic female twins with early onset Tay-Sachs disease are described. The sisters showed similar slowly progressive clinical symptoms and deterioration, however the younger sister also demonstrated intractable myoclonus in the right leg. The serial MR images and (1)H-MR spectroscopy of the brain were obtained in both twins. MR images showed high intensity on T (2)-weighted image in the bilateral white matter, however there were no signal changes in the basal ganglia and thalamus during any of the phases. The ratio of N-acetylaspartate (NAA)/creatine (Cr) was decreased in the both white matter lesions and the corpus striatum, and that of myoinositol (mI)/Cr was increased in the damaged white matter on MR spectroscopy. The elevation of the lactate peak was clearly demonstrated in the left basal ganglia of the younger sister; however it was not shown in cerebral lesions of the elder sister. Changes in metabolites on MR spectroscopy were closely linked to the respective clinical features of each twin. Follow-up examination by (1)H-MR spectroscopy is useful for the evaluation of neuronal changes in children with Tay-Sachs disease.

  2. Haematopoietic Stem Cell Transplantation Arrests the Progression of Neurodegenerative Disease in Late-Onset Tay-Sachs Disease.

    Science.gov (United States)

    Stepien, Karolina M; Lum, Su Han; Wraith, J Edmond; Hendriksz, Christian J; Church, Heather J; Priestman, David; Platt, Frances M; Jones, Simon; Jovanovic, Ana; Wynn, Robert

    2017-12-07

    Tay-Sachs disease is a rare metabolic disease caused by a deficiency of hexosaminidase A that leads to accumulation of GM2 gangliosides predominantly in neural tissue. Late-onset Tay-Sachs disease variant is associated with a higher level of residual HexA activity. Treatment options are limited, and there are a few described cases who have undergone haematopoietic stem cell transplantation (HSCT) with variable outcome.We describe a case of a 23-year-old male patient who presented with a long-standing tremor since 7 years of age. He had gait ataxia, a speech stammer and swallowing problems. His condition had had a static course apart from his tremor that had been gradually deteriorating. Because of the deterioration in his neurological function, the patient had an uneventful, matched-sibling donor bone marrow transplant at the age of 15 years. Eight years post-HSCT, at the age of 23, he retains full donor engraftment, and his white cell beta-HexA of 191 nmol/mg/h is comparable to normal controls (in-assay control = 187). He continues to experience some intentional tremor that is tolerable for daily life and nonprogressive since HSCT. HSCT is a potential treatment option which might arrest neurodegeneration in patients with LOTS.

  3. Crystal Structure of Human [Beta]-Hexosaminidase B: Understanding the Molecular Basis of Sandhoff and Tay-Sachs Disease

    Energy Technology Data Exchange (ETDEWEB)

    Mark, Brian L.; Mahuran, Don J.; Cherney, Maia M.; Zhao, Dalian; Knapp, Spencer; James, Michael N.G.

    2010-12-01

    In humans, two major {beta}-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a heterodimer of subunits {alpha} and {beta} (60% identity), whereas Hex B is a homodimer of {beta}-subunits. Interest in human {beta}-hexosaminidase stems from its association with Tay-Sachs and Sandhoff disease; these are prototypical lysosomal storage disorders resulting from the abnormal accumulation of G{sub M2}-ganglioside (G{sub M2}). Hex A degrades G{sub M2} by removing a terminal N-acetyl-D-galactosamine ({beta}-GalNAc) residue, and this activity requires the G{sub M2}-activator, a protein which solubilizes the ganglioside for presentation to Hex A. We present here the crystal structure of human Hex B, alone (2.4 {angstrom}) and in complex with the mechanistic inhibitors GalNAc-isofagomine (2.2 {angstrom}) or NAG-thiazoline (2.5 {angstrom}). From these, and the known X-ray structure of the G{sub M2}-activator, we have modeled Hex A in complex with the activator and ganglioside. Together, our crystallographic and modeling data demonstrate how {alpha} and {beta}-subunits dimerize to form either Hex A or Hex B, how these isoenzymes hydrolyze diverse substrates, and how many documented point mutations cause Sandhoff disease ({beta}-subunit mutations) and Tay-Sachs disease ({alpha}-subunit mutations).

  4. 76 FR 68625 - Establishment of the Fort Monroe National Monument

    Science.gov (United States)

    2011-11-07

    ... period of slavery in the colonies and, later, this Nation. Two hundred and forty-two years later, Fort... 1863. Thus, Old Point Comfort marks both the beginning and end of slavery in our Nation. The Fort... North Beach area lies the only undeveloped shoreline remaining on Old Point Comfort, providing modern...

  5. Microgrid Enabled Distributed Energy Solutions (MEDES) Fort Bliss Military Reservation

    Science.gov (United States)

    2014-02-01

    FINAL REPORT Microgrid Enabled Distributed Energy Solutions (MEDES) Fort Bliss Military Reservation ESTCP Project EW-201140 FEBRUARY...TITLE AND SUBTITLE Microgrid Enabled Distributed Energy Solutions (MEDES) 5a. CONTRACT NUMBER W912HQ-11-C-0082 Fort Bliss, Texas...Lockheed Martin’s Intelligent Microgrid Solution can provide more energy security while also lowering electric utility costs and greenhouse gas emissions

  6. Fort Valley's early scientists: A legacy of distinction

    Science.gov (United States)

    Andrew J. Sanchez Meador; Susan D. Olberding

    2008-01-01

    When the Riordan brothers of Flagstaff, Arizona, asked Gifford Pinchot to determine why there was a deficit in ponderosa pine seedlings, neither party understood the historical significance of what they were setting in motion for the field of forest research. The direct result of that professional favor was the establishment of the Fort Valley Experiment Station (Fort...

  7. Fort Valley's early scientists: A legacy of distinction (P-53)

    Science.gov (United States)

    Andrew J. Sanchez Meador; Susan D. Olberding

    2008-01-01

    When the Riordan brothers of Flagstaff, Arizona asked Gifford Pinchot to determine why there was a deficit in ponderosa pine seedlings, neither party understood the historical significance of what they were setting in motion for the field of forest research. The direct result of that professional favor was the establishment of the Fort Valley Experiment Station (Fort...

  8. Influence of defect size and localization on the engagement of reverse Hill-Sachs lesions.

    Science.gov (United States)

    Moroder, Philipp; Runer, Armin; Kraemer, Manuel; Fierlbeck, Johann; Niederberger, Alfred; Cotofana, Sebastian; Vasvari, Imre; Hettegger, Bernhard; Tauber, Mark; Hurschler, Christof; Resch, Herbert

    2015-03-01

    Reverse Hill-Sachs (RHS) lesions can cause recurrent posterior shoulder instability because of engagement with the posterior glenoid rim; however, the effect of defect size and localization have yet to be determined. Both size and localization are critical for the engagement of an RHS defect with the posterior glenoid rim. Controlled laboratory study. Ten RHS defects with predefined extent and localization were created through an anterolateral rotator cuff sparing approach in 10 fresh-frozen cadaveric shoulder specimens using a custom-made saw guide. Computed tomography scans of all specimens were completed, and standardized measurements were performed to determine the size (alpha angle) and localization (beta angle) of the defect as well as a combination of both parameters (gamma angle). Internal rotation motions were imposed on the shoulder joint in different arm positions and with varying amount of posterior translation by means of a robot-assisted shoulder simulator. The association between engagement of the defects and the defined parameters (alpha, beta, and gamma angles) was analyzed. In 0° of abduction, a cutoff value between engaging and nonengaging defects of 37.5° for the alpha angle (100% sensitivity; 75% specificity; area under the curve [AUC], 0.875; P = .055) and 36.5° for the beta angle (100% sensitivity; 25% specificity; AUC, 0.708; P = .286) was determined. The gamma angle showed the highest discriminatory power (AUC, 0.938; P = .025) with a cutoff value of 85.5° rendering 100% sensitivity and 75% specificity in the prediction of engagement. An increase in the applied posterior translation force decreased the degrees of internal rotation necessary before engagement occurred. No engagement occurred during internal rotation with the arm in 60° of abduction or 60° of flexion. The size and localization of RHS defects are both critical factors for engagement. The combination of both parameters in terms of the gamma angle measurement might be a

  9. Georges Besse 2. A new era for enrichment

    International Nuclear Information System (INIS)

    2009-01-01

    Since 1978, AREVA group subsidiary EURODIF's Georges Besse plant has been using gaseous diffusion to enrich uranium and meet the requirements of electricity utilities. Georges Besse II plant, which will use far less electricity, will replace George Besse not so long. The Georges Besse II plant is located on the Tricastin nuclear site in southern France. AREVA ensure delivery of uranium enrichment services in accordance with customer expectations. AREVA obtained the right to use URENCO's centrifugation technology on July 3, 2006. This is the process to be used at Georges Besse II. The new uranium enrichment plant will comprise two enrichment units, with a total production capacity of 7.5 million separative work units, which can be extended to 11 million if needed. Each enrichment unit will include: a Centrifuge Assembly Building (CAB), a Centrifuge Utility Building (CUB) with offices and control room, annexes for purification, supply and extraction of uranium hexafluoride (UF 6 ), modules containing the halls that house the centrifuge cascades. The modular design of the Georges Besse II plant will allow production in the first cascade where as the others cascades are build. The first cascade should be operational in the first half of 2009. At an overall cost of 3 billion euros, this project is one of the largest investments of the decade in France On November 24, 2003, AREVA and URENCO signed an agreement under which AREVA would buy 50% of the shares in the Enrichment Technology Company (ETC), which designs and manufactures centrifuges

  10. George Soros : sõda terroriga on kohutav viga / George Soros ; interv. Külli-Riin Tigasson

    Index Scriptorium Estoniae

    Soros, George, 1930-

    2006-01-01

    Ilmunud ka: Infopress 15. dets. nr. 50 lk. 149. Miljardär ja filantroop George Soros vastab küsimustele, mis puudutavad tema raamatus "Ekslikkuse ajastu. Sõda terroriga ja selle tagajärjed" esitatud seisukohti. Ta leiab, et sõjakuulutamine terrorismile oli vale reaktsioon 11. septembri rünnakutele. George W. Bushi kursi muutumine Iraagiga seoses on tulnud liiga hilja. USA peab naasma oma traditsioonilise rolli juurde maailmas ja tegelema inimkonna suurimate probleemidega

  11. Multimedia messages in genetics: design, development, and evaluation of a computer-based instructional resource for secondary school students in a Tay Sachs disease carrier screening program.

    Science.gov (United States)

    Gason, Alexandra A; Aitken, MaryAnne; Delatycki, Martin B; Sheffield, Edith; Metcalfe, Sylvia A

    2004-01-01

    Tay Sachs disease is a recessively inherited neurodegenerative disorder, for which carrier screening programs exist worldwide. Education for those offered a screening test is essential in facilitating informed decision-making. In Melbourne, Australia, we have designed, developed, and evaluated a computer-based instructional resource for use in the Tay Sachs disease carrier screening program for secondary school students attending Jewish schools. The resource entitled "Genetics in the Community: Tay Sachs disease" was designed on a platform of educational learning theory. The development of the resource included formative evaluation using qualitative data analysis supported by descriptive quantitative data. The final resource was evaluated within the screening program and compared with the standard oral presentation using a questionnaire. Knowledge outcomes were measured both before and after either of the educational formats. Data from the formative evaluation were used to refine the content and functionality of the final resource. The questionnaire evaluation of 302 students over two years showed the multimedia resource to be equally effective as an oral educational presentation in facilitating participants' knowledge construction. The resource offers a large number of potential benefits, which are not limited to the Tay Sachs disease carrier screening program setting, such as delivery of a consistent educational message, short delivery time, and minimum financial and resource commitment. This article outlines the value of considering educational theory and describes the process of multimedia development providing a framework that may be of value when designing genetics multimedia resources in general.

  12. The Fort Smith radioactive belt, Northwest Territories

    International Nuclear Information System (INIS)

    Charbonneau, B.W.

    1980-01-01

    The Fort Smith Belt is an elongate zone, about 200 km x 50 km, extending from the East Arm of Great Slave Lake southerly into northeastern Alberta. The major feature of the belt is that it is one of the most radioactive regions so far recognized in the Canadian Shield. Potassium, uranium, and thorium are all enriched but the greatest increase is in thorium. The dominant rock type underlying the area is a foliated porphyritic granite. This rock contains an average of about 80 ppm thorium (with areas of tens of square kilometres containing up to 200 ppm) and approximately 11 ppm uranium. In places, dark elongate zones rich in biotite, apatite, and opaque minerals within the porphyritic granite may contain an order of magnitude more uranium and thorium than the porphyry. Radioactive minerals within both the porphyry and the dark zones are principally monazite (containing up to 16% ThO 2 ) and isolated grains of uraninite. This foliated porphyritic granite is interpreted as being pre- or syntectonic with respect to the Hudsonian event because its foliation parallels that of the surrounding rocks. There has been subsequent deformation. The second characteristic feature of the Fort Smith Belt is the development of a peripheral zone where eU is enriched relative to eTh correlating mainly with granitoid rocks which surround the thorium-rich area and wherein ratios of eU/eTh exceed 1:2 (compared to the crustal average of 1:4). Uranium may have moved laterally into this marginal area from the thorium-rich porphyry, possibly in a vapour phase. There is a possibility that concentrations of uranium as well as other metals such as Cu, Mo, Zn, Sn, and W could exist in the porphyry and its margin in appropriate chemical and/or structural traps. The radioactive granite rocks of the Fort Smith Belt are adjacent to uranium-thorium occurrences in the nearby Proterozoic Nonacho sediments but whether or not a genetic relationship exists between the two situations is uncertain. (auth)

  13. Gynecologic Malignancies Post-LeFort Colpocleisis

    Directory of Open Access Journals (Sweden)

    Rayan Elkattah

    2014-01-01

    Full Text Available Introduction. LeFort colpocleisis (LFC is a safe and effective obliterative surgical option for older women with advanced pelvic organ prolapse who no longer desire coital activity. A major disadvantage is the limited ability to evaluate for post-LFC gynecologic malignancies. Methods. We present the first case of endometrioid ovarian cancer diagnosed after LFC and review all reported gynecologic malignancies post-LFC in the English medical literature. Results. This is the second reported ovarian cancer post-LFC and the first of the endometrioid subtype. A total of nine other gynecologic malignancies post-LFC have been reported in the English medical literature. Conclusions. Gynecologic malignancies post-LFC are rare. We propose a simple 3-step strategy in evaluating post-LFC malignancies.

  14. Master environmental plan for Fort Devens, Massachusetts

    Energy Technology Data Exchange (ETDEWEB)

    Biang, C.A.; Peters, R.W.; Pearl, R.H.; Tsai, S.Y. (Argonne National Lab., IL (United States). Energy Systems Div.)

    1991-11-01

    Argonne National Laboratory has prepared a master environmental plan (MEP) for Fort Devens, Massachusetts, for the US Army Toxic and Hazardous Materials Agency. The MEP is an assessment based on environmental laws and regulations of both the federal government and the Commonwealth of Massachusetts. The MEP assess the physical and environmental status of 58 potential hazardous waste sites, including 54 study areas (SAs) that pose a potential for releasing contamination into the environment and 4 areas of concern (AOCs) that are known to have substantial contamination. For each SA or AOC, this MEP describes the known history and environment, identifies additional data needs, and proposes possible response actions. Most recommended response actions consist of environmental sampling and monitoring and other characterization studies. 74 refs., 63 figs., 50 tabs.

  15. Fort St. Vrain defueling ampersand decommissioning considerations

    International Nuclear Information System (INIS)

    Warembourg, D.

    1994-01-01

    Fort St. Vrain Nuclear Generating Station (FSV) is one of the first commercial reactors to be decommissioned under NRC's decommissioning rule. The defueling and decommissioning of this 330 MWe High Temperature Gas Cooled Reactor (HTGR) has involved many challenges for Public Service Company of Colorado (PSC) including defueling to an Independent Spent Fuel Storage Installation (ISFSI), establishing decommissioning funding, obtaining regulatory approvals, arranging for waste disposal, and managing a large fixed price decommissioning contract. In 1990, a team comprised of the Westinghouse Corporation and Morrison Knudsen Corporation, with the Scientific Ecology Group as a major subcontractor, was contracted by PSC to perform the decommissioning under a fixed price contract. Physical work activities began in August 1992. Currently, physical dismantlement activities are about 45% complete, the project is on schedule, and is within budget

  16. Reflector dowel strength test, Fort St. Vrain

    International Nuclear Information System (INIS)

    Doll, D.W.

    1975-01-01

    The strength of the 44.45 mm (1.75 in.) diameter Fort St. Vrain (FSV) reflector dowel for loads directed radially inward toward the center of the element was measured. For a statically applied load, the strength exceeded 5783 N (1300 lb) in direct shear. This strength remained after load cycling 100 times to 4448 N (1000 lb), 10 times to 4893 N (1100 lb), 10 times to 5338 N (1200 lb), and two times to 5783 N (1300 lb). Typically, the deflection to ultimate failure was approximately 1.0 mm (0.04 in.). At about 3316 N (750 lb) and 0.20 mm (0.008 in.) deflection, one of the webs between the dowel and a coolant hole cracked, apparently redistributing the load. No further failure occurred up to the ultimate load of 5783+ N (1300+ lb)

  17. Fort Collins Science Center Ecosystem Dynamics Branch

    Science.gov (United States)

    Wilson, Jim; Melcher, C.; Bowen, Z.

    2009-01-01

    Complex natural resource issues require understanding a web of interactions among ecosystem components that are (1) interdisciplinary, encompassing physical, chemical, and biological processes; (2) spatially complex, involving movements of animals, water, and airborne materials across a range of landscapes and jurisdictions; and (3) temporally complex, occurring over days, weeks, or years, sometimes involving response lags to alteration or exhibiting large natural variation. Scientists in the Ecosystem Dynamics Branch of the U.S. Geological Survey, Fort Collins Science Center, investigate a diversity of these complex natural resource questions at the landscape and systems levels. This Fact Sheet describes the work of the Ecosystems Dynamics Branch, which is focused on energy and land use, climate change and long-term integrated assessments, herbivore-ecosystem interactions, fire and post-fire restoration, and environmental flows and river restoration.

  18. Operational testing highlights of Fort St. Vrain

    International Nuclear Information System (INIS)

    Cadwell, J.J.; McEachern, D.W.; Read, J.W.; Simon, W.A.; Walker, R.F.

    1975-01-01

    The Fort St. Vrain program has progressed through construction, preoperational testing, fuel loading, initial criticality, and operational testing at power levels up to 2 percent related power. To date, all tests necessary before the rise to full power have been completed, and the rise-to-power program is expected to be resumed again in late 1975. Major plant systems, including the prestressed concrete reactor vessel and circulators, have demonstrated adequate performance. Extensive tests on the reactor core at zero power and up to 2 percent power have demonstrated the accuracy in the design predictions of such core characteristics as critical rod position, control system worths, neutron flux distributions, and temperature coefficients. Gaseous fission product release measurements to date have confirmed the extensive analytical estimates. 6 references

  19. Late onset Tay-Sachs disease in mice with targeted disruption of the Hexa gene: behavioral changes and pathology of the central nervous system.

    Science.gov (United States)

    Miklyaeva, Elena I; Dong, Weijia; Bureau, Alexandre; Fattahie, Roya; Xu, Yongqin; Su, Meng; Fick, Gordon H; Huang, Jing-Qi; Igdoura, Suleiman; Hanai, Nobuo; Gravel, Roy A

    2004-03-19

    Tay-Sachs disease is an autosomal recessive neurodegenerative disease resulting from a block in the hydrolysis of GM2 ganglioside, an intermediate in ganglioside catabolism. The mouse model of Tay-Sachs disease (Hexa -/-) has been described as behaviorally indistinguishable from wild type until at least 1 year of age due to a sialidase-mediated bypass of the metabolic defect that reduces the rate of GM2 ganglioside accumulation. In this study, we have followed our mouse model to over 2 years of age and have documented a significant disease phenotype that is reminiscent of the late onset, chronic form of human Tay-Sachs disease. Onset occurs at 11-12 months of age and progresses slowly, in parallel with increasing storage of GM2 ganglioside. The disease is characterized by hind limb spasticity, weight loss, tremors, abnormal posture with lordosis, possible visual impairment, and, late in the disease, muscle weakness, clasping of the limbs, and myoclonic twitches of the head. Immunodetection of GM2 ganglioside showed that storage varies widely in different regions, but is most intense in pyriform cortex, hippocampus (CA3 field, subiculum), amygdala, hypothalamus (paraventricular supraoptic, ventromedial and arcuate nuclei, and mammilary body), and the somatosensory cortex (layer V) in 1- to 2-year-old mutant mice. We suggest that the Tay-Sachs mouse model is a phenotypically valid model of disease and may provide for a reliable indicator of the impact of therapeutic strategies, in particular geared to the late onset, chronic form of human Tay-Sachs disease.

  20. Splice junction mutation in some Ashkenazi Jews with Tay-Sachs disease: Evidence against a single defect within this ethnic group

    Energy Technology Data Exchange (ETDEWEB)

    Myerowitz, R. (National Institute of Diabetes, Digestive and Kidney Diseases, Bethesda, MD (USA))

    1988-06-01

    Tay-Sachs disease is an inherited disorder in which the {alpha} chain of the lysosomal enzyme {beta}-N-acetylhexosaminidase A bears the mutation. Ashkenazi Jews are found to be carriers for a severe type of Tay-Sachs disease, the classic form, 10 times more frequently than the general population. Ashkenazi Jewish patients with classic Tay-Sachs disease have appeared to be clinically and biochemically identical, and the usual assumption has been that they harbor the same {alpha}-chain mutation. The author has isolated the {alpha}-chain gene from an Ashkenazi Jewish patient, GM2968, with classic Tay-Sachs disease and compared its nucleotide sequences with that of the normal {alpha}-chain gene in the promoter region, exon and splice junction regions, and polyadenylylation signal area. Only one difference was observed between these sequences. The alteration is presumed to be functionally significant and to result in aberrant mRNA splicing. Utilizing the polymerase chain reaction to amplify the region encompassing the mutation, the author developed an assay to screen patients and heterozygote carriers for this mutation. Surprisingly, in each of two Ashkenazi patients, only one {alpha}-chain allele harbored the splice junction mutation. Only one parent of each of these patients was positive for the defect. Another Ashkenazi patient did not bear this mutation at all nor did either of the subject's parents. The data are consistent with the presence of more than one mutation underlying the classic form of Tay-Sachs disease in the Ashkenazi Jewish population.

  1. Splice junction mutation in some Ashkenazi Jews with Tay-Sachs disease: Evidence against a single defect within this ethnic group

    International Nuclear Information System (INIS)

    Myerowitz, R.

    1988-01-01

    Tay-Sachs disease is an inherited disorder in which the α chain of the lysosomal enzyme β-N-acetylhexosaminidase A bears the mutation. Ashkenazi Jews are found to be carriers for a severe type of Tay-Sachs disease, the classic form, 10 times more frequently than the general population. Ashkenazi Jewish patients with classic Tay-Sachs disease have appeared to be clinically and biochemically identical, and the usual assumption has been that they harbor the same α-chain mutation. The author has isolated the α-chain gene from an Ashkenazi Jewish patient, GM2968, with classic Tay-Sachs disease and compared its nucleotide sequences with that of the normal α-chain gene in the promoter region, exon and splice junction regions, and polyadenylylation signal area. Only one difference was observed between these sequences. The alteration is presumed to be functionally significant and to result in aberrant mRNA splicing. Utilizing the polymerase chain reaction to amplify the region encompassing the mutation, the author developed an assay to screen patients and heterozygote carriers for this mutation. Surprisingly, in each of two Ashkenazi patients, only one α-chain allele harbored the splice junction mutation. Only one parent of each of these patients was positive for the defect. Another Ashkenazi patient did not bear this mutation at all nor did either of the subject's parents. The data are consistent with the presence of more than one mutation underlying the classic form of Tay-Sachs disease in the Ashkenazi Jewish population

  2. Impact of gene patents and licensing practices on access to genetic testing and carrier screening for Tay-Sachs and Canavan disease.

    Science.gov (United States)

    Colaianni, Alessandra; Chandrasekharan, Subhashini; Cook-Deegan, Robert

    2010-04-01

    Genetic testing for Tay-Sachs and Canavan disease is particularly important for Ashkenazi Jews, because both conditions are more frequent in that population. This comparative case study was possible because of different patenting and licensing practices. The role of DNA testing differs between Tay-Sachs and Canavan diseases. The first-line screening test for Tay-Sachs remains an enzyme activity test rather than genotyping. Genotyping is used for preimplantation diagnosis and confirmatory testing. In contrast, DNA-based testing is the basis for Canavan screening and diagnosis. The HEXA gene for Tay-Sachs was cloned at the National Institutes of Health, and the gene was patented but has not been licensed. The ASPA gene for Canavan disease was cloned and patented by Miami Children's Hospital. Miami Children's Hospital did not inform family members and patient groups that had contributed to the gene discovery that it was applying for a patent, and pursued restrictive licensing practices when a patent issued in 1997. This led to intense controversy, litigation, and a sealed, nonpublic 2003 settlement that apparently allowed for nonexclusive licensing. A survey of laboratories revealed a possible price premium for ASPA testing, with per-unit costs higher than for other genetic tests in the Secretary's Advisory Committee on Genetics, Health, and Society case studies. The main conclusion from comparing genetic testing for Tay-Sachs and Canavan diseases, however, is that patenting and licensing conducted without communication with patients and advocates cause mistrust and can lead to controversy and litigation, a negative model to contrast with the positive model of patenting and licensing for genetic testing of cystic fibrosis.

  3. Operational experience at Fort St. Vrain

    International Nuclear Information System (INIS)

    Bramblett, G.C.; Fisher, C.R.; Swart, F.E.

    1981-01-01

    The Fort St. Vrain (FSV) station, a 330-MW(e) single reheat steam cycle powered by a high-temperature gas-cooled reactor (HTGR), is the first HTGR to enter commercial operation. Designed and built by General Atomic Company (GA), the plant is owned and operated by Public Service Company of Colorado (PSC). Many unique design features have been incorporated into this reactor system, including high-pressure helium as the primary system coolant, a graphite-moderated prismatic block core design, fission-product-containing carbide coatings on both fissile and fertile fuel particles, steam-driven helium circulators turning on water bearings, and once-through steam generators. All of these systems are contained in a prestressed concrete reactor vessel (PCRV). Extensive testing has been conducted during the rise to power following first criticality early in 1974 to verify system design performance. During this period, the plant has operated at power levels up to 70% and produced over one billion kilowatt hours of electricity. In 1979, the first refueling was conducted in conjunction with an extensive in-core inspection, the addition of in-core instrumentation, and a planned removal of a circulator for inspection. Later in the year, a scheduled shutdown was undertaken for surveillance tests, insertion of core region constraint devices (RCDs), and other maintenance. Fort St. Vrain has encountered problems of the type that would be expected in a first-of-a-kind system. The plant is currently restricted to 70% of design power by the Nuclear Regulatory Commission (NRC) pending resolution of the core region gas outlet temperature fluctuation problem. Even so, the basic performance of the HTGR concept and all of the unique design features have been successfully demonstrated. The system has been characterized by low personnel radiation exposures, operational flexibility, and long time afforded for status evaluation and response. (author)

  4. Moving towards first science with the St. George recoil separator

    Science.gov (United States)

    Meisel, Zachary; Berg, G. P. A.; Gilardy, G.; Moran, M.; Schmitt, J.; Seymour, C.; Stech, E.; Couder, M.

    2015-10-01

    The St. George recoil mass separator has recently been coupled to the 5MV St. Ana accelerator at the University of Notre Dame's Nuclear Science Lab. St. George is a unique tool designed to measure radiative alpha-capture reactions for nuclei up to A = 40 in inverse kinematics in order to directly obtain cross sections required for astrophysical models of stellar and explosive helium burning. Commissioning of St. George is presently taking place with primary beams of hydrogen, helium, and oxygen. In this presentation, results will be shown for the measured energy acceptance of St. George, which compare favorably to COSY results when employing the calculated optimal ion-optical settings. Additionally, future plans will be discussed, such as assessing the angular acceptance of St. George and the re-integration of HiPPO at the separator target position to provide a dense, windowless helium gas-jet target. The material presented in this work is partially supported by the National Science Foundation Grant No. 1419765.

  5. Georges Bank: a leaky incubator of Alexandrium fundyense blooms.

    Science.gov (United States)

    McGillicuddy, D J; Townsend, D W; Keafer, B A; Thomas, M A; Anderson, D M

    2014-05-01

    A series of oceanographic surveys on Georges Bank document variability of populations of the toxic dinoflagellate Alexandrium fundyense on time scales ranging from synoptic to seasonal to interannual. Blooms of A. fundyense on Georges Bank can reach concentrations on the order of 10 4 cells l -1 , and are generally bank-wide in extent. Georges Bank populations of A. fundyense appear to be quasi-independent of those in the adjacent coastal Gulf of Maine, insofar as they occupy a hydrographic niche that is colder and saltier than their coastal counterparts. In contrast to coastal populations that rely on abundant resting cysts for bloom initiation, very few cysts are present in the sediments on Georges Bank. Bloom dynamics must therefore be largely controlled by the balance between growth and mortality processes, which are at present largely unknown for this population. Based on correlations between cell abundance and nutrient distributions, ammonium appears to be an important source of nitrogen for A. fundyense blooms on Georges Bank.

  6. General of the Army George C. Marshall on Operational Leadership

    Science.gov (United States)

    2007-11-06

    145 and he actively pursued it as an end itself, even personally getting involved in the distribution of chocolate and cigarettes.146 When a...Marshall to Mrs. Thomas B. Coles, 26 October 1927, Ibid., 316. Marshall was happy to move and looking forward to the new duty. 51 Lecture, Fort

  7. Generation of HEXA-deficient hiPSCs from fibroblasts of a Tay-Sachs disease patient.

    Science.gov (United States)

    Liu, Zhong; Zhao, Rui

    2016-09-01

    Human iPSC line TSD-01-hiPSC was generated from fibroblasts of a patient with infantile Tay-Sachs disease (TSD). The patient is compound heterozygous at the HEXA gene by carrying a 1278insTATC allele and an IVS12+1G>C allele. STEMCCA lentivirus, which expresses OCT4, SOX2, KLF4, and c-MYC from a polycistronic transcript, were used for reprogramming. TSD-01-hiPSC express pluripotency markers such as OCT4, SOX2, NANOG, Tra-1-60, and alkaline phosphatase, and can differentiate into tissues from all the three embryonic germ layers. This TSD patient-derived hiPSC line may serve as a valuable in vitro tool for disease modeling and drug test. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  8. Generation of HEXA-deficient hiPSCs from fibroblasts of a Tay-Sachs disease patient

    Directory of Open Access Journals (Sweden)

    Zhong Liu

    2016-09-01

    Full Text Available Human iPSC line TSD-01-hiPSC was generated from fibroblasts of a patient with infantile Tay-Sachs disease (TSD. The patient is compound heterozygous at the HEXA gene by carrying a 1278insTATC allele and an IVS12+1G>C allele. STEMCCA lentivirus, which expresses OCT4, SOX2, KLF4, and c-MYC from a polycistronic transcript, were used for reprogramming. TSD-01-hiPSC express pluripotency markers such as OCT4, SOX2, NANOG, Tra-1-60, and alkaline phosphatase, and can differentiate into tissues from all the three embryonic germ layers. This TSD patient-derived hiPSC line may serve as a valuable in vitro tool for disease modeling and drug test.

  9. Time-dependent automorphism-inducing diffeomorphisms, open algebras and the generality of the Kantowski-Sachs vacuum geometry

    Science.gov (United States)

    Christodoulakis, T.; Papadopoulos, G. O.

    2002-10-01

    Following the spirit of a previous work of ours, we investigate the group of those general coordinate transformations (GCTs) which preserve manifest spatial homogeneity. In contrast to the case of Bianchi type models, here we permit an isometry group of motions G4 = SO(3) ⊗ Tr, where Tr is the translations group, along the radial direction, while SO(3) acts multiply transitively on each hypersurface of simultaneity Σt. The basis 1-forms cannot be invariant under the action of the entire isometry group and hence produce an open Lie algebra. In order for these GCTs to exist and have a nontrivial, well-defined action, certain integrability conditions have to be satisfied; their solutions, exhibiting the maximum expected 'gauge' freedom, can be used to simplify the generic, spatially homogeneous, line element. In this way an alternative proof of the generality of the Kantowski-Sachs (KS) vacuum is given, while its most general, manifestly homogeneous, form is explicitly presented.

  10. George Mikenberg honoured by the Sharing Knowledge Foundation

    CERN Multimedia

    CERN Bulletin

    2016-01-01

    The Sharing Knowledge Foundation Prize was presented to the ATLAS physicist at a ceremony on 18 March.   ATLAS physicist George Mikenberg receives the Sharing Knowledge Foundation Prize. From left to right: Peter Jenni, former ATLAS Spokesperson, Rolf Heuer, former CERN Director-General, Frédérick Bordry, CERN Director for Accelerators and Technology, George Mikenberg, Patrick Fassnacht, CERN International Relations sector, Eliane Jacot-Des-Combes Mikenberg, Herwig Schopper, former CERN Director-General, Constantin Lanaras, treasurer of the Sharing Knowledge Foundation, Robert Klapisch, President of the Sharing Knowledge Foundation, Federico Saldana, logistical support, Catherine Cesarsky, Vice-President of the CERN Council, Eliezer Rabinovici, Vice-President of the CERN Council and of SESAME. (Image: Sophia Bennett/CERN) The Sharing Knowledge Foundation awarded its 2016 prize to George Mikenberg, a member of the LHC's ATLAS experiment who has been...

  11. Murine Sialidase Neu3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease.

    Science.gov (United States)

    Seyrantepe, Volkan; Demir, Secil Akyildiz; Timur, Zehra Kevser; Von Gerichten, Johanna; Marsching, Christian; Erdemli, Esra; Oztas, Emin; Takahashi, Kohta; Yamaguchi, Kazunori; Ates, Nurselin; Dönmez Demir, Buket; Dalkara, Turgay; Erich, Katrin; Hopf, Carsten; Sandhoff, Roger; Miyagi, Taeko

    2018-01-01

    Tay-Sachs disease is a severe lysosomal storage disorder caused by mutations in Hexa, the gene that encodes for the α subunit of lysosomal β-hexosaminidase A (HEXA), which converts GM2 to GM3 ganglioside. Unexpectedly, Hexa -/- mice have a normal lifespan and show no obvious neurological impairment until at least one year of age. These mice catabolize stored GM2 ganglioside using sialidase(s) to remove sialic acid and form the glycolipid GA2, which is further processed by β-hexosaminidase B. Therefore, the presence of the sialidase (s) allows the consequences of the Hexa defect to be bypassed. To determine if the sialidase NEU3 contributes to GM2 ganglioside degradation, we generated a mouse model with combined deficiencies of HEXA and NEU3. The Hexa -/- Neu3 -/- mice were healthy at birth, but died at 1.5 to 4.5months of age. Thin-layer chromatography and mass spectrometric analysis of the brains of Hexa -/- Neu3 -/- mice revealed the abnormal accumulation of GM2 ganglioside. Histological and immunohistochemical analysis demonstrated cytoplasmic vacuolation in the neurons. Electron microscopic examination of the brain, kidneys and testes revealed pleomorphic inclusions of many small vesicles and complex lamellar structures. The Hexa -/- Neu3 -/- mice exhibited progressive neurodegeneration with neuronal loss, Purkinje cell depletion, and astrogliosis. Slow movement, ataxia, and tremors were the prominent neurological abnormalities observed in these mice. Furthermore, radiographs revealed abnormalities in the skeletal bones of the Hexa -/- Neu3 -/- mice. Thus, the Hexa -/- Neu3 -/- mice mimic the neuropathological and clinical abnormalities of the classical early-onset Tay-Sachs patients, and provide a suitable model for the future pre-clinical testing of potential treatments for this condition. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Structural remains at the early mediaeval fort at Raibania, Orissa

    Directory of Open Access Journals (Sweden)

    Bratati Sen

    2013-11-01

    Full Text Available The fortifications of mediaeval India occupy an eminent position in the history of military architecture. The present paper deals with the preliminary study of the structural remains at the early mediaeval fort at Raibania in the district of Balasore in Orissa. The fort was built of stone very loosely kept together. The three-walled fortification interspersed by two consecutive moats, a feature evidenced at Raibania, which is unparallel in the history of ancient and mediaeval forts and fortifications in India. Several other structures like the Jay-Chandi Temple Complex, a huge well, numerous tanks and remains of an ancient bridge add to the uniqueness of the Fort in the entire eastern region.

  13. Fortælling og fortolkning i Jyske Bank

    DEFF Research Database (Denmark)

    Albrechtsen, Charlotte

    Afhandlingen præsenterer en undersøgelse af et konkret eksempel på storytelling brugt som strategisk ledelses- og kommunikationsredskab i en organisations interne kommunikation. Eksemplet er fortællingen "Slaget ved Vejle", som stammer fra Jyske Bank og udgør under afhandlingens case. De overordn......Afhandlingen præsenterer en undersøgelse af et konkret eksempel på storytelling brugt som strategisk ledelses- og kommunikationsredskab i en organisations interne kommunikation. Eksemplet er fortællingen "Slaget ved Vejle", som stammer fra Jyske Bank og udgør under afhandlingens case. De......, at medarbejderne forholder sig reflekteret, nuanceret og kritisk til den strategiske fortælling, og at der er stor diversitet i deres oplevelser, fortolkninger og vurderinger af fortællingen. Desuden ser afhandlingen nærmere på hvad begrebet "storytelling" dækker over, og hvordan der hidtil er forsket i...

  14. Strategic Analysis and Plan for Implementing Telemedicine at Fort Greely

    National Research Council Canada - National Science Library

    Bolton, Karl

    2003-01-01

    .... To best accomplish this, a strategic analysis and business case analysis was conducted. Introspective strategic analysis tools revealed an organization that is capable of supporting a telemedicine program at Fort Greely...

  15. RadNet Air Data From Fort Smith, AR

    Science.gov (United States)

    This page presents radiation air monitoring and air filter analysis data for Fort Smith, AR from EPA's RadNet system. RadNet is a nationwide network of monitoring stations that measure radiation in air, drinking water and precipitation.

  16. Wood-Fired Boiler System Evaluation at Fort Stewart, GA

    National Research Council Canada - National Science Library

    Potts, Noel

    2002-01-01

    Part of the plan to modernize the central energy plant (CEP) at Fort Stewart, GA is focused on the installations wood-fired boiler, which provides steam for heating, cooling, and domestic hot water. The U.S...

  17. George Sand: últimas novelas, últimos ensueños...

    Directory of Open Access Journals (Sweden)

    Àngels Santa

    2014-04-01

    Full Text Available A propósito de la obra de George Sand, Œuvres complètes.Sous la direction de Béatrice Didier. 1870, I :Malgrétout. Édition critique par Dominique Laporte (París, Honoré Champion, 2013, 247 p. ISBN : 9782745322968 y de George Sand, Œuvres complètes.Sous la direction de Béatrice Didier. 1876 :La Tour de Percemont. Édition critique par Dominique Laporte (París, Honoré Champion, 2013, 247 p. ISBN : 9782745322951.

  18. Bulgaria and Serbia in the historical works of George Akropolites and George Pachymeres

    Directory of Open Access Journals (Sweden)

    Stanković Vlada

    2009-01-01

    Full Text Available The paper analyzes the well-known historical works of George Akropolites and George Pachymeres from the viewpoint of their literary composition structure, lexica and narrative techniques used for describing events and personalities from Bulgaria and Serbia. An attempt is made to surpass the 'traditional' methodology and the usually applied standard approach that focuses almost exclusively to the search for the 'strong' historical facts in these literary works par excellence, in that way overlooking their strong literary character, careful expressions, sometimes evident and sometimes more subtle style differences whose purpose was to underline the focal points and the conclusions of the content of the stories - in short, the information and the stories about Bulgaria and Serbia by Akropolites and Pachymeres are analyzed in their entirety, viewed as the integral parts of the complex literary works, and not as isolated episodes, divided from the rest of the narrative. The ways in which George Akropolites depicted and described deaths of the Bulgarian rulers were studied as one of the best examples of his conscious endeavors to bring the literary style of his History in accord with his own judgments about the rulers of the neighboring country. Scenes of death, conspicuously frequent in Akropolites' relatively short historical work, play an important role in characterizing personalities, and the same principle which Akropolites uses for judging the Byzantines is applied to the Bulgarians as well: generally positive opinion about somebody's life and achievements is emphasized with a depiction of his death in a positive way (Ivan II Asen, and vice versa, Akropolites' 'villains' receive their horrible deaths as a deserved punishment for their bad deeds (Kalojan's death. On the other hand, Akropolites' scarce information about Serbs is supplemented with his depiction of the Serbian king Uroš (1243-1276, and his stance after the death of John Batatzes

  19. Le Fort I Maxillary Advancement Using Distraction Osteogenesis

    OpenAIRE

    Combs, Patrick D.; Harshbarger, Raymond J.

    2014-01-01

    Treatment of maxillary hypoplasia has traditionally involved conventional Le Fort I osteotomies and advancement. Advancements of greater than 10 mm risk significant relapse. This risk is greater in the cleft lip and palate population, whose anatomy and soft tissue scarring from prior procedures contributes to instability of conventional maxillary advancement. Le Fort I advancement with distraction osteogenesis has emerged as viable, stable treatment modality correction of severe maxillary hyp...

  20. Structural remains at the early mediaeval fort at Raibania, Orissa

    OpenAIRE

    Sen, Bratati

    2013-01-01

    The fortifications of mediaeval India occupy an eminent position in the history of military architecture. The present paper deals with the preliminary study of the structural remains at the early mediaeval fort at Raibania in the district of Balasore in Orissa. The fort was built of stone very loosely kept together. The three-walled fortification interspersed by two consecutive moats, a feature evidenced at Raibania, w...

  1. Undervisning mellem fortælling og feedback

    DEFF Research Database (Denmark)

    Andersen, Kirsten Margrethe

    2016-01-01

    Feedback gør det muligt for den enkelte at forstå, hvordan jeg kan blive bedre til det, jeg er ved at lære. Fortællinger gør det muligt for den enkelte at udvide horisonten og derved komme til en forståelse af, hvilke mulige perspektiver der er for at forholde sig til den verden, som fortællingen...

  2. BRAND EQUITY OF LAHORE FORT AS A TOURISM DESTINATION BRAND

    OpenAIRE

    KASHIF, MUHAMMAD; SAMSI, SITI ZAKIAH MELATU; SARIFUDDIN, SYAMSULANG

    2015-01-01

    ABSTRACTStudies that measure the brand equity of destination brands by using the Customer-Based Brand Equity (CBBE) model in a developing country context are scarce. The present study investigates the destination brand equity of the Lahore Fort by employing the CBBE model in a developing country context of Pakistan. Following the positivist tradition, we adopted a survey-based approach to collect data from 237 tourists visiting the Lahore Fort. Data were collected through a questionnaire deve...

  3. FOUR YEARS OF OPERATIONS AND RESULTS WITH FORTE

    International Nuclear Information System (INIS)

    D. ROUSSEL-DUPRE; P. KLINGNER; L. CARLSON; ET AL

    2001-01-01

    The FORTE (Fast Onboard Recording of Transient Events) satellite was launched on 29 August 1997 and has been in continuous operation since that time. FORTE was placed in a nearly circular, 825-km-altitude, 70 degrees inclination orbit by a Pegasus rocket funded by Air Force Space Test Program. The Department of Energy funded the FORTE satellite, which was designed and built at Los Alamos. FORTE's successful launch and engineered robustness were a result of several years of dedicated work by the joint Los Alamos National Laboratory/Sandia National Laboratory project team, led through mission definition, payload and satellite development, and launch by Dr. Stephen Knox. The project is now led by Dr. Abram Jacobson. FORTE carries a suite of instruments, an optical system and a rf system, for the study of lightning and anthropogenic signals. As a result of this effort, new understandings of lightning events have emerged as well as a more complete understanding of the relationship between optical and rf lightning events. This paper will provide an overview of the FORTE satellite and will discuss the on orbit performance of the subsystems

  4. Cancer incidence in Fort Chipewyan, Alberta : 1995-2006

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Y. [Alberta Cancer Board, Edmonton, AB (Canada). Division of Population Health and Information Surveillance

    2009-02-15

    A high number of cases of cholangiocarcinoma, a rare form of bile duct cancer, as well as high rates of other cancers were reported by a physician working in Fort Chipewyan, Alberta in 2006. Concerns were raised by local residents, attributing cancers in their community to environmental contamination from a range of industrial development including the oil sands development, uranium mining and pulp mills. However, an initial review of the Alberta Cancer Registry did not confirm an increased incidence of cancer in Fort Chipewyan. In the summer/fall of 2007, a working group was formed to support the Alberta Cancer Board in doing a cluster investigation based on the guidelines of the United States Centre for Disease Control and Prevention. This report presented an investigation to determine if there was an elevated rate of cholangiocarcinoma in Fort Chipewyan and whether there was an elevated rate of cancers overall in Fort Chipewyan. The report provided background information on the Athabasca oil sands, uranium mining, and Fort Chipewyan as well as previous investigations of cancer incidence in Fort Chipewyan. Study methods were also presented with particular reference to study and comparison populations; cancer classification and inclusion criteria; active case ascertainment and verification; methods of analysis; and ethical approval. Results were also presented. The specific cancers that were discussed were cholangiocarcinoma, leukemia, colon cancer, and cancer in First Nations in Alberta. It was concluded that the observed number of cases of cholangiocarcinoma was within the expected range. 121 refs., 12 tabs., 3 figs., 5 appendices.

  5. Fort Collins Science Center fiscal year 2010 science accomplishments

    Science.gov (United States)

    Wilson, Juliette T.

    2011-01-01

    The scientists and technical professionals at the U.S. Geological Survey (USGS), Fort Collins Science Center (FORT), apply their diverse ecological, socioeconomic, and technological expertise to investigate complicated ecological problems confronting managers of the Nation's biological resources. FORT works closely with U.S. Department of the Interior (DOI) agency scientists, the academic community, other USGS science centers, and many other partners to provide critical information needed to help answer complex natural-resource management questions. In Fiscal Year 2010 (FY10), FORT's scientific and technical professionals conducted ongoing, expanded, and new research vital to the science needs and management goals of DOI, other Federal and State agencies, and nongovernmental organizations in the areas of aquatic systems and fisheries, climate change, data and information integration and management, invasive species, science support, security and technology, status and trends of biological resources (including the socioeconomic aspects), terrestrial and freshwater ecosystems, and wildlife resources, including threatened and endangered species. This report presents selected FORT science accomplishments for FY10 by the specific USGS mission area or science program with which each task is most closely associated, though there is considerable overlap. The report also includes all FORT publications and other products published in FY10, as well as staff accomplishments, appointments, committee assignments, and invited presentations.

  6. Operational experience at Fort St. Vrain

    Energy Technology Data Exchange (ETDEWEB)

    Bramblett, G. C.; Fisher, C. R.; Swart, F. E. [General Atomic Co., San Diego, CA (USA)

    1981-01-15

    The Fort St. Vrain (FSV) station, a 330-MW(e) single reheat steam cycle powered by a high-temperature gas-cooled reactor (HTGR), is the first HTGR to enter commercial operation. Designed and built by General Atomic Company (GA), the plant is owned and operated by Public Service Company of Colorado (PSC). Many unique design features have been incorporated into this reactor system, including high-pressure helium as the primary system coolant, a graphite-moderated prismatic block core design, fission-product-containing carbide coatings on both fissile and fertile fuel particles, steam-driven helium circulators turning on water bearings, and once-through steam generators. All of these systems are contained in a prestressed concrete reactor vessel (PCRV). Extensive testing has been conducted during the rise to power following first criticality early in 1974 to verify system design performance. During this period, the plant has operated at power levels up to 70% and produced over one billion kilowatt hours of electricity. In 1979, the first refueling was conducted in conjunction with an extensive in-core inspection, the addition of in-core instrumentation, and a planned removal of a circulator for inspection.

  7. Miniplate fixation of Le Fort I osteotomies.

    Science.gov (United States)

    Rosen, H M

    1986-12-01

    The use of rigid, internal, three-dimensional fixation using vitallium bone plates in 28 consecutive Le Fort I osteotomies is presented. A minimum follow-up period of 6 months was required for inclusion in this patient group. Maxillary movements included advancements (17), intrusions (9), lengthenings (5), and retrusions (2). The majority of maxillae were moved in more than one plane of space. Technical details, complications, and relapse potential are discussed. Advantages of rigid plate fixation include marked reductions in the length of intermaxillary fixation with light training elastics only. Immediate postoperative airway problems are thereby eliminated. Six months of follow-up would appear to indicate a low potential for osseous relapse when compared to wire osteosynthesis, regardless of the direction of maxillary movement. The major disadvantage is the decreased ability of postoperative orthodontics to move dento-osseous segments if skeletal occlusal disharmony persists postoperatively. For this reason, close attention to preoperative planning and operative technique is critical for the success of this fixation method.

  8. Fort St. Vrain hot functional test results

    International Nuclear Information System (INIS)

    Phelps, R.D.

    1974-01-01

    A description is given of Fort St. Vrain hot functional tests performed to evaluate the initial nonnuclear performance of the primary coolant system and the associated effects on the various internal components of the reactor vessel and primary coolant system. The components included the twelve steam generator modules, the four helium circulators, the PCRV thermal barrier and liner coolant system, the helium purification system, and the primary and secondary closures at each of the PCRV penetrations. Additional objectives included analysis of the parallel operation of the four helium circulators and the performance of several circulator start/stop transients under various conditions of primary coolant temperature and pressure. Vibration and acoustical phenomena within the vessel were measured, recorded, and compared to theoretical analyses; a verification of reverse flow in the shutdown loop steam generator during one loop operation was performed; the PCRV was again observed for its structural response to internal pressure; and comparisons were made relative to data recorded during the initial pressure test completed in July 1971. (U.S.)

  9. Kurt Alfred Georg Mendelssohn 7 January 1906 - 18 September 1980

    International Nuclear Information System (INIS)

    Shoenberg, D.

    1983-01-01

    The life, career and main themes of scientific achievement of Kurt Alfred Georg Mendelssohn are described in detail under the headings: early life in Germany, scientific work, early work in Germany, superconductivity, transport properties of metals, liquid helium, medical physics, Oxford and later life. (U.K.)

  10. Festschrift Charpak (Georges) on his 65th birthday

    CERN Document Server

    Lehmann, Pierre; Rubbia, Carlo; Saudinos, Jean; CERN. Geneva

    1989-01-01

    On the occasion of the 65th birthday of Georges Charpak and of his retirement, the Director-General and the EP Division invite you to a symposium in his h. Chairman : P. Lehmann. - Opening address : C. Rubbia. - Message from V. Weisskopf. - L. Lederman : Superstrings needs sealing wax. - J. Saudinos : Quelques applications des detecteurs gazeux a la medecine et a la biologie.

  11. George Herbert Mead, Um, osoba i društvo

    OpenAIRE

    Bačeković, Alica

    2004-01-01

    Prikaz knjige George Herbert Mead, Um, osoba i društvo sa stajališta socijalnog biheviorista, priredio i uvod napisao Charles W. Morris, s engleskoga preveo Srđan Dvornik, Naklada Jesenski i Turk, Hrvatsko sociološko društvo, Zagreb 2003, xxx + 392 str

  12. George Herbert Mead's Contribution to the Philosophy of American Education.

    Science.gov (United States)

    Renger, Paul, III

    1980-01-01

    George Herbert Mead's general philsophy showed that he regarded the development of distinctively human behavior as essentially the result of an individual's meaningful participation in the social process of the community to which he belongs. Mead believed that education was a social process involving the meaningful interaction and communication…

  13. Drivers Dexterity: George Osodi's Oeuvre on Safety on the Nigerian ...

    African Journals Online (AJOL)

    This paper attempted a reading of the Nigerian condition using the censure of images culled from an exhibition by the Nigerian photographer George Osodi. Drawing attention to the shapes of the images and imageries that constitute the profile of the condition of a major infrastructure, the road and transportation, I have ...

  14. George W. Bush in Political Time: The Janus Presidency

    NARCIS (Netherlands)

    Magliocca, G.N.

    2009-01-01

    This essay places George W. Bush's presidency and the Bush administration in some historical context by applying the model of “political time” developed in recent books by Stephen Skowronek (2008) and Keith Whittington (2007). My thesis is that Bush's political failure during his second term was

  15. Georges Paillard 1932-2004 - French version only

    CERN Multimedia

    2004-01-01

    J'ai eu le plaisir de rencontrer Georges en juillet 1959 au groupe Magnet de la division PS au moment où le centième et dernier aimant venait d'être installé dans l'anneau. Le groupe commençait déjà à s'orienter vers de nouveaux projets et mon premier travail avec Georges consista dans des essais de tubes flash de puissance pour la future chambre à bulles. Ensuite, bien que travaillant dans des domaines différents, mais toujours dans les mêmes divisions, nous ne nous sommes jamais perdus de vue. J'ai rencontré Georges en janvier 1961 au laboratoire d'électronique de la division NPA. Georges avait déjà six ans de CERN : c'était un ancien. Au début, il y eut la chambre à « boules ». Le chef de la division NPA, M. Colin Ramm, australien, éprouvait une certaine difficulté à prononcer le « u » français et une machine classique de la physique nucléaire est devenue l'unique chambre à « boules » jamais construite. Chambre qui connut son heure de gloire puisqu'elle enreg...

  16. The George Wallace Shooting: News Diffusion and the Sleeper Effect.

    Science.gov (United States)

    Steinfatt, Thomas M.; And Others

    Previous studies conclude that the assassination attempt on George C. Wallace was a news event of high, but not maximum, importance, implying that the majority of respondents in any sample would report first learning of the attack via mass media sources. The authors interviewed 144 persons in Ann Arbor, Michigan, regarding their awareness of the…

  17. Catalog of the George Alan Connor Esperanto Collection.

    Science.gov (United States)

    Smith, Karin, Comp.; Haake, Susan, Comp.

    This catalog inventories the collection of books, monographs, serials and periodicals, dictionaries, pamphlets, ephemera, and correspondence concerning Esperanto in the collection of George Alan Connor housed at the University of Oregon Library. Overall, the catalog contains approximately 475 serial entries and 3,000 author entries. Connor was a…

  18. St. George at Prague Castle and Perseus: an Impossible Encounter?

    Czech Academy of Sciences Publication Activity Database

    Bažant, Jan

    2015-01-01

    Roč. 19, 1/2 (2015), s. 189-201 ISSN 1212-5865 Institutional support: RVO:67985955 Keywords : Bellerophon * St. George * iconography * Prague Castle * classical tradition Subject RIV: AB - History http://studiahercynia.ff.cuni.cz/wp-content/uploads/sites/79/2016/05/jan_bazant_189-201.pdf

  19. Tuning the Self : George Herbert's poetry as cognitive behaviour

    NARCIS (Netherlands)

    van Es, Eelco

    2013-01-01

    This book provides a cognitive analysis of the poetry of George Herbert (1593- 1633). From Herbert’s own thinking, recorded in his prose treatises, can be deduced that his poems should serve a specific function: teaching self-knowledge to his readers. Self-knowledge is a necessary skill, to be

  20. Plea Bargaining: A Recreation of George Orwell's Animal Farm in ...

    African Journals Online (AJOL)

    Animal Farm is an allegorical novel by George Orwell in which he depicted social injustice precipitated by the insincerity of leadership and the betrayal of the people by their leaders which has made the novel relevant as the experience depicted therein “seamlessly steps into the realities of everyday life” (Achebe 2012) in ...

  1. Sorose suured soovid / George Soros ; interv. Neeme Raud

    Index Scriptorium Estoniae

    Soros, George, 1930-

    2006-01-01

    Miljardär ja filantroop George Soros vastab küsimustele, mis puudutavad tema raamatut "Ekslikkuse ajastu", kriitika USA kui maailma korratuse ühe põhjustaja suhtes, terroriohu vähendamist maailmas, avatud ühiskonnast

  2. Thymus transplantation for complete DiGeorge syndrome

    DEFF Research Database (Denmark)

    Davies, E Graham; Cheung, Melissa; Gilmour, Kimberly

    2017-01-01

    BACKGROUND: Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS). METHODS: Twelve patients with cDGS underwent transplantation with allogeneic cultured thymus. OBJECTIVE: We sought to confirm and extend the results previously obtained in a ...

  3. George F. Kennan and the Division of Europe

    Czech Academy of Sciences Publication Activity Database

    Smetana, Vít

    2013-01-01

    Roč. 15, č. 4 (2013), s. 225-232 ISSN 1520-3972 Institutional support: RVO:68378114 Keywords : George F. Kennan * Cold War * U.S. foreign policy Subject RIV: AB - History Impact factor: 0.349, year: 2013

  4. George de Hevesy (1885 - 1966), founder of radioanalytical chemistry

    International Nuclear Information System (INIS)

    Niese, S.

    2006-01-01

    George de Hevesy has founded Radioanalytical Chemistry and Nuclear Medicine, discovered the element hafnium and first separated stable isotopes. For all these different important achievements he was nominated for the Nobel Prize. On occasion of his discovery of activation analysis 70 years ago his life and work will be illustrated. (author)

  5. George Smoot, Blackbody, and Anisotropy of the Cosmic Microwave Background

    Science.gov (United States)

    the Cosmic Microwave Background Radiation Resources with Additional Information * Videos 'George Smoot anisotropy of the cosmic microwave background radiation." '1 Smoot previously won the Ernest Orlando . Smoot, blackbody, and anisotropy of the Cosmic Microwave Background (CMB) radiation is available in full

  6. In Praise of George Peabody, 1795-1869.

    Science.gov (United States)

    Parker, Franklin

    1994-01-01

    One of several papers on self-made millionaire and educational philanthropist George Peabody defends Peabody's philanthropy against those who criticized him as miserly, highlighting his establishment of colleges, libraries, museums, hospitals, and other charities. The paper details his life, work, politics, and death, describing the causes that he…

  7. Georges et les trésors du cosmos

    CERN Document Server

    Hawking, Lucy; Parsons, Gary

    2011-01-01

    Les voisins excentriques de Georges, Annie et Eric, ont déménagé en Floride à l'agence Spatiale Globale. Là-bas, Eric s'occupe de son nouveau robot, Homer, qui doit détécter les signes de vie sur Mars. Mais bientôt, Georges reçoit un e-mail : Annie lui demande de la rejoindre au plus vite pour une " mission cosmique " secrète. La jeune fille est persuadée qu'il se passe de drôles de choses sur Mars, car Homer vient de recevoir un message extraterrestre ! Georges et Anne (avec l'aide d'Emmett, un petit garçon bizarre) décident de réparer Cosmos, leur super-ordinateur, et d'aller voir par eux-mêmes... Emmaillotés dans des combinaisons spéciales, ils se lancent alors dans une fabuleuse chasse aux trésors sur Mars, sur les lunes de Saturne, puis sur Titan, avant de se perdre en orbite autour d'Alpha Centauri B... Mais avant de pouvoir élucider le mystère du message extraterrestre, Georges et Annie devront se livrer à une bataille spatiale acharnée qui mettra leur vie en danger, ainsi que ce...

  8. Vehicles to Belief: Aristotle's Enthymeme and George Campbell's Vivacity Compared.

    Science.gov (United States)

    Roob, Andy

    The central concepts from two rhetorical systems (the enthymeme in Aristotle's rhetoric and vivacity in George Campbell's) may be understood as the connection between speech act and ascension to belief. A review of the literature indicates a gap in the scholarly works seeking to compare and contrast the periods developed by D. Ehninger's systems…

  9. Moessbauer Study of Sedimentary Rocks from King George Island, Antarctica

    International Nuclear Information System (INIS)

    Kuzmann, E.; Souza, P. A. de; Schuch, L. A.; Oliveira, A. C. de; Garg, R.; Garg, V. K.

    2002-01-01

    The separation of continents at the periphery of Antarctica occurred about 180 ma ago due to volcanic activity. Geological faults can be very important in the study of geological occurrences. Such geological faults occur across the Admiralty Bay, King George Island, and have been studied in detail previously. Controversial statements were given in earlier works, based on conventional geological investigations, as to whether altered 'Jurassic' and unaltered Tertiary rocks were separated by a major fault which goes across the Admiralty Bay, or whether there is no difference in the alteration of the rocks located at either side of the fault. The aim of our work is to investigate rock samples from the Admiralty Bay of King George Island, Antarctica, from different locations on both sides of the geological fault. For these investigations 57 Fe Moessbauer spectroscopy and X-ray diffractometry were used. We have found that the phase composition, and the iron distribution among the crystallographic sites of iron-bearing minerals, are characteristic of the location of the rock samples from the Admiralty Bay of King George Island. There is a much higher amount of iron oxides in the rocks from the south part of the geological fault than in the north part. The differences in the mineral composition and iron distribution showed that the rocks in the southern part of the geological fault of King George Island are significantly altered compared to the rocks in the northern part. Our present results support and complement well the results obtained earlier on soils from King George Island.

  10. 75 FR 41922 - Notice of Intent To Rule on Request To Release Airport Property at Fort Smith Regional Airport...

    Science.gov (United States)

    2010-07-19

    ... To Release Airport Property at Fort Smith Regional Airport, Fort Smith, AR AGENCY: Federal Aviation... rule and invites public comment on the release of land at Fort Smith Regional Airport under the.... John Parker, Airport Director, Fort Smith Regional Airport, at the following address: Fort Smith...

  11. Molecular characterization of a novel HEXA mutation at the +3 position of intron 8 in a Tay-Sachs disease patient

    Energy Technology Data Exchange (ETDEWEB)

    Richard, M.; Triggs-Raine, B. [Univ. of Manitoba, Winnipeg (Canada); Natowicz, M. [E.K. Shriver Center, Waltham, MA (United States)

    1994-09-01

    Tay-Sachs disease is an autosomal recessive lysosomal storage disorder resulting from mutations in the HEXA gene that cause a deficiency in the activity of that enzyme {beta}-hexosaminidase A (Hex A). This deficiency leads to the build-up of G{sub M2} ganglioside, resulting in neurodegeneration and death. Biochemical analysis of a non-Jewish patient with a late-infantile form of Tay-Sachs disease revealed a substantial level of Hex A activity (38.4%) when 4-MUG was used as the substrate. However, when a substrate (4-MUGS) specific for the {alpha}-subunit of Hex A ({alpha}{beta}) was used, almost no activity was detected in the HEXA gene of the patient using SSCP analysis followed by sequencing. The first mutation, a G533A substitution in exon 5, is previously described and associated with the B1 form of Tay-Sachs disease. The second mutation is a novel a-to-g base change at the +3 position of intron 8. This was confirmed using the AIRS method, whereby a MaeIII site was created in the presence of the mutation. Normal and patient mRNA was reverse transcribed and exons 7 to 9 were PCR-amplified from the cDNA. An abnormally sized amplification product detected only in the patient cDNA was sequenced; exon 8 had been deleted and exons 7 and 9 were spliced together. A substantial level of normally-sized PCR product was also detected in the patient`s cDNA. Experiments are in progress to determine if this is produced from the allele harboring the G533A mutation. Given that previous mutations of this type have been associated with 97-100% abnormal splicing, this mutation is likely to be the cause, together with the G533A mutation, of Tay-Sachs disease in this patient.

  12. Area density of localization-entropy II: double cone-localization and quantum origin of the Bondi-Metzner-Sachs symmetry

    Energy Technology Data Exchange (ETDEWEB)

    Schroer, Bert

    2006-04-15

    The holographic encoding is generalized to subalgebras of QFT localized in double cones. It is shown that as a result of this radically different spacetime encoding the modular group acts geometrically on the holographic image. As a result we obtain a formula for localization entropy which is identical to the previously derived formula for the wedge-localized subalgebra. The symmetry group in the holographic encoding turns out to be the Bondi-Metzner-Sachs group. (author)

  13. 77 FR 59035 - Notice of Intent To Rule on Request To Release Airport Property at the St. George Airport, St...

    Science.gov (United States)

    2012-09-25

    ... To Release Airport Property at the St. George Airport, St. George, UT AGENCY: Federal Aviation... rule and invite public comment on the release of land at St. George Airport under the provisions of..., City of St. George, Utah, at the following address: Mr. Gary Esplin, City Manager, City of St. George...

  14. Georges Darrieus, inventor of vertical axis wind-turbines; Georges Darrieus, pere des eoliennes a axe vertical

    Energy Technology Data Exchange (ETDEWEB)

    Rogier, E.

    2000-10-01

    This article is a short biography of the French man Georges Darrieus (1888-1979) who invented the vertical wind turbine. G.Darrieus can be considered as the first industrial researcher in France, he was a double authority in sciences and in industry machinery. He presented more than 50 patterns and wrote about 700 scientific and technological articles.

  15. Master Mentors: Linda Golian-Lui University of Hawaii-Hilo--George Oberle George Mason University, VA

    Science.gov (United States)

    Library Journal, 2005

    2005-01-01

    Many movers and shakers inspire admiration and even awe, but those who inspire love are the mentors who have changed lives. That is why many grateful librarians nominated Linda Golian-Lui and George Oberle. Both of them were accidental librarians, work-study students whose supervisors encouraged them to become paraprofessionals, and then…

  16. U.S. GLOBEC Georges Bank Long-Term Moored Program. Part 1. Mooring Configuration

    National Research Council Canada - National Science Library

    Irish, J. D; Kerry, S; Fucile, P; Beardsley, R. C; Lord, J; Brink, K. H

    2005-01-01

    As part of the U.S. GLOBEC Northwest Atlantic/Georges Bank program, moorings were deployed on Georges Bank as part of the broad-scale survey component to help measure the temporal variability of both physical...

  17. 78 FR 76189 - Supplemental Environmental Impact Statement for the Route 460 Location Study, Prince George...

    Science.gov (United States)

    2013-12-16

    ... DEPARTMENT OF TRANSPORTATION Federal Highway Administration Supplemental Environmental Impact Statement for the Route 460 Location Study, Prince George County to Suffolk, Virginia AGENCY: Federal... roughly parallel to the existing Route 460 corridor between Interstate 295 in Prince George County and...

  18. Thunderstorm and Lightning Studies using the FORTE Optical Lightning System (FORTE/OLS)

    Energy Technology Data Exchange (ETDEWEB)

    Argo, P.; Franz, R.; Green, J.; Guillen, J.L.; Jacobson, A.R.; Kirkland, M.; Knox, S.; Spalding, R.; Suszcynsky, D.M.

    1999-02-01

    Preliminary observations of simultaneous RF and optical emissions from lightning as seen by the FORTE spacecraft are presented. RF/optical pairs of waveforms are routinely collected both as individual lightning events and as sequences of events associated with cloud-to-ground (CG) and intra-cloud (IC) flashes. CG pulses can be distinguished from IC pulses based on the properties of the RF and optical waveforms, but mostly based on the associated RF spectrograms. The RF spectrograms are very similar to previous ground-based VHF observations of lightning and show signatures associated with return strokes, stepped and dart leaders, and attachment processes,. RF emissions are observed to precede the arrival of optical emissions at the satellite by a mean value of 280 microseconds. The dual phenomenology nature of these observations are discussed in terms of their ability to contribute to a satellite-based lightning monitoring mission.

  19. Thunderstorm and Lightning Studies using the FORTE Optical Lightning System (FORTE/OLS)

    International Nuclear Information System (INIS)

    Argo, P.; Franz, R.; Green, J.; Guillen, J.L.; Jacobson, A.R.; Kirkland, M.; Knox, S.; Spalding, R.; Suszcynsky, D.M.

    1999-01-01

    Preliminary observations of simultaneous RF and optical emissions from lightning as seen by the FORTE spacecraft are presented. RF/optical pairs of waveforms are routinely collected both as individual lightning events and as sequences of events associated with cloud-to-ground (CG) and intra-cloud (IC) flashes. CG pulses can be distinguished from IC pulses based on the properties of the RF and optical waveforms, but mostly based on the associated RF spectrograms. The RF spectrograms are very similar to previous ground-based VHF observations of lightning and show signatures associated with return strokes, stepped and dart leaders, and attachment processes,. RF emissions are observed to precede the arrival of optical emissions at the satellite by a mean value of 280 microseconds. The dual phenomenology nature of these observations are discussed in terms of their ability to contribute to a satellite-based lightning monitoring mission

  20. Georges et les secrets de l'univers

    CERN Document Server

    Hawking, Lucy; Galfard, Christophe; Parsons, Gary

    2011-01-01

    Un voyage ex-tra-or-di-naire dans l'Univers! Le jour où Georges rencontre ses nouveaux voisins, il est loin d'imaginer que sa vision du monde va être totalement bouleversée. Chez la jeune Annie et Éric, son scientifique de père, le garçon découvre Cosmos, l'ordinateur le plus perfectionné qui soit. Sa super intelligence permet de protéger Georges et ses amis aux quatre coins de l'Univers ! Hélas, quelqu'un nourrit de sombres projets... et quand on sait qu'il n y a rien de plus dangereux dans l'espace qu'un trou noir, il y a du souci à se faire ! Un roman essentiel sur l'Univers avec les découvertes de Stephen Hawking.

  1. Il Cabinet d'amateur di Georges Perec

    Directory of Open Access Journals (Sweden)

    Valeria Cammarata

    2011-06-01

    Full Text Available In 1979 one year after the publication of La vie mode d’emploi, George Perec wrote Cabinet d’amateur. Histoire d’un tableau, in which he tells the story of a painting belonging to the genre of the cabinet d’amateur, also known as kunst- und wunderkammer. It is a collection of different paintings, usually belonging to a rich man or a notable, portrayed (once again in a single picture celebrating the magnificence of this more or less real collection. As for the pictorial genre, so the short novel is the celebration of the images collected by George Perec in his previous masterpiece, La vie mode d’emploi. This is why I assume this work to be referable to the genre of structural homology more than to that of ékphrasis, being the pictorial structure not only the thematic inspiration or motif of the novel but its own underlying structure

  2. Georges Didi-Huberman, Images malgré tout

    OpenAIRE

    Shafto, Sally

    2008-01-01

    « Pour savoir, il faut s’imaginer. »Georges Didi-Huberman Avec son dernier livre, Images malgré tout, l’historien de l’art et philosophe Georges Didi-Huberman nous livre un texte élégamment écrit et divisé en trois mouvements. Il y a d’abord une réimpression de son article, paru auparavant dans le catalogue, Mémoires des camps. Photographies des camps de concentration et d’extermination nazis 1933-1999. Il s’agit d’une lecture phénoménologique de quatre photographies, prises à Auschwitz en ao...

  3. 77 FR 1716 - James River National Wildlife Refuge, Prince George County, VA

    Science.gov (United States)

    2012-01-11

    ... River National Wildlife Refuge, Prince George County, VA AGENCY: Fish and Wildlife Service, Interior... (the refuge, NWR), which is located in Prince George County, Virginia. We provide this notice in... River NWR, in Prince George County, Virginia. This notice complies with our CCP policy to advise other...

  4. 75 FR 21243 - Marine Mammals; Subsistence Taking of Northern Fur Seals; St. George

    Science.gov (United States)

    2010-04-23

    ... Mammals; Subsistence Taking of Northern Fur Seals; St. George AGENCY: National Marine Fisheries Service... (APA). The Pribilof Island Community of St. George Island, Traditional Council (Council) petitioned... St. George Island to take male fur seal young of the year during the fall. NMFS solicits public...

  5. Le Fort I Maxillary Advancement Using Distraction Osteogenesis

    Science.gov (United States)

    Combs, Patrick D.; Harshbarger, Raymond J.

    2014-01-01

    Treatment of maxillary hypoplasia has traditionally involved conventional Le Fort I osteotomies and advancement. Advancements of greater than 10 mm risk significant relapse. This risk is greater in the cleft lip and palate population, whose anatomy and soft tissue scarring from prior procedures contributes to instability of conventional maxillary advancement. Le Fort I advancement with distraction osteogenesis has emerged as viable, stable treatment modality correction of severe maxillary hypoplasia in cleft, syndromic, and noncleft patients. In this article, the authors provide a review of current data and recommendations concerning Le Fort I advancement with distraction osteogenesis. In addition, they outline their technique for treating severe maxillary hypoplasia with distraction osteogenesis using internal devices. PMID:25383054

  6. Targeting Net Zero Energy at Fort Carson: Assessment and Recommendations

    Energy Technology Data Exchange (ETDEWEB)

    Anderson, K.; Markel, T.; Simpson, M.; Leahey, J.; Rockenbaugh, C.; Lisell, L.; Burman, K.; Singer, M.

    2011-10-01

    The U.S. Army's Fort Carson installation was selected to serve as a prototype for net zero energy assessment and planning. NREL performed the comprehensive assessment to appraise the potential of Fort Carson to achieve net zero energy status through energy efficiency, renewable energy, and electric vehicle integration. This report summarizes the results of the assessment and provides energy recommendations. This study is part of a larger cross-laboratory effort that also includes an assessment of renewable opportunities at seven other DoD Front Range installations, a microgrid design for Fort Carson critical loads and an assessment of regulatory and market-based barriers to a regional secure smart grid.

  7. Cranial nerve injury after Le Fort I osteotomy.

    Science.gov (United States)

    Kim, J-W; Chin, B-R; Park, H-S; Lee, S-H; Kwon, T-G

    2011-03-01

    A Le Fort I osteotomy is widely used to correct dentofacial deformity because it is a safe and reliable surgical method. Although rare, various complications have been reported in relation to pterygomaxillary separation. Cranial nerve damage is one of the serious complications that can occur after Le Fort I osteotomy. In this report, a 19-year-old man with unilateral cleft lip and palate underwent surgery to correct maxillary hypoplasia, asymmetry and mandibular prognathism. After the Le Fort I maxillary osteotomy, the patient showed multiple cranial nerve damage; an impairment of outward movement of the eye (abducens nerve), decreased vision (optic nerve), and paraesthesia of the frontal and upper cheek area (ophthalmic and maxillary nerve). The damage to the cranial nerve was related to an unexpected sphenoid bone fracture and subsequent trauma in the cavernous sinus during the pterygomaxillary osteotomy. Copyright © 2010 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  8. Festschrift Charpak (Georges) on his 65th birthday

    CERN Document Server

    Saudinos, Jean; Garwin, Richard L; Uggerhøj, Erik; CERN. Geneva

    1989-01-01

    On the occasion of the 65th birthday of Georges Charpak and of his retirement, the Director-General and the EP Division invite you to a symposium in his h. Chairman : P. Lehmann. - J. Saudinos : Quelques applications des détecteurs gazeux à la médecine et à la biologie. - E. Uggerhøj : New steps in the channeling of relativistic particles. - R. Garwin : Remarks from the non-physical world. - G. Charpak : Final speech.

  9. Georges Lema\\^itre: Life, Science and Legacy

    OpenAIRE

    Mitton, Simon

    2016-01-01

    This paper celebrates the remarkable life, science and legacy of Abb\\'e Georges Lema\\^itre, the Belgian cleric and professor of physics; he was the architect of the fireworks model for the origin of the universe. He died half a century ago, three days after learning that Arno Penzias and Robert Wilson had discovered the cosmic microwave background. Despite being gravely ill from leukaemia, Lema\\^itre lucidly praised this news, which confirmed the explosive genesis of our universe.

  10. DiGeorge Syndrome: a not so rare disease

    OpenAIRE

    Fomin,Angela BF; Pastorino,Antonio Carlos; Kim,Chong Ae; Pereira,CA; Carneiro-Sampaio,Magda; Abe-Jacob,Cristina Miuki

    2010-01-01

    INTRODUCTION: The DiGeorge Syndrome was first described in 1968 as a primary immunodeficiency resulting from the abnormal development of the third and fourth pharyngeal pouches during embryonic life. It is characterized by hypocalcemia due to hypoparathyroidism, heart defects, and thymic hypoplasia or aplasia. Its incidence is 1:3000 live births and, despite its high frequency, little is known about its natural history and progression. ?This is probably due to diagnostic difficulties and the ...

  11. UF6 overfilling prevention at Eurodif production Georges Besse plant

    Energy Technology Data Exchange (ETDEWEB)

    Reneaud, J.M. [Eurodif Production, Pierrelatte (France)

    1991-12-31

    Risk of overfilling exists on different equipments of Georges BESSE Plant: cylinders, desublimers and intermediate tanks. The preventive measures are composed of technical devices: desublimers weighing, load monitoring alarms, automatic controls ... and procedures, training, safety organization. In thirteen years of operation, some incidents have occurred but none of them has caused any personal injuries. They are related and discussed. The main factors involved in the Sequoyah fuel facility accident on 1/4/1986 have been analyzed and taken into account.

  12. Obituary: George Hamilton Bowen Jr. (1925-2009)

    Science.gov (United States)

    Willson, Lee Anne; Struck, Curtis

    2011-12-01

    Our colleague and collaborator George Hamilton Bowen, Jr., passed away November 1, 2009 in Ames, Iowa. George was born June 20, 1925 in Tulsa, Oklahoma to George and Dorothy (Huntington) Bowen. He married Marjorie Brown June 19, 1948 in Redondo Beach, California; they had five children, with eight grandchildren and five great-grandchildren at the time of his death. George H. Bowen's third or perhaps his fourth career was in astronomy. He was drafted into the navy in 1944, at the end of his first year as a student at Caltech, and ended his war-time service as an electronic technician on the aircraft carrier Shangri-La. He later said "In just nine months, starting from scratch (Ohm's law!), we learned an amazing amount - not by memorization, of course, but by study and real understanding of the basic function of the most advanced AC circuits then being used for instrumentation, measurements, communications, control systems, and much more." He gained a confidence that he could quickly and accurately diagnose and solve technical problems that stood him well in future work. One accomplishment he took particular pride in was figuring out how the radar control used cams and gears to solve the trigonometry for accurate pointing. He also described how the captain was alarmed when weather conditions changed so that refraction no longer showed them distant, small boats around the curvature of Earth. After the war, George Bowen returned to undergraduate and eventually graduate study at Caltech, where he was recruited to the biophysics research group headed by future Nobel Laureate Max Delbrück. George often described his joy in working with these first-rate scientists and finding himself accepted as a part of the effort. He finished his BS with honors in 1949 and his PhD in 1953 with a thesis on "Kinetic Studies on the Mechanism of Photoreactivation of Bacteriophase T2 Inactivated by Ultraviolet Light" involving work with E Coli. This work was supported by grants from the U

  13. DiGeorge Syndrome: a not so rare disease

    Directory of Open Access Journals (Sweden)

    Angela BF Fomin

    2010-01-01

    Full Text Available INTRODUCTION: The DiGeorge Syndrome was first described in 1968 as a primary immunodeficiency resulting from the abnormal development of the third and fourth pharyngeal pouches during embryonic life. It is characterized by hypocalcemia due to hypoparathyroidism, heart defects, and thymic hypoplasia or aplasia. Its incidence is 1:3000 live births and, despite its high frequency, little is known about its natural history and progression. ←This is probably due to diagnostic difficulties and the great variety of names used to describe it, such as velocardiofacial, Shprintzen, DiGeorge, and CATCH 22 Syndromes, as well as conotruncal facial anomaly. All represent the same genetic condition, chromosome 22q11.2 deletion, which might have several clinical expressions. OBJECTIVES: To describe clinical and laboratorial data and phenotypic characteristics of patients with DiGeorge Syndrome. METHODS: Patients underwent standard clinical and epidemiological protocol and tests to detect heart diseases, facial abnormalities, dimorphisms, neurological or behavioral disorders, recurrent infections and other comorbidities. RESULTS: Of 14 patients (8m - 18y11m, only one did not have 22q11.2 deletion detected. The main findings were: conotruncal malformation (n = 12, facial abnormalities (n = 11, hypocalcemia (n = 5 and low lymphocyte count (n=2. CONCLUSION: The authors pointed out the necessity of DGS suspicion in all patient presenting with heart defects, facial abnormalities (associated or not with hypocalcemia, and immunological disorders because although frequency of DGS is high, few patients with a confirmed diagnosis are followed up.

  14. Memory effects of transformation textures in steel and its prediction by the double Kurdjumov–Sachs relation

    International Nuclear Information System (INIS)

    Tomida, T.; Wakita, M.; Yasuyama, M.; Sugaya, S.; Tomota, Y.; Vogel, S.C.

    2013-01-01

    The phenomenon that the transformation texture near the initial texture reproduces after the phase transformation cycle such as ferrite (α, body-centered cubic) → austenite (γ, face-centered cubic) → α is called a texture memory. In this study, the texture change in a 0.1% C–1% Mn hot-rolled steel sheet during the α → γ → α transformation cycle was studied via neutron diffraction and the transformation texture prediction based on a variant selection rule that we call the double Kurdjumov–Sachs (K–S) relation. The texture change observed by neutron diffraction, which clearly showed the texture memory, could be quantitatively reproduced by the proposed variant selection rule adopted into the calculation method based on the spherical harmonics expansion of orientation distribution functions. Therefore, it is most likely that the texture memory in steel is caused by the preferential selection of those K–S variants that reduce the interfacial energy between a precipitate and two adjoining parent phase grains at the same time, which we call the double K–S relation

  15. A direct gene transfer strategy via brain internal capsule reverses the biochemical defect in Tay-Sachs disease.

    Science.gov (United States)

    Martino, S; Marconi, P; Tancini, B; Dolcetta, D; De Angelis, M G Cusella; Montanucci, P; Bregola, G; Sandhoff, K; Bordignon, C; Emiliani, C; Manservigi, R; Orlacchio, A

    2005-08-01

    Therapy for neurodegenerative lysosomal Tay-Sachs (TS) disease requires active hexosaminidase (Hex) A production in the central nervous system and an efficient therapeutic approach that can act faster than human disease progression. We combined the efficacy of a non-replicating Herpes simplex vector encoding for the Hex A alpha-subunit (HSV-T0alphaHex) and the anatomic structure of the brain internal capsule to distribute the missing enzyme optimally. With this gene transfer strategy, for the first time, we re-established the Hex A activity and totally removed the GM2 ganglioside storage in both injected and controlateral hemispheres, in the cerebellum and spinal cord of TS animal model in the span of one month's treatment. In our studies, no adverse effects were observed due to the viral vector, injection site or gene expression and on the basis of these results, we feel confident that the same approach could be applied to similar diseases involving an enzyme defect.

  16. Tay–Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum–associated degradation

    Science.gov (United States)

    Dersh, Devin; Iwamoto, Yuichiro; Argon, Yair

    2016-01-01

    Loss of function of the enzyme β-hexosaminidase A (HexA) causes the lysosomal storage disorder Tay–Sachs disease (TSD). It has been proposed that mutations in the α chain of HexA can impair folding, enzyme assembly, and/or trafficking, yet there is surprisingly little known about the mechanisms of these potential routes of pathogenesis. We therefore investigated the biosynthesis and trafficking of TSD-associated HexA α mutants, seeking to identify relevant cellular quality control mechanisms. The α mutants E482K and G269S are defective in enzymatic activity, unprocessed by lysosomal proteases, and exhibit altered folding pathways compared with wild-type α. E482K is more severely misfolded than G269S, as observed by its aggregation and inability to associate with the HexA β chain. Importantly, both mutants are retrotranslocated from the endoplasmic reticulum (ER) to the cytosol and are degraded by the proteasome, indicating that they are cleared via ER-associated degradation (ERAD). Leveraging these discoveries, we observed that manipulating the cellular folding environment or ERAD pathways can alter the kinetics of mutant α degradation. Additionally, growth of patient fibroblasts at a permissive temperature or with chemical chaperones increases cellular Hex activity by improving mutant α folding. Therefore modulation of the ER quality control systems may be a potential therapeutic route for improving some forms of TSD. PMID:27682588

  17. Tay-Sachs Carrier Screening by Enzyme and Molecular Analyses in the New York City Minority Population.

    Science.gov (United States)

    Mehta, Nikita; Lazarin, Gabriel A; Spiegel, Erica; Berentsen, Kathleen; Brennan, Kelly; Giordano, Jessica; Haque, Imran S; Wapner, Ronald

    2016-09-01

    Carrier screening for Tay-Sachs disease is performed by sequence analysis of the HEXA gene and/or hexosaminidase A enzymatic activity testing. Enzymatic analysis (EA) has been suggested as the optimal carrier screening method, especially in non-Ashkenazi Jewish (non-AJ) individuals, but its utilization and efficacy have not been fully evaluated in the general population. This study assesses the reliability of EA in comparison with HEXA sequence analysis in non-AJ populations. Five hundred eight Hispanic and African American patients (516 samples) had EA of their leukocytes performed and 12 of these patients who tested positive by EA ("carriers") had subsequent HEXA gene sequencing performed. Of the 508 patients, 25 (4.9%) were EA positive and 40 (7.9%) were inconclusive. Of the 12 patients who were sequenced, 11 did not carry a pathogenic variant and one carried a likely deleterious mutation (NM_000520.4(HEXA):c.1510C>T). High inconclusive rates and poor correlation between positive/inconclusive enzyme results and identification of pathogenic mutations suggest that ethnic-specific recalibration of reference ranges for EA may be necessary. Alternatively, HEXA gene sequencing could be performed.

  18. Mortality incidence estimation using federal death certificate and natality data with an application to Tay-Sachs disease.

    Science.gov (United States)

    Jalal, Kabir; Carter, Randy L

    2015-09-01

    For confidentiality reasons, US federal death certificate data are incomplete with regards to the dates of birth and death for the decedents, making calculation of total lifetime of a decedent impossible and thus estimation of mortality incidence difficult. This paper proposes the use of natality data and an imputation-based method to estimate age-specific mortality incidence rates in the face of this missing information. By utilizing previously determined probabilities of birth, a birth date and death date are imputed for every decedent in the dataset. Thus, the birth cohort of each individual is imputed, and the total on-study time can be calculated. This idea is implemented in two approaches for estimation of mortality incidence rates. The first is an extension of a person-time approach, while the second is an extension of a life table approach. Monte Carlo simulations showed that both approaches perform well in comparison to the ideal complete data methods, but that the person-time method is preferred. An application to Tay-Sachs disease is demonstrated. It is concluded that the imputation methods proposed provide valid estimates of the incidence of death from death certificate data without the need for additional assumptions under which usual mortality rates provide valid estimates. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  19. Assessing the potential success of cystic fibrosis carrier screening: lessons learned from Tay-Sachs disease and beta-thalassemia.

    Science.gov (United States)

    Laberge, A-M; Watts, C; Porter, K; Burke, W

    2010-01-01

    The objective of this study was to identify factors involved in the success of 2 well-established population-based carrier screening programs - Tay-Sachs disease (TSD) in Ashkenazi Jews and beta-thalassemia in Sardinia and Cyprus - and to assess the potential for success of a population-based cystic fibrosis (CF) carrier screening strategy using these factors. We performed a literature review and key informant interviews. Factors involved in the success of TSD and beta-thalassemia carrier screening programs include disease characteristics (well-defined population at risk, severe disease with predictable course, availability of effective treatment), test characteristics (high sensitivity, straightforward interpretation of results), and community characteristics (involvement of community, support of families and advocacy groups, consensus in favor of avoiding affected births). Current CF screening strategies include few of the factors listed above. Unlike TSD and beta-thalassemia, the purpose of current CF carrier screening strategies is informed reproductive decision-making, without an explicit goal of reducing disease incidence. When compared to TSD and beta-thalassemia, CF is a less favorable candidate for population-based carrier screening. Because of its different purpose, CF carrier screening will require different measures of success than those used for TSD and beta-thalassemia carrier screening, and a consensus on the value or success of CF carrier screening may be difficult to achieve.

  20. Initial-boundary-value problem of the self-gravitating scalar field in the Bondi-Sachs gauge

    International Nuclear Information System (INIS)

    Frittelli, Simonetta; Gomez, Roberto

    2007-01-01

    It is shown that, in the Bondi-Sachs gauge that fixes the speed of incoming light rays to the value 1, the Einstein equations coupled to a scalar field in spherical symmetry are cast into a symmetric-hyperbolic system of equations for the scalar field, lapse and shift as fundamental variables. In this system of equations, the lapse and shift are incoming characteristic fields, and the scalar field has three components: incoming, outgoing and static. A constraint-preserving boundary condition is prescribed by imposing the projection of the Einstein equation normal to the boundary at the outer value of the radial coordinate. The boundary condition specifies one of the two incoming metric fields. The remaining incoming metric field and the incoming scalar field component need to be specified arbitrarily. Numerical simulations of the scattering of the scalar field by a black hole in the nonlinear regime are presented that illustrate interesting facts about black-hole physics and the behavior of the characteristic variables of the problem

  1. Fort Collins Science Center-Fiscal year 2009 science accomplishments

    Science.gov (United States)

    Wilson, Juliette T.

    2010-01-01

    Public land and natural resource managers in the United States are confronted with increasingly complex decisions that have important ramifications for both ecological and human systems. The scientists and technical professionals at the U.S. Geological Survey Fort Collins Science Center?many of whom are at the forefront of their fields?possess a unique blend of ecological, socioeconomic, and technological expertise. Because of this diverse talent, Fort Collins Science Center staff are able to apply a systems approach to investigating complicated ecological problems in a way that helps answer critical management questions. In addition, the Fort Collins Science Center has a long record of working closely with the academic community through cooperative agreements and other collaborations. The Fort Collins Science Center is deeply engaged with other U.S. Geological Survey science centers and partners throughout the Department of the Interior. As a regular practice, we incorporate the expertise of these partners in providing a full complement of ?the right people? to effectively tackle the multifaceted research problems of today's resource-management world. In Fiscal Year 2009, the Fort Collins Science Center's scientific and technical professionals continued research vital to Department of the Interior's science and management needs. Fort Collins Science Center work also supported the science needs of other Federal and State agencies as well as non-government organizations. Specifically, Fort Collins Science Center research and technical assistance focused on client and partner needs and goals in the areas of biological information management and delivery, enterprise information, fisheries and aquatic systems, invasive species, status and trends of biological resources (including human dimensions), terrestrial ecosystems, and wildlife resources. In the process, Fort Collins Science Center science addressed natural-science information needs identified in the U

  2. Black Swan Event Assessment for Fort Leonard Wood, Missouri

    Science.gov (United States)

    2016-03-01

    ER D C/ CE RL S R- 16 -1 Net Zero Planning for Fort Leonard Wood Black Swan Event Assessment for Fort Leonard Wood, Missouri Co ns...search for other technical reports published by ERDC, visit the ERDC online library at http://acwc.sdp.sirsi.net/client/default. Net Zero Planning for...1.8 degrees Celsius knots 0.5144444 meters per second miles (US statute) 1,609.347 meters miles per hour 0.44704 meters per second ERDC/CERL SR

  3. Sundhedsprofessionelles begejstring for fortællinger fra levet erfaring

    DEFF Research Database (Denmark)

    Liveng, Anne; Larsen, Christine; Lange, Mads

    2018-01-01

    I 2013 etablerede psykiatrien i Region Hovedstaden, Danmark, et undervisningsprogram om recovery for sundhedsprofessionelle. Evalueringer af programmet viste et udtalt engagement i fortællingen fra underviseren med levet erfaring. Artiklen diskuterer hvordan dette kan forstås. Evalueringsmaterialet...... analyseres ud fra et læringsteoretisk perspektiv og fokuserer på: 1) Betydningen af fortællingens emotionelle indhold, 2) Rolle-bytningen mellem personen med levet erfaring og sundhedsprofessionelle, og 3) Workshoppene som et læringsrum, der aktiverer refleksioner over strukturer og organisering af...

  4. Renewable Energy Opportunities at Fort Campbell, Tennessee/Kentucky

    Energy Technology Data Exchange (ETDEWEB)

    Hand, James R.; Horner, Jacob A.; Kora, Angela R.; Orrell, Alice C.; Russo, Bryan J.; Weimar, Mark R.; Nesse, Ronald J.

    2011-03-31

    This document provides an overview of renewable resource potential at Fort Campbell, based primarily upon analysis of secondary data sources supplemented with limited on-site evaluations. This effort focuses on grid-connected generation of electricity from renewable energy sources and also on ground source heat pumps for heating and cooling buildings. The effort was funded by the U.S. Army Installation Management Command (IMCOM) as follow-on to the 2005 Department of Defense (DoD) Renewables Assessment. The site visit to Fort Campbell took place on June 10, 2010.

  5. Renewable Energy Opportunities at Fort Drum, New York

    Energy Technology Data Exchange (ETDEWEB)

    Brown, Scott A.; Orrell, Alice C.; Solana, Amy E.; Williamson, Jennifer L.; Hand, James R.; Russo, Bryan J.; Weimar, Mark R.; Rowley, Steven; Nesse, Ronald J.

    2010-10-20

    This document provides an overview of renewable resource potential at Fort Drum, based primarily upon analysis of secondary data sources supplemented with limited on-site evaluations. This effort focuses on grid-connected generation of electricity from renewable energy sources and also on ground source heat pumps for heating and cooling buildings. The effort was funded by the U.S. Army Installation Management Command (IMCOM) as follow-on to the 2005 Department of Defense (DoD) Renewables Assessment. The site visit to Fort Drum took place on May 4 and 5, 2010.

  6. Construction experience on PCRV liners at Fort St. Vrain

    International Nuclear Information System (INIS)

    Cliff, J.O.; Wunderlich, R.G.

    1976-01-01

    The construction of the steel liners for the Fort St. Vrain prestressed concrete reactor vessel presented many unique problems for which techniques were developed to satisfy the rigid specification requirements. The PCRV cavity liner was fabricated from 1.9cm carbon steel plate. The liners were partially fabricated by Pittsburgh-Des Moines Steel Company at their Pittsburgh manufacturing facility. The liners were then shipped by rail to within approximately five miles of the jobsite and then trucked the remaining distance. The construction techniques, dimensional control, concrete support and testing utilized on the Fort St. Vrain project are presented in detail and demonstrate the flexibility of the PCRV for field construction. (author)

  7. En fascinerende fortælling om det 20. århundredes musik

    DEFF Research Database (Denmark)

    Bonde, Lars Ole

    2011-01-01

    Anmeldelse af Karl Aage Rasmussen: Musik i det tyvende århundrede: En fortælling. Gyldendal 2011.......Anmeldelse af Karl Aage Rasmussen: Musik i det tyvende århundrede: En fortælling. Gyldendal 2011....

  8. George Bush peab sõjalist jõudu viimaseks valikuks / George W. Bush ; interv. Kaarel Kaas

    Index Scriptorium Estoniae

    Bush, George W., 1946-

    2005-01-01

    Ilmunud ka: Põhjarannik 7. mai 2005, lk. 2, Severnoje Poberezhje 7. mai, 2005, lk. 2. USA president George Bush andis 5. mail Valges Majas intervjuu kuuele Euroopa ajakirjanikule. 9. mai pidustustest Moskvas, USA-Läti, Venemaa-Balti riikide suhetest, demokraatiast Venemaal, Ameerika Ühendriikide ning Baltimaade viisarežiimist, vabaduse mõistestst, kinnipeetavate kohtlemisest Guantanamo sõjaväebaasis, relvade kasutamise vajadusest, terrorismist. Vt. samas: Kuidas usutlus võimalikuks sai

  9. George Soros : maailma hädad sünnivad avatuse ja suletuse vastuoludest / George Soros ; interv. Urmet Kook

    Index Scriptorium Estoniae

    Soros, George, 1930-

    2004-01-01

    George Soros vastab küsimustele, kas postkommunistlikel riikidel oleks olnud mõttekas ja võimalik valida veidi pehmem tee turumajandusele üleminekul, kas ta usub liberaalse demokraatia lõplikku võidukäiku maailmas, millised on suurimad ohud tänapäeva maailma ees, samuti küsimustele demokraatlike riikide alliansi otstarbe ja mahajäänud ühiskondade jaluleaitamise kohta

  10. Fort Independence: An Eighteenth-Century Frontier Homesite and Militia Post in South Carolina.

    Science.gov (United States)

    1982-12-01

    included in this instance as a condiment , but it could also indicate that the Fort Independence garrison was familiar with the strategy employed by the Fort...archeological investigation of Fort Charlotte, McCormick County, South Carolina. Notebook, Institute of Archeology and Anthropology, University of South

  11. 40 CFR 81.63 - Metropolitan Fort Smith Interstate Air Quality Control Region.

    Science.gov (United States)

    2010-07-01

    ... 40 Protection of Environment 17 2010-07-01 2010-07-01 false Metropolitan Fort Smith Interstate Air... Air Quality Control Regions § 81.63 Metropolitan Fort Smith Interstate Air Quality Control Region. The Metropolitan Fort Smith Interstate Air Quality Control Region (Arkansas-Oklahoma) has been revised to consist...

  12. Case Study: Fort Mill High School--A Culture of Continuous Improvement

    Science.gov (United States)

    Southern Regional Education Board (SREB), 2014

    2014-01-01

    This is the latest in a series of case studies highlighting best practices High Schools That Work (HSTW) network schools and districts are implementing to prepare students better for further studies and careers. Fort Mill High School is in Fort Mill, South Carolina, an outlying suburb of Charlotte, North Carolina. Fort Mill links high quality…

  13. Fort Peck-Wolf Point transmission line project, Montana

    International Nuclear Information System (INIS)

    1992-01-01

    The primary objective of the project is to replace the existing 36-mile Fort Peck-Wolf Point transmission line which has reached the end of its useful service life. Presently, the overall condition of this existing section of the 47-year-old line is poor. Frequent repairs have been required because of the absence of overhead ground wires. The continued maintenance of the line will become more expensive and customer interruptions will persist because of the damage due to lightning. The expense of replacing shell rotted poles, and the concern for the safety of the maintenance personnel because of hazards caused by severe shell rot are also of primary importance. The operational and maintenance problems coupled with power system simulation studies, demonstrate the need for improvements to the Wolf Point area to serve area loads. Western's Wolf Point Substation is an important point of interconnection for the power output from the Fort Peck Dam to area loads as far away as Williston, North Dakota. The proposed transmission line replacement would assure that there will continue to be reliable transmission capacity available to serve area electrical loads, as well as provide a reliable second high-voltage transmission path from the Fort Peck generation to back-up a loss of the Fort Peck-Wolf Point 115-kV Line No. 1

  14. Safety and licensing analyses for the Fort St. Vrain HTGR

    International Nuclear Information System (INIS)

    Ball, S.J.; Conklin, J.C.; Harrington, R.M.; Cleveland, J.C.; Clapp, N.E. Jr.

    1982-01-01

    The Oak Ridge National Laboratory (ORNL) safety analysis program for the HTGR includes development and verification of system response simulation codes, and applications of these codes to specific Fort St. Vrain reactor licensing problems. Licensing studies addressed the oscillation problems and the concerns about large thermal stresses in the core support blocks during a postulated accident

  15. Site-Based Budgeting in Fort Worth, Texas.

    Science.gov (United States)

    Peternick, Lauri; Sherman, Joel

    1998-01-01

    Examines the Fort Worth Independent School District's decentralized decision-making system through three lenses: a review of site-based decision-making procedures at several schools; an examination of who participates; and stakeholders' perceptions. Some schools operated democratically, significantly including teachers, parents, and community…

  16. 22nd Spring Research Festival Showcases Fort Detrick Science | Poster

    Science.gov (United States)

    Rainy weather couldn’t dampen the spirits of visitors to the 2018 Spring Research Festival, which brought together scientists from the Frederick National Laboratory (FNL), NCI at Frederick, and the U.S. Army Medical Research and Materiel Command (USAMRMC) and showcased the important research that takes place every day at Fort Detrick.

  17. The health of loblolly pine stands at Fort Benning, GA

    Science.gov (United States)

    Soung-Ryoul Ryu; G. Geoff Wang; Joan L. Walker

    2013-01-01

    Approximately two-thirds of the red-cockaded woodpecker (Picoides borealis) (RCW) groups at Fort Benning, GA, depend on loblolly pine (Pinus taeda) stands for nesting or foraging. However, loblolly pine stands are suspected to decline. Forest managers want to replace loblolly pine with longleaf pine (P. palustris...

  18. Forest pathology and entomology at Fort Valley Experimental Forest

    Science.gov (United States)

    Brian W. Geils

    2008-01-01

    Forest pathology and entomology have been researched at Fort Valley Experimental Forest throughout its history. The pathogens and insects of particular interest are mistletoes, decay and canker fungi, rusts, bark beetles, and various defoliators. Studies on life history, biotic interactions, impacts, and control have been published and incorporated into silvicultural...

  19. Tile forts of the Liesbeeck Frontier | Sleigh | Scientia Militaria: South ...

    African Journals Online (AJOL)

    Scientia Militaria: South African Journal of Military Studies. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 27 (1997) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. Tile forts of the Liesbeeck Frontier.

  20. Opening remarks for the Fort Valley Centennial Celebration

    Science.gov (United States)

    G. Sam Foster

    2008-01-01

    The Rocky Mountain Research Station recognizes and values the contributions of our scientists and collaborators for their work over the past century at Fort Valley Experimental Forest. With the help of our partners and collaborators, Rocky Mountain Research Station is working to improve coordination across its research Program Areas and Experimental Forests and Ranges...

  1. Opening remarks for the Fort Valley Centennial Celebration (P-53)

    Science.gov (United States)

    G. Sam Foster

    2008-01-01

    The Rocky Mountain Research Station recognizes and values the contributions of our scientists and collaborators for their work over the past century at Fort Valley Experimental Forest. With the help of our partners and collaborators, Rocky Mountain Research Station is working to improve coordination across its research Program Areas and Experimental Forests and Ranges...

  2. Informal report on measurements of slant TEC by FORTE

    International Nuclear Information System (INIS)

    Massey, R.S.

    1997-01-01

    Los Alamos National Laboratory's Space and Atmospheric Sciences group is now operating the FORTE satellite, which has two sets of instruments: optical detectors and radio detectors. In this report the author describes work with one set of radio detectors that allow measurements of the total electron content (TEC) traversed by VHF radiation originating at an electromagnetic pulse (EMP) generator located at Los Alamos

  3. Assessment of DoD Wounded Warrior Matters -- Fort Riley

    Science.gov (United States)

    2013-08-06

    acceptable excuses included At Remote Care, Regular Leave, Maternity and Paternity Leave, Terminal Leave, Permanent Change of Station, and Transferred to...risk of negative medication interactions and reactions for Soldiers assigned to the Fort Riley WTB. B.2. Background The Joint Commission, an...reconciliation is to minimize medication errors such as omissions, duplications, dosing errors, and drug interactions . Medical reconciliation should

  4. The Forte Kreis : an Attempt to Spiritual Leadership over Europe

    NARCIS (Netherlands)

    Poorthuis, Marcel

    2017-01-01

    Just before the outbreak of World War 1, a group of writers, artists and philosophers decided to establish a spiritual rule over Europe, the Forte Kreis. The group aimed at a reconciliation in Europe, by establishing pacifism, but also between East and West by creating a new language. Their thoughts

  5. Early thinning experiments established by the Fort Valley Experimental Forest

    Science.gov (United States)

    Benjamin P. De Blois; Alex. J. Finkral; Andrew J. Sanchez Meador; Margaret M. Moore

    2008-01-01

    Between 1925 and 1936, the Fort Valley Experimental Forest (FVEF) scientists initiated a study to examine a series of forest thinning experiments in second growth ponderosa pine stands in Arizona and New Mexico. These early thinning plots furnished much of the early background for the development of methods used in forest management in the Southwest. The plots ranged...

  6. 77 FR 24579 - Establishment of the Fort Ord National Monument

    Science.gov (United States)

    2012-04-25

    ... 1775-1776, Anza established the first overland route from ``New Spain,'' as Mexico was then known, to..., approximately 6 miles of which pass through the Fort Ord area. Although much of the historic route currently... tourists and recreationalists from near and far, and enhance its unique natural resources, for the...

  7. Fort Valley studies: A natural laboratory for research and education

    Science.gov (United States)

    Brian W. Geils

    2008-01-01

    Drought, wildfire, extinction, and invasive species are considered serious threats to the health of our forests. Although these issues have global connections, we most readily see their consequences locally and attempt to respond with management based on science. For 100 years, the Fort Valley Experimental Forest (FVEF) has provided educational and experimental support...

  8. Calculation of Void in the Fort Saint Vrain Material

    Energy Technology Data Exchange (ETDEWEB)

    Potter, David Charles [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Taylor, Craig Michael [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Coons, James Elmer [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2017-05-11

    The percent void of the Fort Saint Vrain (FSV) material is estimated to be 21.1% based on the volume of the gap at the top of the drums, the volume of the coolant channels in the FSV fuel element, and the volume of the fuel handling channel in the FSV fuel element.

  9. George Gamow: a most talented and creative scientist

    International Nuclear Information System (INIS)

    Yang Qingyu

    2002-01-01

    As one of the most prominent scientists of the 20th century, George Gamow made notable contributions to many fields of science in his life time. In nuclear physics he proposed the well known 'nuclear potential barrier channel effect' and the Gamow-Teller transition probability of beta decay. In cosmology he did his almost to advocate the 'big bang' theory. Through extraordinary intuition he proposed a theory about how genetic codes work in molecular biology, which was later verified by Watson, Crick and Nirenbery. Gamow's unique style of research and his spirit of bringing forth new ideas in interdisciplinary subjects has greatly benefited scientific research

  10. George Herbert Mead: contributions to history of the social psychology

    OpenAIRE

    Souza, Renato Ferreira de

    2011-01-01

    Com este artigo pretende-se contribuir para a compreensão histórica de um autor/personagem da Psicologia. Analisamos e acrescemos conhecimento sobre George Herbert Mead e os desdobramentos de sua teoria psicossocial. Para esse propósito, explicitaremos, no texto, uma das vertentes analíticas utilizadas em nossa dissertação, qual seja: por meio da abordagem social em história da psicologia, confrontamos a vida de Mead com momentos de constituição da psicologia, colocando em relevo aspectos cen...

  11. George Herbert Mead on consciousness: antidote to Cartesian absurdities?

    DEFF Research Database (Denmark)

    Willert, Søren

    The article explicates George Herbert Mead's theory of consciousness as presented in Mind, Self and Society. According to Mead, the term consciousness may refer to three different sets of phenomena: (1) the environment as implied by our goal-directed action; Mead names this consciousness aspect...... experience; it is shared by humans and subhuman animals alike; (2) consciousness of environmental experience; Mead names this consciousness aspect awareness; it is exclusively human; (3) the peculiar sensed qualities attaching to consciousness, equalling what is today named qualia. Descartes......-inspired psychology makes the third consciousness aspect all-important. Within Mead's framework for a darwinistically inspired psycholgy, it becomes theoretically insignificant....

  12. Georg Trakl y la melancolía

    Directory of Open Access Journals (Sweden)

    Alejandro Peña-Arroyave

    2016-01-01

    Full Text Available Resumen: Se identificaron algunos aspectos determinantes de la obra poética de Georg Trakl, tomando como punto de partida la conciencia del escritor acerca de la muerte de Dios, desde la perspectiva de Nietzsche. Se hizo un énfasis sobre la melancolía, soporte de la creación, testimonio y reconocimiento del errar y el duelo del hombre, a partir de la cual el autor pudo expresar el desconsuelo por la ruina y el ocaso de Occidente.

  13. 'In front of your nose': the existentialism of George Orwell

    OpenAIRE

    Dulley, Paul Richard

    2015-01-01

    George Orwell’s reputation as a writer rests largely upon his final two works, selected essays and some of his journalism. As a novelist, he is often considered limited, and it is for this reason that his writing has perhaps received less serious attention than that of many of his contemporaries.\\ud \\ud Some recent publications have sought to redress this balance, identifying an impressive level of artistry, not only in his more recognised works, but in the neglected novels of the 1930s. Yet,...

  14. George Kelly: cognitive psychologist, humanistic psychologist, or something else entirely?

    Science.gov (United States)

    Benjafield, John G

    2008-11-01

    George Kelly was regarded by some of his contemporaries as a cognitive psychologist and by others as a humanistic psychologist. Kelly himself resisted being rubricized. He did, however, name several people who had been influential in his life and work, one of whom was J.F. Herbart. A comparison of Herbart and Kelly reveals several similarities. Both shared a belief that psychology was fundamentally a mathematical discipline. Both eliminated distinctions usually taken for granted in psychology, such as emotion versus cognition. Reconstructing Kelly's relation to Herbart allows one to see more clearly why Kelly was such a unique figure in 20th century psychology.

  15. Como Georg Simmel chegou à modernidade e lhe permaneceu fiel?

    Directory of Open Access Journals (Sweden)

    Otthein Rammstedt

    2015-04-01

    Full Text Available How did Georg Simmel, the “first sociologist of modernity” according to David Frisby, get to know and to conceive modernity? We believe that this happened during the phase in which the author participated in Naturalism – a social movement that had a strong impact on German society and culture. We argue that Simmel was strongly influenced by Naturalism, as many aspects of his theoretical and methodological ideas depend directly on the concept of modernity in the sense given to it by Naturalism.

  16. Morphological anomalies in two Lutzomyia (Psathyromyia) shannoni (Diptera: Psychodidae: Phlebotominae) specimens collected from Fort Rucker, Alabama, and Fort Campbell, Kentucky.

    Science.gov (United States)

    Florin, David A; Lawyer, Phillip; Rowton, Edgar; Schultz, George; Wilkerson, Richard; Davies, Stephen J; Lipnick, Robert; Keep, Lisa

    2010-09-01

    This report describes two male specimens of the sand fly species Lutzomyia shannoni (Dyar) (Diptera: Psychodidae: Phlebotominae) collected at Fort Rucker, AL, and Fort Campbell, KY, in dry ice-baited light traps during September 2005. The specimens were observed to have anomalies to the number of spines on the gonostyli. The taxonomic keys of Young and Perkins (Mosq. News 44: 263-285; 1984) use the number of spines on the gonostylus in the first couplet to differentiate two major groupings of North American sand flies. The two anomalous specimens were identified as L. shannoni based on the following criteria: (1) both specimens possess antennal ascoids with long, distinct proximal spurs (a near diagnostic character of L. shannoni in North America), (2) the sequences of the partial cytochrome c oxidase subunit 1 gene from both specimens indicated L. shannoni, and (3) the sequences of the internal transcribed spacer 2 molecular marker from both specimens indicated L. shannoni. The anomalous features are fundamentally different from each other as the Fort Rucker specimen possesses a fifth spine (basally located) on just one gonostylus, whereas the Fort Campbell specimen possesses five spines (extra spines subterminally located) on both gonostyli. Because the gonostyli are part of the external male genitalia, anomalies in the number of spines on the gonostyli may have serious biological consequences, such as reduced reproductive success, for the possessors. These anomalies are of taxonomic interest as the specimens could easily have been misidentified using available morphological keys.

  17. Tay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradation.

    Science.gov (United States)

    Dersh, Devin; Iwamoto, Yuichiro; Argon, Yair

    2016-12-01

    Loss of function of the enzyme β-hexosaminidase A (HexA) causes the lysosomal storage disorder Tay-Sachs disease (TSD). It has been proposed that mutations in the α chain of HexA can impair folding, enzyme assembly, and/or trafficking, yet there is surprisingly little known about the mechanisms of these potential routes of pathogenesis. We therefore investigated the biosynthesis and trafficking of TSD-associated HexA α mutants, seeking to identify relevant cellular quality control mechanisms. The α mutants E482K and G269S are defective in enzymatic activity, unprocessed by lysosomal proteases, and exhibit altered folding pathways compared with wild-type α. E482K is more severely misfolded than G269S, as observed by its aggregation and inability to associate with the HexA β chain. Importantly, both mutants are retrotranslocated from the endoplasmic reticulum (ER) to the cytosol and are degraded by the proteasome, indicating that they are cleared via ER-associated degradation (ERAD). Leveraging these discoveries, we observed that manipulating the cellular folding environment or ERAD pathways can alter the kinetics of mutant α degradation. Additionally, growth of patient fibroblasts at a permissive temperature or with chemical chaperones increases cellular Hex activity by improving mutant α folding. Therefore modulation of the ER quality control systems may be a potential therapeutic route for improving some forms of TSD. © 2016 Dersh et al. This article is distributed by The American Society for Cell Biology under license from the author(s). Two months after publication it is available to the public under an Attribution–Noncommercial–Share Alike 3.0 Unported Creative Commons License (http://creativecommons.org/licenses/by-nc-sa/3.0).

  18. Effect of cyclic, low dose pyrimethamine treatment in patients with Late Onset Tay Sachs: an open label, extended pilot study.

    Science.gov (United States)

    Osher, Etty; Fattal-Valevski, Aviva; Sagie, Liora; Urshanski, Nataly; Sagiv, Nadav; Peleg, Leah; Lerman-Sagie, Tally; Zimran, Ari; Elstein, Deborah; Navon, Ruth; Valevski, Avi; Stern, Naftali

    2015-04-17

    Late Onset Tay- Sachs disease (LOTS) is a rare neurodegenerative lysosomal storage disease which results from mutations in the gene encoding the α subunit (HEXA) of β-hexosaminidase enzyme (HexA). At the present time, no effective treatment exists for LOTS and other neurodegenerative diseases involving the central nerve system (CNS). Pyrimethamine (PMT) was previously shown to act as a HexA chaperone in human fibroblasts in vitro carrying some (e.g., αG269S), but not all LOTS-related mutations. The present study assessed the effect of cyclic, low dose and long term pyrimethamine treatment on HexA in subjects with LOTS. In an open label trial in 4 LOTS patients, PMT was initiated at an average daily dose of ~2.7 mg and administered cyclically guided by blood lymphocyte HexA activity for a mean duration of 82.8 (±22.5; SD) weeks (~1.5 year). HexA activity rose in all subjects, with a mean peak increase of 2.24 folds (±0.52; SD) over baseline activity (range 1.87-3). The mean treatment time required to attain this peak was of 15.7 (±4.8; SD) weeks. Following increase in activity, HexA gradually declined with the continued use of PMT, which was then stopped, resulting in the return of HexA activity to baseline. A second cycle of PMT treatment was then initiated, resulting again in an increase in HexA activity. Three of the patients experienced a measurable neuropsychiatric deterioration whereas one subject remained entirely stable. Cyclic low dose of PMT can increase HexA activity in LOTS patients. However, the observed increase is repeatedly transient and not associated with discernible beneficial neurological or psychiatric effects.

  19. A Drosophila protein family implicated in pheromone perception is related to Tay-Sachs GM2-activator protein.

    Science.gov (United States)

    Starostina, Elena; Xu, Aiguo; Lin, Heping; Pikielny, Claudio W

    2009-01-02

    Low volatility, lipid-like cuticular hydrocarbon pheromones produced by Drosophila melanogaster females play an essential role in triggering and modulating mating behavior, but the chemosensory mechanisms involved remain poorly understood. Recently, we showed that the CheB42a protein, which is expressed in only 10 pheromone-sensing taste hairs on the front legs of males, modulates progression to late stages of male courtship behavior in response to female-specific cuticular hydrocarbons. Here we report that expression of all 12 genes in the CheB gene family is predominantly or exclusively gustatory-specific, and occurs in many different, often non-overlapping patterns. Only the Gr family of gustatory receptor genes displays a comparable variety of gustatory-specific expression patterns. Unlike Grs, however, expression of all but one CheB gene is sexually dimorphic. Like CheB42a, other CheBs may therefore function specifically in gustatory perception of pheromones. We also show that CheBs belong to the ML superfamily of lipid-binding proteins, and are most similar to human GM2-activator protein (GM2-AP). In particular, GM2-AP residues involved in ligand binding are conserved in CheBs but not in other ML proteins. Finally, CheB42a is specifically secreted into the inner lumen of pheromone-sensing taste hairs, where pheromones interact with membrane-bound receptors. We propose that CheB proteins interact directly with lipid-like Drosophila pheromones and modulate their detection by the gustatory signal transduction machinery. Furthermore, as loss of GM2-AP in Tay-Sachs disease prevents degradation of GM2 gangliosides and results in neurodegeneration, the function of CheBs in pheromone response may involve biochemical mechanisms critical for lipid metabolism in human neurons.

  20. A Drosophila Protein Family Implicated in Pheromone Perception Is Related to Tay-Sachs GM2-Activator Protein*

    Science.gov (United States)

    Starostina, Elena; Xu, Aiguo; Lin, Heping; Pikielny, Claudio W.

    2009-01-01

    Low volatility, lipid-like cuticular hydrocarbon pheromones produced by Drosophila melanogaster females play an essential role in triggering and modulating mating behavior, but the chemosensory mechanisms involved remain poorly understood. Recently, we showed that the CheB42a protein, which is expressed in only 10 pheromone-sensing taste hairs on the front legs of males, modulates progression to late stages of male courtship behavior in response to female-specific cuticular hydrocarbons. Here we report that expression of all 12 genes in the CheB gene family is predominantly or exclusively gustatory-specific, and occurs in many different, often non-overlapping patterns. Only the Gr family of gustatory receptor genes displays a comparable variety of gustatory-specific expression patterns. Unlike Grs, however, expression of all but one CheB gene is sexually dimorphic. Like CheB42a, other CheBs may therefore function specifically in gustatory perception of pheromones. We also show that CheBs belong to the ML superfamily of lipid-binding proteins, and are most similar to human GM2-activator protein (GM2-AP). In particular, GM2-AP residues involved in ligand binding are conserved in CheBs but not in other ML proteins. Finally, CheB42a is specifically secreted into the inner lumen of pheromone-sensing taste hairs, where pheromones interact with membrane-bound receptors. We propose that CheB proteins interact directly with lipid-like Drosophila pheromones and modulate their detection by the gustatory signal transduction machinery. Furthermore, as loss of GM2-AP in Tay-Sachs disease prevents degradation of GM2 gangliosides and results in neurodegeneration, the function of CheBs in pheromone response may involve biochemical mechanisms critical for lipid metabolism in human neurons. PMID:18952610

  1. Evaluation of the risk for Tay-Sachs disease in individuals of French Canadian ancestry living in new England.

    Science.gov (United States)

    Martin, Dianna C; Mark, Brian L; Triggs-Raine, Barbara L; Natowicz, Marvin R

    2007-03-01

    The assessment of risk for Tay-Sachs disease (TSD) in individuals of French Canadian background living in New England is an important health issue. In preliminary studies of the enzyme-defined carrier frequency for TSD among Franco-Americans in New England, we found frequencies (1:53) higher than predicted from the incidence of infantile TSD in this region. We have now further evaluated the risk for TSD in the Franco-American population of New England. Using a fluorescence-based assay for beta-hexosaminidase activity, we determined the carrier frequencies for TSD in 2783 Franco-Americans. DNA analysis was used to identify mutations causing enzyme deficiency in TSD carriers. We determined the enzyme-defined carrier frequency for TSD as 1:65 (95% confidence interval 1:49 to 1:90). DNA-based analysis of 24 of the enzyme-defined carriers revealed 21 with sequence changes: 9 disease-causing, 4 benign, and 8 of unknown significance. Six of the unknowns were identified as c.748G>A p.G250S, a mutation we show by expression analysis to behave similarly to the previously described c.805G>A p.G269S adult-onset TSD mutation. This putative adult-onset TSD c.748G>A p.G250S mutation has a population frequency similar to the common 7.6 kb deletion mutation that occurs in persons of French Canadian ancestry. We estimate the frequency of deleterious TSD alleles in Franco-Americans to be 1:73 (95% confidence interval 1:55 to 1:107). These data provide a more complete data base from which to formulate policy recommendations regarding TSD heterozygosity screening in individuals of French Canadian background.

  2. Fermat Potentials of Embedded Lensing, the Integrated Sachs-Wolfe Effect, and Weak-Lensing of CMB by Cosmic Voids

    Science.gov (United States)

    Chen, Bin; Kantowski, R.; Dai, X.

    2014-01-01

    We have developed an accurate gravitational lens theory for an inhomogeneity embedded in an otherwise homogeneous universe, which to the lowest order is applicable to any mass distribution. We derive the Fermat potential for a spherically symmetric lens embedded in a FLRW cosmology and use it to investigate the late-time integrated Sachs-Wolfe effect (ISW) caused by individual large scale inhomogeneities, in particular, cosmic voids. We present a simple analytical expression for the CMB temperature fluctuation across such a lens as the derivative of the lens Fermat potential. Our formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. Our results are particularly useful for modeling ISW effects extracted through stacking large numbers of cosmic voids and clusters (that is, the aperture photometry method). For structures co-expanding with the background cosmology, i.e., for time-independent density contrasts, we find that the gravitational lensing time delay alone can produce fluctuations of the order of seen in recent observations by WMAP and Planck. We revisit the possibility of explaining the non-Gaussian cold spot on the south hemisphere via the Rees-Sciama effect of a large cosmic void using constraints obtained from the most recent void catalogs and our new void-lensing formalism, and compare it with other explanations such as a collapsing cosmic texture. We also study the remapping of primordial CMB anisotropies, the weak-lensing shear, and magnification caused by void lensing.

  3. Traumatic glenohumeral bone defects and their relationship to failure of arthroscopic Bankart repairs: significance of the inverted-pear glenoid and the humeral engaging Hill-Sachs lesion.

    Science.gov (United States)

    Burkhart, S S; De Beer, J F

    2000-10-01

    Our goal was to analyze the results of 194 consecutive arthroscopic Bankart repairs (performed by 2 surgeons with an identical suture anchor technique) in order to identify specific factors related to recurrence of instability. Case series. We analyzed 194 consecutive arthroscopic Bankart repairs by suture anchor technique performed for traumatic anterior-inferior instability. The average follow-up was 27 months (range, 14 to 79 months). There were 101 contact athletes (96 South African rugby players and 5 American football players). We identified significant bone defects on either the humerus or the glenoid as (1) "inverted-pear" glenoid, in which the normally pear-shaped glenoid had lost enough anterior-inferior bone to assume the shape of an inverted pear; or (2) "engaging" Hill-Sachs lesion of the humerus, in which the orientation of the Hill-Sachs lesion was such that it engaged the anterior glenoid with the shoulder in abduction and external rotation. There were 21 recurrent dislocations and subluxations (14 dislocations, 7 subluxations). Of those 21 shoulders with recurrent instability, 14 had significant bone defects (3 engaging Hill-Sachs and 11 inverted-pear Bankart lesions). For the group of patients without significant bone defects (173 shoulders), there were 7 recurrences (4% recurrence rate). For the group with significant bone defects (21 patients), there were 14 recurrences (67% recurrence rate). For contact athletes without significant bone defects, there was a 6.5% recurrence rate, whereas for contact athletes with significant bone defects, there was an 89% recurrence rate. (1) Arthroscopic Bankart repairs give results equal to open Bankart repairs if there are no significant structural bone deficits (engaging Hill-Sachs or inverted-pear Bankart lesions). (2) Patients with significant bone deficits as defined in this study are not candidates for arthroscopic Bankart repair. (3) Contact athletes without structural bone deficits may be treated by

  4. Georges Bataille and the Transgression of Taboos by Stephan Dedalus

    Directory of Open Access Journals (Sweden)

    Shataw Naseri

    2014-07-01

    Full Text Available James Joyce's A Portrait of the Artist as a Young Man is not a pornographic novel like Georges Bataille' Story of the Eye; however, the present study attempts to trace Bataille's heterological notions in A Portrait. This paper attempts to see whether or not Stephan Dedalus as the main character of Joyce's literary work, has the potential to carry Batatille's heterogeneous elements which shatter any religious, linguistic, economic and political system. In order to reach this goal, the paper first attempts to establish the powerful dominance of the Catholic discourse, one of the strictest religious systems in the world, over Ireland which is the main setting of A Portrait. In the next part it will trace the authoritarian shadow of Catholicism and its impression upon Stephen Dedalus. Here, the principal aim is to indicate the significance of Stephen's transgressive acts in the novel and to see whether these transgressions have Bataillean nature as they shatter a very strict religious structure. Afterward, Stephen's transgressions as well as the implicit reaction of the religious system toward these transgressions will be investigated regarding Georges Bataille's heterogeneous notions.

  5. Master Environmental Plan: Fort Wingate Depot Activity, Gallup, New Mexico

    Energy Technology Data Exchange (ETDEWEB)

    Biang, C.A.; Yuen, C.R.; Biang, R.P.; Antonopoulos, A.A.; Ditmars, J.D.

    1990-12-01

    The master environmental plan is based on an environmental assessment of the areas requiring environmental evaluation (AREEs) at Fort Wingate Depot Activity near Gallup, New Mexico. The Fort Wingate Depot Activity is slated for closure under the Base Closure and Realignment Act, Public Law 100--526. The MEP assesses the current status, describes additional data requirements, recommends actions for the sites, and establishes a priority order for actions. The plan was developed so that actions comply with hazardous waste and water quality regulations of the State of New Mexico and applicable federal regulations. It contains a brief history of the site, relevant geological and hydrological information, and a description of the current status for each AREE along with a discussion of the available site-specific data that pertain to existing or potential contamination and the impact on the environment. 35 refs., 27 figs., 23 tabs.

  6. Miglustat in late-onset Tay-Sachs disease: a 12-month, randomized, controlled clinical study with 24 months of extended treatment.

    Science.gov (United States)

    Shapiro, Barbara E; Pastores, Gregory M; Gianutsos, John; Luzy, Cécile; Kolodny, Edwin H

    2009-06-01

    To evaluate the safety and efficacy of miglustat in patients with GM2 gangliosidosis. A randomized, multicenter, open-label, 12-month study involving patients aged 18 years or older, randomized 2:1 to miglustat (200 mg TID) or "no miglustat treatment." This study was followed by 24 months of extended treatment during which all patients received miglustat. Primary efficacy endpoints were change in eight measures of isometric muscle strength in the limbs and isometric grip strength, evaluated at baseline, and months 12 and 36. Secondary efficacy endpoints included gait, balance, disability, and other neurological assessments. Safety evaluations included adverse event reporting. Thirty patients (67% male, age range 18-56 years) with late-onset Tay-Sachs disease were enrolled; 20 were randomized to miglustat and 10 to "no miglustat treatment." Muscle and grip strength generally decreased over the study period. No differences were observed between the two groups in any efficacy measure, either during the 12-month randomized phase or the full 36 months. The most common treatment-related adverse events were decrease in weight and diarrhea. Miglustat treatment was not shown to lead to measurable benefits in this cohort of patients with late-onset Tay-Sachs disease. The observed safety profile was consistent with that of the approved dose (100 mg TID) in type 1 Gaucher disease.

  7. In silico analyses of essential interactions of iminosugars with the Hex A active site and evaluation of their pharmacological chaperone effects for Tay-Sachs disease.

    Science.gov (United States)

    Kato, Atsushi; Nakagome, Izumi; Nakagawa, Shinpei; Kinami, Kyoko; Adachi, Isao; Jenkinson, Sarah F; Désiré, Jérôme; Blériot, Yves; Nash, Robert J; Fleet, George W J; Hirono, Shuichi

    2017-11-15

    The affinity of a series of iminosugar-based inhibitors exhibiting various ring sizes toward Hex A and their essential interactions with the enzyme active site were investigated. All the Hex A-inhibiting iminosugars tested formed hydrogen bonds with Arg178, Asp322, Tyr421 and Glu462 and had the favorable cation-π interaction with Trp460. Among them, DMDP amide (6) proved to be the most potent competitive inhibitor with a K i value of 0.041 μM. We analyzed the dynamic properties of both DMDP amide (6) and DNJNAc (1) in aqueous solution using molecular dynamics (MD) calculations; the distance of the interaction between Asp322 and 3-OH and Glu323 and 6-OH was important for stable interactions with Hex A, reducing fluctuations in the plasticity of the active site. DMDP amide (6) dose-dependently increased intracellular Hex A activity in the G269S mutant cells and restored Hex A activity up to approximately 43% of the wild type level; this effect clearly exceeded the border line treatment for Tay-Sachs disease, which is regarded as 10-15% of the wild type level. This is a significantly greater effect than that of pyrimethamine, which is currently in Phase 2 clinical trials. DMDP amide (6), therefore, represents a new promising pharmacological chaperone candidate for the treatment of Tay-Sachs disease.

  8. Fort St. Vrain improvement program plan. Draft final report

    International Nuclear Information System (INIS)

    1980-03-01

    The restraints are described which inhibit the Fort St. Vrain (FSV) Nuclear Power Station, a high temperature gas cooled reactor (HTGR) plant, from achieving full power operation with high availability. The actions necessary to overcome these restraints are outlined. The restraints originated from problems in both hardware related and institutional areas. The report summarizes what has been accomplished, what is currently being done, and what should be done to resolve the problems

  9. Bastion on the Border: Fort Bliss, 1854-1943

    Science.gov (United States)

    1993-01-01

    Journals , Record Group (RG) 165, Records of the War Department General and Special Staffs, National Archives, Washington, D.C. "’Richard Estrada, Border...it was the Fort Bliss garrison and the other troops deployed by Steever in the El Paso Patrol District that would have to provide the figurative glue ...little military value. For example, on March 30 three carloads of oats, flour , corn, and hay were dispatched; on April 7 fourteen carloads of hay, gasoline

  10. Basewide Energy Study, Fort Wainwright Alaska: Volume 1-Executive Summary

    Science.gov (United States)

    1982-04-01

    more accurate condensate wiett?Ing. 2.2 ENERGY OSAGE ANALISIS 4 top~down anay2ts was mad" of FY’•0 ener;’ uxage’ t Fort wrtsvrigFnt. The spporiIonments...vice, at each receptacle cluster . It should be thermally sensitlve. rtdtcing through-put from 600 watts at -SOOT to0soer power at 100? outside air

  11. Die opleiding van bedryfsielkundiges aan die universiteit van Fort Hare

    Directory of Open Access Journals (Sweden)

    W. Botha

    1977-11-01

    Full Text Available Die Departement Bedryfsielkunde aan die Universiteit van Fort Hare is 'n relatiewe jong departement en het eers in 1965 tot stand gekom. Voor hierdie datum is Bedryfsielkunde as 'n kort kursus deur die departement van suiwer Sielkunde aangebied en een van die destydse dosente, Dr. W. Backer, het die inisiatief geneem om 'n selfstandige departement van Bedryfsielkunde in die Fakulteit van Ekonomiese Wetenskappe op die been te bring.

  12. Assessment of DOD Wounded Warrior Matters -- Fort Drum

    Science.gov (United States)

    2011-09-30

    their relation to military duties. The six factors that are evaluated are: physical capacity or stamina , upper extremities, lower extremities...Health Net Federal Services contractor. The Fort Drum MEDDAC Referral Management Office created a “Reports Cell ” which was responsible for obtaining...Care Division had created a CLR/Reports Cell group that focused specifically on obtaining CLRs, inputting them into patients’ AHLTA records and

  13. Leaktightness in HTGRs - experience at Fort St. Vrain

    International Nuclear Information System (INIS)

    Neylan, A.J.; Barker, R.A.; Deardorff, A.F.

    1976-01-01

    The Fort St. Vrain Prestressed Concrete Reactor Vessel is the first utilized to contain the helium coolant of a High Temperature Gas-Cooled Reactor. Because the helium coolant contains fission products, leakage from the vessel is limited to 15 percent of vessel inventory per year. This paper describes the fabrication methods and development tests used to assure this leaktightness and the leakage test conducted to verify it. (author)

  14. Neutron flux distribution measurement in the Fort St. Vrain initial core (results of Fort St. Vrain start-up test A-7)

    International Nuclear Information System (INIS)

    Marshall, A.C.; Brown, J.R.

    1975-01-01

    A description is given of a test to measure the axial flux distribution at several radial locations in the Fort St. Vrain core representing unrodded, rodded, and partially rodded regions. The measurements were intended to verify the calculational accuracy of the three-dimensional calculational model used to compute axial power distributions for the Fort St. Vrain core. (U.S.)

  15. Liquid radwaste processing history at Fort Calhoun Nuclear Station

    International Nuclear Information System (INIS)

    Bilau, A.; Rutar, F.

    1989-01-01

    This report presents a historical perspective of liquid radwaste processing at the Fort Calhoun Unit 1 Nuclear Power Station, located in east central Nebraska. Of particular interest is the textual and graphical comparison of the operational implications of the various waste processing methods employed in the last ten years at the Fort Calhoun Station. Fort Calhoun's waste collection and processing systems are described in detail. These process systems include evaporation and solidification employing an in-plant drum solidification system. This solidification system was later replaced with vendor solidification services which solidified wastes in large liners. Ultimately, the plant converted its processing operation to ion exchange cleanup using ion selective media. The operational and economic impact of each of these process systems is discussed including overall costs, personnel exposure, capital expenditure requirements, burial volumes generated, maintenance and reliability assessments. Operational goals and performance criteria employed in the decision-making process for selection of the optimal technology are discussed, including the impact of various influent and effluent requirements

  16. BRAND EQUITY OF LAHORE FORT AS A TOURISM DESTINATION BRAND

    Directory of Open Access Journals (Sweden)

    Muhammad Kashif

    2015-06-01

    Full Text Available Studies that measure the brand equity of destination brands by using the Customer-Based Brand Equity (CBBE model in a developing country context are scarce. The present study investigates the destination brand equity of the Lahore Fort by employing the CBBE model in a developing country context of Pakistan. Following the positivist tradition, we adopted a survey-based approach to collect data from 237 tourists visiting the Lahore Fort. Data were collected through a questionnaire developed to explain the relationship of brand awareness, brand image, brand association, and brand loyalty with Lahore Fort’s overall brand equity. We used various robust statistical techniques such as correlation, regression and confirmatory factor analysis (using PLS method to reach meaningful conclusions and found that brand image and brand associations positively contribute to brand loyalty. Furthermore, brand loyalty significantly contributes towards overall brand equity. Pragmatically, this study measures the customer based brand equity of the Lahore Fort, a destination brand. The results are useful as they suggest a few strategies that can help policy makers to enhance Lahore Fort’s brand performance.

  17. National Training Center Fort Irwin expansion area aquatic resources survey

    Energy Technology Data Exchange (ETDEWEB)

    Cushing, C.E.; Mueller, R.P.

    1996-02-01

    Biologists from Pacific Northwest National Laboratory (PNNL) were requested by personnel from Fort Irwin to conduct a biological reconnaissance of the Avawatz Mountains northeast of Fort Irwin, an area for proposed expansion of the Fort. Surveys of vegetation, small mammals, birds, reptiles, amphibians, and aquatic resources were conducted during 1995 to characterize the populations and habitats present with emphasis on determining the presence of any species of special concern. This report presents a description of the sites sampled, a list of the organisms found and identified, and a discussion of relative abundance. Taxonomic identifications were done to the lowest level possible commensurate with determining the status of the taxa relative to its possible listing as a threatened, endangered, or candidate species. Consultation with taxonomic experts was undertaken for the Coleoptera ahd Hemiptera. In addition to listing the macroinvertebrates found, the authors also present a discussion related to the possible presence of any threatened or endangered species or species of concern found in Sheep Creek Springs, Tin Cabin Springs, and the Amargosa River.

  18. George Kubler Shifting South: Architecture History Following Geopolitics

    Directory of Open Access Journals (Sweden)

    Sousa Santos, Eliana

    2016-07-01

    Full Text Available This essay aims to present the shifting relations between North America and the South - South America and the South of Europe - through the work of the historian George Kubler. At the beginning of his career as a scholar, Kubler was invited by the Department of State to participate in a conference on inter-American relations. Later, with the positioning of the United States in World War II, the transatlantic relationship between the US and Europe became more prominent. Kubler’s research interests followed this change: His research shift from South America to peripheral Southern Europe reflects an availability of funding given his country's geopolitical interests. So far, artists and other scholars have praised Kubler’s vast work regarding the art and architecture of different 'Souths' mainly as a sign of 'nonalignment' and of his attention to the condition of peripheral countries.

  19. GEORGE HERBERT MEAD'IN SOSYAL AHLAK ANLAYIŞI

    OpenAIRE

    Mustafa Kınağ

    2017-01-01

    Klasik Amerikan pragmatistlerinden George Herbert Mead’a göre benlik, ne salt zihinden ibarettir, ne de ontolojik çatallaşmayı içerir. O, bunun yerine sadece analitik bir ayrım olarak benliği (self) özne benlik (I) ve nesne benlik (me) olarak ifade eder. Ancak ne özne benliği nesne benlik olmaksızın, ne de nesne benliği özne benlik olmaksızın düşünebiliriz. Özne benlik, doğrudan deneyimlerimizde kendisini göstermez, eylemin gerçekleşmesinden sonra biliş alanına girer. O, haf...

  20. [George Herbert Mead. Thought as the conversation of interior gestures].

    Science.gov (United States)

    Quéré, Louis

    2010-01-01

    For George Herbert Mead, thinking amounts to holding an "inner conversation of gestures ". Such a conception does not seem especially original at first glance. What makes it truly original is the "social-behavioral" approach of which it is a part, and, particularly, two ideas. The first is that the conversation in question is a conversation of gestures or attitudes, and the second, that thought and reflexive intelligence arise from the internalization of an external process supported by the social mechanism of communication: that of conduct organization. It imports then to understand what distinguishes such ideas from those of the founder of behavioral psychology, John B. Watson, for whom thinking amounts to nothing other than subvocal speech.

  1. Georges Bank drilling moratorium gets extended until 2015

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    2010-07-15

    A moratorium on oil and gas exploration and drilling on the Georges Bank will continue until the end of 2015. Governments require a full public review and research concerning the potential environmental impacts of the project before lifting the moratorium. Canada's federal and provincial governments have agreed to gather information related to the region's fragile ecosystem, and investigate the potential impacts that petroleum-related activities may have in the area. Preliminary reviews are being conducted to study the socio-economic impacts of offshore petroleum activities and to assess new technologies and drilling practices that may be used to reduce the impacts of oil and gas operations. 1 fig.

  2. Georges Lemaître Life, Science and Legacy

    CERN Document Server

    Mitton, Simon

    2012-01-01

    The year 2011 marked the 80th anniversary of Georges Lemaître’s primeval atom model of the universe, forerunner of the modern day Big Bang theory. Prompted by this momentous anniversary the Royal Astronomical Society decided to publish a volume of essays on the life, work and faith of this great cosmologist, who was also a Roman Catholic priest. The papers presented in this book examine in detail the historical, cosmological, philosophical and theological issues surrounding the development of the Big Bang theory from its beginnings in the pioneering work of Lemaître through to the modern day. This book offers the best account in English of Lemaître’s life and work. It will be appreciated by professionals and graduate students interested in the history of cosmology.

  3. Un monochrome poétique de Georges Fourest

    OpenAIRE

    Robert, Laurent

    2007-01-01

    Cet article propose une lecture du « Pseudo-sonnet que les amateurs de plaisanterie facile proclameront le plus beau du recueil » extrait de La Négresse blonde (1909) de Georges Fourest. La démarche de Fourest est mise en relation à la fois avec celle des peintures «monochroïdales » d’Alphonse Allais et avec celle du « sonnet en yx » de Stéphane Mallarmé. Elle est finalement perçue dans toute son ambiguïté, entre la mise en cause radicale de la poésie et la facétie fin de siècle. Peer revi...

  4. The botanical activities of George Edward Post (1838-1909).

    Science.gov (United States)

    Musselman, Lytton John

    2006-01-01

    George Edward Post wrote the first flora of the Middle East in English. His other botanical activities are less familiar. In addition to the flora, this paper discusses his teaching, fieldwork, contribution to Bible dictionaries, relations with the Boissier Herbarium in Geneva, establishment of the herbarium, and letters. Those letters are used here for the first time. Post corresponded with botanical luminaries of his day including Autran, Baker, Balfour, Barbey, Boissier, Bornmüller, Carruthers, Denslow, Haussknecht, Hooker, Schweinfurth, Thistleton-Dyer, Torrey, and Winkler. His long-term relationship with the herbarium at Geneva is highlighted. In addition, some of the lesser understood aspects of his life including chaplaincy during the American Civil War, and missionary to Syria are discussed.

  5. Georges Lemaître: Science and Religion

    Science.gov (United States)

    Coyne, George V.

    In order to appreciate the contribution which Georges Lemaître made to the relationship between religion and science it is necessary to understand how the Catholic Church, of which he was a priest, passed in the course of three centuries, from a position of conflict with the sciences to one of compatible openness and dialogue. In doing this I hope to show that the natural sciences have played a significant role in helping to establish the kind of dialogue that is absolutely necessary for the enrichment of the multifaceted aspects of human culture. I will speak of the following four periods of history: (l) the rise of modern atheism in the seventeenth and eighteenth centuries; (2) anticlericalism in Europe in the nineteenth century; (3) the awakening within the Catholic Church to modern science in the first six decades of the twentieth century; (4) the Church's view today.

  6. Rethinking the Institutional Theory of George Dickie: The Art Circles

    Directory of Open Access Journals (Sweden)

    Marko Đorđević

    2016-04-01

    Full Text Available This paper initially revisits the institutional theory of George Dickie, which originated in relation to Arthur Danto’s “The Artworld” and the works of other analytic philosophers dealing with art theory at the time. This contextualization is followed by an attempt at finding possible relations and theoretical developments of Dickie’s work in theorists such as Howard S. Becker and Pierre Bourdieu. The aim is to find basic correlations and differences between Dickie’s theory, Becker’s symbolic interactionism and Bourdieu’s field theory. The final segment consists of linking the interpreted theoretical terms and summing up the possibilities that arise out of them for contemporary art theory.

  7. An aerial radiological survey of the Fort Calhoun Nuclear Power Plant and surrounding area, Fort Calhoun, Nebraska

    International Nuclear Information System (INIS)

    1994-05-01

    An aerial radiological survey was conducted over the Fort Calhoun Nuclear Power Plant in Fort Calhoun, Nebraska, during the period June 19 through June 28, 1993. The survey was conducted at an altitude of 150 feet (46 meters) over a 25-square-mile (65-square-kilometer) area centered on the power station. The purpose of the survey was to document the terrestrial gamma radiation environment of the Fort Calhoun Nuclear Power Plant and surrounding area. The results of the aerial survey are reported as inferred gamma radiation exposure rates at 1 meter above ground level in the form of a contour map. Outside the plant boundary, exposure rates were found to vary between 6 and 12 microroentgens per hour and were attributed to naturally-occurring uranium, thorium, and potassium. The aerial data were compared to ground-based benchmark exposure rate measurements and radionuclide assays of soil samples obtained within the survey boundary. The ground-based measurements were found to be in good agreement with those inferred from the aerial measuring system. A previous survey was conducted on August 9 and 10, 1972, before the plant began operation. Exposure rates measured in both surveys were consistent with normal terrestrial background

  8. The geology and mechanics of formation of the Fort Rock Dome, Yavapai County, Arizona

    Science.gov (United States)

    Fuis, Gary S.

    1996-01-01

    The Fort Rock Dome, a craterlike structure in northern Arizona, is the erosional product of a circular domal uplift associated with a Precambrian shear zone exposed within the crater and with Tertiary volcanism. A section of Precambrian to Quaternary rocks is described, and two Tertiary units, the Crater Pasture Formation and the Fort Rock Creek Rhyodacite, are named. A mathematical model of the doming process is developed that is consistent with the history of the Fort Rock Dome.

  9. Analysis of Delayed Sea Breeze Onset for Fort Ord Prescribed Burning Operations

    Science.gov (United States)

    2015-12-01

    DELAYED SEA BREEZE ONSET FOR FORT ORD PRESCRIBED BURNING OPERATIONS by Dustin D. Hocking December 2015 Thesis Advisor: Wendell Nuss Second...AND DATES COVERED Master’s thesis 4. TITLE AND SUBTITLE ANALYSIS OF DELAYED SEA BREEZE ONSET FOR FORT ORD PRESCRIBED BURNING OPERATIONS 5...release; distribution is unlimited 12b. DISTRIBUTION CODE 13. ABSTRACT (maximum 200 words) The U.S. Army conducts prescribed burns at Fort Ord

  10. St George the dragon-slayer at Prague Castle - the eternal pilgrim without a home?

    Czech Academy of Sciences Publication Activity Database

    Benešovská, Klára

    2007-01-01

    Roč. 55, č. 1 (2007), s. 28-39 ISSN 0049-5123 Institutional research plan: CEZ:AV0Z80330511 Keywords : Gothic bronze sculpture * St George the dragon -slayer * Martin and George of Cluj * Prague Castle Subject RIV: AL - Art, Architecture, Cultural Heritage http://www.umeni-art.cz/cz/soubory/benesovska.pdf

  11. "Georg" : za 13 sjomotshnõhh dnei do filma / Marko Matvere ; intervjueerinud Boris Tuch

    Index Scriptorium Estoniae

    Matvere, Marko, 1968-

    2006-01-01

    Peeter Simmi mängufilmi "Georg" võtteperioodi lõpuni on jäänud loetud päevad. Filmist ja oma tegelaskujust räägivad näitlejad Marko Matvere, Anastasia Makejeva (artiklis ekslikult Matvejeva), laulja Renars Kaupers. Filmi stsenarist Mati Põldre räägib Georg Otsa ja KGB suhetest

  12. Prescient … and Not Always Accounted For: The Wisdom and Foresight of George A. Roeper

    Science.gov (United States)

    Delisle, Jim; Ruff, Marcia

    2016-01-01

    When George A. Roeper enlisted the aid of Dr. A. Harry Passow and other leaders in the field to help George convert his Michigan independent school to focus on gifted children in 1956, there was only one other elementary school in the country at the time devoted exclusively to gifted child education. Though his wife and school cofounder,…

  13. Automated Environmental Data Collection at Fort Benning, Georgia, from May 1999 to July 2001

    National Research Council Canada - National Science Library

    Hahn, Charles

    2002-01-01

    The Department of Defense, Strategic Environmental Research and Development Program, Ecosystem Management Project, Ecosystem Characterization and Monitoring initiative Program at Fort Benning, Georgia...

  14. Tay-Sachs Disease

    Science.gov (United States)

    ... better understanding of how neurological deficits arise in lipid storage diseases and on the development of new treatments targeting disease mechanisms. Specific research on the gangliodisoses including expanding the use of ...

  15. Tay-Sachs Disease

    Science.gov (United States)

    ... During the next months — or even years — the baby will progressively lose the ability to see, hear, and move. A red spot will develop in the back of the child's eyes. The child will stop smiling, crawling, turning over, and reaching out for things. By ...

  16. ALARA and decommissioning: The Fort St. Vrain experience

    Energy Technology Data Exchange (ETDEWEB)

    Borst, T.; Niehoff, M. [Public Service Co. of Colorado, Platteville, CO (United States); Zachary, M. [Scientific Ecology Group, Platteville, CO (United States)

    1995-03-01

    The Fort St. Vrain Nuclear Generating Station, the first and only commercial High Temperature Gas Cooled Reactor to operate in the United States, completed initial fuel loading in late 1973 and initial startup in early 1974. Due to a series of non-nuclear technical problems, Fort St. Vrain never operated consistently, attaining a lifetime capacity factor of slightly less than 15%. In August of 1989, the decision was made to permanently shut down the plant due to control rod drive and steam generator ring header failures. Public Service Company of Colorado elected to proceed with early dismantlement (DECON) as opposed to SAFSTOR on the bases of perceived societal benefits, rad waste, and exposure considerations, regulatory uncertainties associated with SAFSTOR, and cost. The decommissioning of Fort St. Vrain began in August of 1992, and is scheduled to be completed in early 1996. Decommissioning is being conducted by a team consisting of Westinghouse, MK-Ferguson, and Scientific Ecology Group. Public Service Company of Colorado as the licensee provides contract management and oversight of contractor functions. An aggressive program to maintain project radiation exposures As Low As Reasonably Achievable (ALARA) has been established, with the following program elements: temporary and permanent shielding contamination control; mockup training; engineering controls; worker awareness; integrated work package reviews communication; special instrumentation; video camera usage; robotics application; and project committees. To date, worker exposures have been less than project estimates. from the start of the project through Februrary of 1994, total exposure has been 98.666 person-rem, compared to the project estimate of 433 person-rem and goal of 347 person-rem. The presentation will discuss the site characterization efforts, the radiological performance indicator program, and the final site release survey plans.

  17. Study of neutral composition of lower thermosphere at Fort Churchill.

    Science.gov (United States)

    Nier, A. O.; Hickman, D. R.

    1973-01-01

    On Feb. 4 and 6, 1969, and May 11, 1970, Aerobee rockets carrying neutral mass spectrometers were flown at Fort Churchill, Canada during conditions of low geomagnetic activity. As in earlier flights at White Sands, New Mexico, each rocket carried both 'open' and 'closed' ion source instruments. Vertical profiles of N2, O2, O, Ar, and He were measured. Results obtained were essentially the same as those observed at White Sands except that for the winter flights helium appeared to be in diffusive equilibrium.

  18. Archeological Testing Fort Hood: 1994-1995. Volume 2

    Science.gov (United States)

    1996-10-01

    ASSOCI TES, INC. (662-22) Archeological Testing at Fort Hood. 1994-199.5 569 -48-1941.1080-134 1935 -058 Figure 7.17 Selected Perforator Types: Awl and...Department of Anthropology, University of Arkansas. Huskey, V. 1935 An Archeological Survey of the Nueces Canyon of Texas, Bulletin of the Texas... epr 064lL.Tan I lms expected 08-FH1 Yellow 4 expected expedctd cd .9 15.Q W n• I less M 0 ~ *~Tax~on Total Total Inmr 53 nac na Vertebra.es 1. FcAuifnm

  19. A reactivity accidents simulation of the Fort Saint Vrain HTGR

    International Nuclear Information System (INIS)

    Fainer, Gerson

    1980-01-01

    A reactivity accidents analysis of the Fort Saint Vrain HTGR was made. The following accidents were analysed 1) A rod pair withdrawal accident during normal operation, 2) A rod pair ejection accident, 3) A rod pair withdrawal accident during startup operations at source levels and 4) Multiple rod pair withdrawal accident. All the simulations were performed by using the BLOOST-6 nuclear code The steady state reactor operation results obtained with the code were consistent with the design reactor data. The numerical analysis showed that all accidents - except the first one - cause particle failure. (author)

  20. Dynamic computer simulation of the Fort St. Vrain steam turbines

    International Nuclear Information System (INIS)

    Conklin, J.C.

    1983-01-01

    A computer simulation is described for the dynamic response of the Fort St. Vrain nuclear reactor regenerative intermediate- and low-pressure steam turbines. The fundamental computer-modeling assumptions for the turbines and feedwater heaters are developed. A turbine heat balance specifying steam and feedwater conditions at a given generator load and the volumes of the feedwater heaters are all that are necessary as descriptive input parameters. Actual plant data for a generator load reduction from 100 to 50% power (which occurred as part of a plant transient on November 9, 1981) are compared with computer-generated predictions, with reasonably good agreement

  1. Construction, testing, and initial operation of Fort St. Vrain PCRV

    International Nuclear Information System (INIS)

    Ople, F.S. Jr.; Neylan, A.J.

    1975-01-01

    The Fort St. Vrain (FSV) Nuclear Generating Station is the first station in the USA to use a prestressed concrete reactor vessel (PCRV). The PCRV was designed and constructed by General Atomic. Construction of the PCRV was completed in 1970; the pressure and leak tests were completed in 1971. The structural behavior of the PCRV has been monitored by installed instrumentation since start of construction. The highlights of the actual construction, testing, and initial operation of the PCRV, including a comparison of structural behavior, where possible, between observed data and analytical predictions. (U.S.)

  2. Fort Carson Building 1860 Biomass Heating Analysis Report

    Energy Technology Data Exchange (ETDEWEB)

    Hunsberger, Randolph [National Renewable Energy Lab. (NREL), Golden, CO (United States); Tomberlin, Gregg [National Renewable Energy Lab. (NREL), Golden, CO (United States); Gaul, Chris [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2015-09-01

    As part of the Army Net-Zero Energy Installation program, the Fort Carson Army Base requested that NREL evaluate the feasibility of adding a biomass boiler to the district heating system served by Building 1860. We have also developed an Excel-spreadsheet-based decision support tool--specific to the historic loads served by Building 1860--with which users can perform what-if analysis on gas costs, biomass costs, and other parameters. For economic reasons, we do not recommend adding a biomass system at this time.

  3. George Orwell's Animal Farm: A metonym for a dictatorship

    Directory of Open Access Journals (Sweden)

    H. Sewlall

    2002-08-01

    Full Text Available George Orwell’s Animal Farm is traditionally read as a satire on dictatorships in general, and the Bolshevik Revolution in particular. This article postulates the notion that the schema of the book has attained the force of metonymy to such an extent that whenever one alludes to the title of the book or some lines from it, one conjures up images associated with a dictatorship. The title of the book has become a part of the conceptual political lexicon of the English language to refer to the corruption of a utopian ideology. As an ideological state, Animal Farm has its vision, which is embedded in its constitution; it has the vote, a national anthem and a flag. It even has its patriots, double-dealers, social engineers and lechers. In this way the title Animal Farm, like Joseph Heller’s Catch-22, or Thomas More’s Utopia, functions metonymically to map a conceptual framework which matches the coordinates of the book. The article concludes with a look at contemporary society to show how Orwell’s satire endorses the words of Lord Acton, namely, that power corrupts and absolute power corrupts absolutely.

  4. Fab Four: When John and George Play Gravitation and Cosmology

    Directory of Open Access Journals (Sweden)

    J.-P. Bruneton

    2012-01-01

    Full Text Available Scalar-tensor theories of gravitation attract again a great interest since the discovery of the Chameleon mechanism and of the Galileon models. The former allows reconciling the presence of a scalar field with the constraints from Solar System experiments. The latter leads to inflationary models that do not need ad hoc potentials. Further generalizations lead to a tensor-scalar theory, dubbed the “Fab Four,” with only first and second order derivatives of the fields in the equations of motion that self-tune to a vanishing cosmological constant. This model needs to be confronted with experimental data in order to constrain its large parameter space. We present some results regarding a subset of this theory named “John,” which corresponds to a nonminimal derivative coupling between the scalar field and the Einstein tensor in the action. We show that this coupling gives rise to an inflationary model with very unnatural initial conditions. Thus, we include the term named “George,” namely, a nonminimal, but nonderivative, coupling between the scalar field and Ricci scalar. We find a more natural inflationary model, and, by performing a post-Newtonian analysis, we derive the set of equations that constrain the parameter space with data from experiments in the Solar System.

  5. The story of George Huntington and his disease

    Directory of Open Access Journals (Sweden)

    Kalyan B Bhattacharyya

    2016-01-01

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

  6. AIDS. 1st annual George H. Gallup Memorial Survey.

    Science.gov (United States)

    1988-06-01

    Acquired immunodeficiency syndrome (AIDS) was selected as the subject for the 1st annual George H Gallup Memorial Survey. This survey, conducted in August 1987-April 1988 in 35 countries, measured the level of awareness of AIDS, the extent of concern about AIDS, knowledge, changes in behavior resulting form the AIDS epidemic, and attitudes toward people with AIDS. Overall, the poll's findings attest to the effectiveness of the health education efforts of governmental and nongovernmental organizations. Awareness that AIDS poses an urgent international health problem was almost universal in the 35 samples. In about half of these countries, AIDS was identified as the most important national health problem; in the remaining countries, AIDS was ranked 2nd to cancer. The proportion of respondents expressing a fear of personally contracting the AIDS virus ranged from lows of under 10% in most of Europe to a high of 45% among South African blacks. A majority of respondents in the US, Colombia, the Philippines, Brazil, Nigeria, Ecuador, El Salvador, and Chile believed that AIDS will soon spread beyond current risk groups to the general population. Despite widespread awareness of the grave threat posed by AIDS, insufficient numbers of respondents reported that they had made specific behavioral changes intended to protect themselves form HIV infection. Overall, about half of those interviewed indicated they are now more cautious in their choice of sexual partners; similarly, about half are using condoms more or for the 1st time.

  7. Speedup and fracturing of George VI Ice Shelf, Antarctic Peninsula

    Directory of Open Access Journals (Sweden)

    T. O. Holt

    2013-05-01

    Full Text Available George VI Ice Shelf (GVIIS is located on the Antarctic Peninsula, a region where several ice shelves have undergone rapid breakup in response to atmospheric and oceanic warming. We use a combination of optical (Landsat, radar (ERS 1/2 SAR and laser altimetry (GLAS datasets to examine the response of GVIIS to environmental change and to offer an assessment on its future stability. The spatial and structural changes of GVIIS (ca. 1973 to ca. 2010 are mapped and surface velocities are calculated at different time periods (InSAR and optical feature tracking from 1989 to 2009 to document changes in the ice shelf's flow regime. Surface elevation changes are recorded between 2003 and 2008 using repeat track ICESat acquisitions. We note an increase in fracture extent and distribution at the south ice front, ice-shelf acceleration towards both the north and south ice fronts and spatially varied negative surface elevation change throughout, with greater variations observed towards the central and southern regions of the ice shelf. We propose that whilst GVIIS is in no imminent danger of collapse, it is vulnerable to ongoing atmospheric and oceanic warming and is more susceptible to breakup along its southern margin in ice preconditioned for further retreat.

  8. Violence and the Sacred in Georges Bataille's Philosophy

    Directory of Open Access Journals (Sweden)

    A. ZYGMONT

    2015-06-01

    Full Text Available The article considers the concepts of violence and the sacred in the work of the French philosopher Georges Bataille. The author traces back the transformations of the concept of violence throughout the thinker’s carrier, namely in his early literary and philosophical experiences of the late 20s — early 30s, his reports for the College of Sociology (1937–1939 and his «systematic» postwar writings (1945–1962. The author also puts forward the hypothesis of the connection between the concept violence and the concept the sacred and demonstrates that it is most thoroughly represented in the last period of his work, although it may have been noted before. Diachronic consideration of the formation of these concepts allows the author to trace their correlation from the more or less random links mediated by artistic images and the other notions (sacrifi ce, for example to their explicit identification with each other and the systematic development in relation to such concepts as nature, life, being, intimacy, the animal state, expenditure, sacrifice, war and eroticism

  9. GEORG GRODDECK: "THE PINCH OF PEPPER" OF PSYCHOANALYSIS(.).

    Science.gov (United States)

    Poster, Mark F; Hristeva, Galina; Giefer, Michael

    2016-06-01

    The life and works of Georg Groddeck are reviewed and placed in historical context as a physician and a pioneer of psychoanalysis, psychosomatic medicine, and an epistolary style of writing. His Das Es concept stimulated Freud to construct his tripartite model of the mind. Groddeck, however, used Das Es to facilitate receptivity to unconscious communication with his patients. His "maternal turn" transformed his treatment approach from an authoritarian position to a dialectical process. Groddeck was a generative influence on the development of Frieda Fromm-Reichmann, Erich Fromm, and Karen Horney. He was also the mid-wife of the late-life burst of creativity of his friend and patient Sándor Ferenczi. Together, Groddeck and Ferenczi provided the impetus for a paradigm shift in psychoanalysis that emphasized the maternal transference, child-like creativity, and a dialogue of the unconscious that foreshadowed contemporary interest in intersubjectivity and field theory. They were progenitors of the relational turn and tradition in psychoanalysis. Growing interest in interpsychic communication and field theory is bringing about a convergence of theorizing among pluralistic psychoanalytic schools that date back to 1923 when Freud appropriated Groddeck's Das Es and radically altered its meaning and use.

  10. Learning from eponyms: George F. Odland and Odland bodies

    Directory of Open Access Journals (Sweden)

    Rajiv Joshi

    2014-01-01

    Full Text Available Odland bodies (lamellar bodies are small sub-cellular structures of size 200-300 nm that are present in the upper spinous and granular cell layers of the epidermis. These act as processing and repository areas for lipids that contribute to the epidermal permeability barrier. They also contain proteases, cathepsin D, kallikrein and other proteins including corneo-desmosins. Recent information also credits them with a role in the local innate immune response as they contain beta 2 defensins, which are anti-microbial peptides with potent activity against Gram-negative bacteria and candida. Odland bodies are important for maintaining homeostasis of the epidermis and are involved in epidermal permeability barrier function, desquamation of keratinocytes, formation of the cornified envelope and in local anti-microbial immunity. This article reviews the structure and functions of these bodies with a brief biography of George F. Odland who first described these bodies in 1960 and whose name is eponymically associated with them.

  11. Phrenology, heredity and progress in George Combe's Constitution of Man.

    Science.gov (United States)

    Jenkins, Bill

    2015-09-01

    The Constitution of Man by George Combe (1828) was probably the most influential phrenological work of the nineteenth century. It not only offered an exposition of the phrenological theory of the mind, but also presented Combe's vision of universal human progress through the inheritance of acquired mental attributes. In the decades before the publication of Darwin's Origin of Species, the Constitution was probably the single most important vehicle for the dissemination of naturalistic progressivism in the English-speaking world. Although there is a significant literature on the social and cultural context of phrenology, the role of heredity in Combe's thought has been less thoroughly explored, although both John van Wyhe and Victor L. Hilts have linked Combe's views on heredity with the transformist theories of Jean-Baptiste Lamarck. In this paper I examine the origin, nature and significance of his ideas and argue that Combe's hereditarianism was not directly related to Lamarckian transformism but formed part of a wider discourse on heredity in the early nineteenth century.

  12. MEMBEDAH TEORI SOSIOLOGI: Teori Pertukaran (Exchange Theory George Caspar Homans

    Directory of Open Access Journals (Sweden)

    Wardani Wardani

    2016-05-01

    Full Text Available This article is aimed at analyzing critically George Caspar Homans’s theory of exchange. The critical analysis that may be called here simply as “maping ideology” embodied in theory is needed to place the theory within the specific social and political condition, intellectual background, school of thought having great influence on it, and so forth, and also to assesses its limitedness as a theoretical frame-work of scientific research to explain complicated social phenomenon or human interaction. Influenced by psychological and economic theories, Homans’s theory of exchange has been intended at the beginning by the author as attack against Marxism domination that addressed criticism to capitalism as macro-structure oppressing the proletariat by the ruling class and against Durkheimian structural functionalism. By addressing the attack against Marxism and because of his intellectual and social background, Homans’s theory then has been regarded as an explanation justifying the existing situation. Being attributed as psychological reductionism, as neglecting the important role of structure, and deterministic are some of the objections addressed to his theory.

  13. George Marinesco in the Constellation of Modern Neuroscience

    Directory of Open Access Journals (Sweden)

    Ioan Opris

    2017-12-01

    Full Text Available George Marinesco is the founder of Romanian School of Neurology and one of the most remarkable neuroscientists of the last century. He was the pupil of Jean-Martin Charcot in Salpêtrière Hospital in Paris, France, but visited many other neurological centers where he met the entire constellation of neurologists of his time, including Camillo Golgi and Santiago Ramón y Cajal. The last made the preface of Nervous Cell, written in French by Marinesco. The original title was “La Cellule Nerveuse” and is considered even now a basic reference book for specialists in the field. He was a refined clinical observer with an integrative approach, as could be seen from the multitude of his discoveries. The descriptions of the succulent hand in syringomyelia, senile plaque in old subjects, palmar jaw reflex known as Marinesco-Radovici sign, or the application of cinematography in medicine are some of his important contributions. He was the first who described changes of locus niger in a patient affected by tuberculosis, as a possible cause in Parkinson disease. Before modern genetics, Marinesco and Sjögren described a rare and complex syndrome bearing their names. He was a hardworking man, focused on his scientific research, did not accepted flattering of others and was a great fighter against the injustice of the time.

  14. George Marinesco in the Constellation of Modern Neuroscience.

    Science.gov (United States)

    Opris, Ioan; Nestianu, Valeriu S; Nestianu, Adrian; Bilteanu, Liviu; Ciurea, Jean

    2017-01-01

    George Marinesco is the founder of Romanian School of Neurology and one of the most remarkable neuroscientists of the last century. He was the pupil of Jean-Martin Charcot in Salpêtrière Hospital in Paris, France, but visited many other neurological centers where he met the entire constellation of neurologists of his time, including Camillo Golgi and Santiago Ramón y Cajal. The last made the preface of Nervous Cell, written in French by Marinesco. The original title was "La Cellule Nerveuse" and is considered even now a basic reference book for specialists in the field. He was a refined clinical observer with an integrative approach, as could be seen from the multitude of his discoveries. The descriptions of the succulent hand in syringomyelia, senile plaque in old subjects, palmar jaw reflex known as Marinesco-Radovici sign, or the application of cinematography in medicine are some of his important contributions. He was the first who described changes of locus niger in a patient affected by tuberculosis, as a possible cause in Parkinson disease. Before modern genetics, Marinesco and Sjögren described a rare and complex syndrome bearing their names. He was a hardworking man, focused on his scientific research, did not accepted flattering of others and was a great fighter against the injustice of the time.

  15. Narrative and epistemology: Georges Canguilhem's concept of scientific ideology.

    Science.gov (United States)

    Chimisso, Cristina

    2015-12-01

    In the late 1960s, Georges Canguilhem introduced the concept of 'scientific ideology'. This concept had not played any role in his previous work, so why introduce it at all? This is the central question of my paper. Although it may seem a rather modest question, its answer in fact uncovers hidden tensions in the tradition of historical epistemology, in particular between its normative and descriptive aspects. The term ideology suggests the influence of Althusser's and Foucault's philosophies. However, I show the differences between Canguilhem's concept of scientific ideology and Althusser's and Foucault's respective concepts of ideology. I argue that Canguilhem was in fact attempting to solve long-standing problems in the tradition of historical epistemology, rather than following the lead of his younger colleagues. I argue that Canguilhem's 'refurbishment without rejection' of Bachelard's epistemology, which the concept of scientific ideology was aimed to implement, was necessary to justify the historical narratives that Canguilhem had constructed in his own work as a historian of concepts. A strict acceptance of Bachelard's epistemology would have made it impossible to justify them. Canguilhem's concept of scientific ideology therefore served as a theoretical justification of his practice as a historian. I maintain that the concept of scientific ideology was needed to reconcile Bachelard's normative epistemology with Canguilhem's view of the history of science and its aims, which differed from Bachelard's more than it is generally acknowledged. Copyright © 2015 Elsevier Ltd. All rights reserved.

  16. Classification of Kantowski-Sachs and Bianchi Type III Space-Times According to Their Killing Vector Fields in Teleparallel Theory of Gravitation

    International Nuclear Information System (INIS)

    Shabbir, Ghulam; Khan, Suhail

    2010-01-01

    In this paper we classify Kantowski-Sachs and Bianchi type III space-times according to their teleparallel Killing vector fields using direct integration technique. It turns out that the dimension of the teleparallel Killing vector fields are 4 or 6, which are the same in numbers as in general relativity. In case of 4 the teleparallel Killing vector fields are multiple of the corresponding Killing vector fields in general relativity by some function of t. In the case of 6 Killing vector fields the metric functions become constants and the Killing vector fields in this case are exactly the same as in general relativity. Here we also discuss the Lie algebra in each case. (general)

  17. Orientation relationship in various 9% Cr ferritic/martensitic steels-EBSD comparison between Nishiyama-Wassermann, Kurdjumov-Sachs and Greninger-Troiano

    International Nuclear Information System (INIS)

    Barcelo, F.; Bechade, J. L.; Fournier, B.

    2010-01-01

    EBSD measurements were carried out on four different martensitic steels (T91, P92, EM10 and Eurofer) in various metallurgical conditions (nine different microstructural states). The usual orientation relationships (ORs) between the parent austenitic phase and the resulting martensite in martensitic steels are those of Nishiyama-Wassermann (NW) and Kurjumov-Sachs (KS). The present study first proposes a methodology based on the combined analysis of the misorientation distribution, the pole figures (PFs) and the angle/axis pairs. This methodology leads to the conclusion that neither NW nor KS relationships are able to account for all the features observed whatever the material under study. A third OR proposed by Greninger and Troiano (GT) proves to describe the relationship between austenite and ferrite in all four different martensitic steels much more accurately. (authors)

  18. Studien zum "Horrendum Bellum Grammaticale" des Justus Georg Schottelius (1673): Vorstellung des Dissertationsvorhabens / Tuomo Fonsén

    Index Scriptorium Estoniae

    Fonsén, Tuomo, 1976-

    2006-01-01

    Autori dissertatsioonist, mis tutvustab Justus Georg Schotteliuse ladinakeelset allegoorilist saksa keele grammatikat ja keeleajalugu käsitlevat tööd 17. sajandist. Schottelius, Justus Georg. Horrendum Bellum Grammaticale Teutonum antiquissimorum [...]. Braunschweig : [s.n.], 1673

  19. Crystallographic Structure of Human β-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 Ganglioside Hydrolysis

    Science.gov (United States)

    Lemieux, M. Joanne; Mark, Brian L.; Cherney, Maia M.; Withers, Stephen G.; Mahuran, Don J.; James, Michael N. G.

    2010-01-01

    Lysosomal β-hexosaminidase A (Hex A) is essential for the degradation of GM2 gangliosides in the central and peripheral nervous system. Accumulation of GM2 leads to severely debilitating neurodegeneration associated with Tay-Sachs disease (TSD), Sandoff disease (SD) and AB variant. Here, we present the X-ray crystallographic structure of Hex A to 2.8 Å resolution and the structure of Hex A in complex with NAG-thiazoline, (NGT) to 3.25 Å resolution. NGT, a mechanism-based inhibitor, has been shown to act as a chemical chaperone that, to some extent, prevents misfolding of a Hex A mutant associated with adult onset Tay Sachs disease and, as a result, increases the residual activity of Hex A to a level above the critical threshold for disease. The crystal structure of Hex A reveals an αβ heterodimer, with each subunit having a functional active site. Only the α-subunit active site can hydrolyze GM2 gangliosides due to a flexible loop structure that is removed post-translationally from β, and to the presence of αAsn423 and αArg424. The loop structure is involved in binding the GM2 activator protein, while αArg424 is critical for binding the carboxylate group of the N-acetyl-neuraminic acid residue of GM2. The β-subunit lacks these key residues and has βAsp452 and βLeu453 in their place; the β-subunit therefore cleaves only neutral substrates efficiently. Mutations in the α-subunit, associated with TSD, and those in the β-subunit, associated with SD are discussed. The effect of NGT binding in the active site of a mutant Hex A and its effect on protein function is discussed. PMID:16698036

  20. GM2 gangliosidoses in Spain: analysis of the HEXA and HEXB genes in 34 Tay-Sachs and 14 Sandhoff patients.

    Science.gov (United States)

    Gort, Laura; de Olano, Natalia; Macías-Vidal, Judit; Coll, M A Josep

    2012-09-10

    The GM2 gangliosidoses are autosomal recessive lysosomal storage diseases caused by a deficiency of the β-hexosaminidase A enzyme. This enzyme is composed of two polypeptide chains designated the α- and β- subunits and it interacts with the GM2 activator protein. The HEXA and HEXB genes encode the α-subunit and the β-subunit, respectively. Mutations in these genes are causative of Tay-Sachs disease (HEXA) and Sandhoff disease (HEXB). We analyzed the complete HEXA gene in 34 Spanish patients with Tay-Sachs disease and the HEXB gene in 14 Spanish patients with Sandhoff disease. We identified 27 different mutations, 14 of which were novel, in the HEXA gene and 14 different mutations, 8 of which unreported until now, in the HEXB gene, and we attempted to correlate these mutations with the clinical presentation of the patients. We found a high frequency of c.459+5G>A (IVS4+5G>A) mutation in HEXA affected patients, 22 of 68 alleles, which represent the 32.4%. This is the highest percentage found of this mutation in a population. All patients homozygous for mutation c.459+5G>A presented with the infantile form of the disease and, as previously reported, patients carrying mutation p.R178H in at least one of the alleles presented with a milder form. In HEXB affected patients, the novel deletion c.171delG accounts for 21.4% of the mutant alleles (6/28). All patients with this deletion showed the infantile form of the disease. The Spanish GM2 gangliosidoses affected patients show a great mutational heterogeneity as seen in other inherited lisosomal diseases in this country. Copyright © 2012. Published by Elsevier B.V.

  1. Design, synthesis, and biological evaluation of enantiomeric beta-N-acetylhexosaminidase inhibitors LABNAc and DABNAc as potential agents against Tay-Sachs and Sandhoff disease.

    Science.gov (United States)

    Rountree, J S Shane; Butters, Terry D; Wormald, Mark R; Boomkamp, Stephanie D; Dwek, Raymond A; Asano, Naoki; Ikeda, Kyoko; Evinson, Emma L; Nash, Robert J; Fleet, George W J

    2009-03-01

    N-Acetylhexosaminidases are of considerable importance in mammals and are involved in various significant biological processes. In humans, deficiencies of these enzymes in the lysosome, resulting from inherited genetic defects, cause the glycolipid storage disorders Tay-Sachs and Sandhoff diseases. One promising therapy for these diseases involves the use of beta-N-acetylhexosaminidase inhibitors as chemical chaperones to enhance the enzyme activity above sub-critical levels. Herein we describe the synthesis and biological evaluation of a potent inhibitor, 2-acetamido-1,4-imino-1,2,4-trideoxy-L-arabinitol (LABNAc), in a high-yielding 11-step procedure from D-lyxonolactone. The N-benzyl and N-butyl analogues were also prepared and found to be potent inhibitors. The enantiomers DABNAc and NBn-DABNAc were synthesised from L-lyxonolactone, and were also evaluated. The L-iminosugar LABNAc and its derivatives were found to be potent noncompetitive inhibitors of some beta-N-acetylhexosaminidases, while the D-iminosugar DABNAc and its derivatives were found to be weaker competitive inhibitors. These results support previous work postulating that D-iminosugar mimics inhibit D-glycohydrolases competitively, and that their corresponding L-enantiomers show noncompetitive inhibition of these enzymes. Molecular modelling studies confirm that the spatial organisation in enantiomeric inhibitors leads to a different overlay with the monosaccharide substrate. Initial cell-based studies suggest that NBn-LABNAc can act as a chemical chaperone to enhance the deficient enzyme's activity to levels that may cause a positive pharmacological effect. LABNAc, NBn-LABNAc, and NBu-LABNAc are potent and selective inhibitors of beta-N-acetylhexosaminidase and may be useful as therapeutic agents for treating adult Tay-Sachs and Sandhoff diseases.

  2. Strategic Energy Management Plan For Fort Buchanan, Puerto Rico

    Energy Technology Data Exchange (ETDEWEB)

    Parker, Steven A.; Hunt, W. D.

    2001-10-31

    This document reports findings and recommendations as a result of a design assistance project with Fort Buchanan with the goals of developing a Strategic Energy Management Plan for the Site. A strategy has been developed with three major elements in mind: 1) development of a strong foundation from which to build, 2) understanding technologies that are available, and 3) exploring financing options to fund the implementation of improvements. The objective of this report is to outline a strategy that can be used by Fort Buchanan to further establish an effective energy management program. Once a strategy is accepted, the next step is to take action. Some of the strategies defined in this Plan may be implemented directly. Other strategies may require the development of a more sophisticated tactical, or operational, plan to detail a roadmap that will lead to successful realization of the goal. Similarly, some strategies are not single events. Rather, some strategies will require continuous efforts to maintain diligence or to change the culture of the Base occupants and their efforts to conserve energy resources.

  3. Neurosensory changes of palatal mucousa following Le Fort I osteotomy

    Directory of Open Access Journals (Sweden)

    Bijan Movahedian Attar

    2009-09-01

    Full Text Available

    • BACKGROUND: This study evaluated the sensation of palatal ucosa before and after Le Fort I osteotomy and compared it based on whether greater palatine nerve has been dissected or not.
    • METHODS: Sixteen patients were studied within one week before  urgery and then one week, 6 weeks, 3 months and 6 months after surgery. Four tests including sharp-blunt discrimination, cold perception, pin prick sensation and electrical stimulation were performed.
    • RESULTS: Mean values of electrical stimulation were significantly higher 6 months after surgery (p < 0.05, on the other hand mean values of pin-prick sensation were significantly lower (p < 0.05. All patients regardless of the condition of greater palatine nerve were responsive to cold perception and sharp-blunt discrimination 6 months after surgery.
    • CONCLUSIONS: Following Le Fort I osteotomy, palatal  esponsiveness to electrical stimulation decreases and mechanical hyper sensitization occurs. Dissection of greater palatine nerve was shown to have no effect on the results.
    • KEYWORDS: Lefort I Osteotomy, Palatal Mocousa, Nerve Recovery.

  4. Fort Stewart integrated resource assessment. Volume 3: Resource assessment

    Energy Technology Data Exchange (ETDEWEB)

    Sullivan, G.P.; Keller, J.M.; Stucky, D.J.; Wahlstrom, R.R.; Larson, L.L.

    1993-10-01

    The US Army Forces Command (FORSCOM) has tasked the US Department of Energy (DOE) Federal Energy Management Program (FEMP), supported by the Pacific Northwest Laboratory, to identify, evaluate, and assist in acquiring all cost-effective energy projects at Fort Stewart. This is part of a model program that PNL is designing to support energy-use decisions in the federal sector. This report provides the results of the fossil fuel and electric energy resource opportunity (ERO) assessments performed by PNL at the FORSCOM Fort Stewart facility located approximately 25 miles southwest of Savannah, Georgia. It is a companion report to Volume 1, Executive Summary, and Volume 2, Baseline Detail. The results of the analyses of EROs are presented in 11 common energy end-use categories (e.g., boilers and furnaces, service hot water, and building lighting). A narrative description of each ERO is provided, along with a table detailing information on the installed cost, energy and dollar savings; impacts on operations and maintenance (O&M); and, when applicable, a discussion of energy supply and demand, energy security, and environmental issues. A description of the evaluation methodologies and technical and cost assumptions is also provided for each ERO. Summary tables present the cost-effectiveness of energy end-use equipment before and after the implementation of each ERO. The tables also present the results of the life-cycle cost (LCC) analysis indicating the net present value (NPV) and savings to investment ratio (SIR) of each ERO.

  5. Information management needs for Fort Calhoun's design basis reconstitution project

    International Nuclear Information System (INIS)

    Beach, D.R.; Erickson, E.A.; Gambhir, S.K.; Parsons, R.D.

    1989-01-01

    While the need for information management is not new to the nuclear industry or Omaha Public Power District (OPPD), the interrelationship among design information, multiple systems, and design basis issues has necessitated the management of this information in new ways. The project team involved in the reconstitution of the design basis for OPPD's Fort Calhoun nuclear station has experienced the need for the developed effective methods for managing the vast amount of interrelated information associated with this effort. This management of information has been necessary to ensure that design basis documents (DBDs) adequately reflect the interrelated nature of component, system, and plant design; are complete and accurate; and are produced and maintained in a cost-effective manner. Fort Calhoun's aggressive design basis reconstitution project began in early 1987. The present scope of the project includes the production of 52 system and plant level DBDs; currently the project is ∼50% complete with DBDs in various stages of completion, from pilot DBDs through DBDs with approved formats, which have been issued for use. The experience in producing these documents has lead to a growing understanding of the special need for information management in each stage of the project. The development of the information tracking and management processes for the various stages of DBD development has proven to be cost-effective and gives a level of assurance that information has been included in the DBDs consistently and accurately

  6. Comprehensive Inventory and Determinations of Eligibility for Fort Riley Buildings: 1857-1963

    Science.gov (United States)

    2009-09-01

    become fashionable . Stone residences built at Fort Riley after the 1850s all have rock-faced walls and most have contrasting smooth-faced lintels...507 is significant as a wood-framed Folk Victorian cottage. While Building 507 is one of four Folk Victorian buildings at Fort Riley, it possesses a

  7. A visual progression of the Fort Valley Restoration Project treatments using remotely sensed imagery (P-53)

    Science.gov (United States)

    Joseph E. Crouse; Peter Z. Fule

    2008-01-01

    The landscape surrounding the Fort Valley Experimental Forest in northern Arizona has changed dramatically in the past decade due to the Fort Valley Restoration Project, a collaboration between the Greater Flagstaff Forest Partnership, Coconino National Forest, and Rocky Mountain Research Station. Severe wildfires in 1996 sparked community concern to start restoration...

  8. 75 FR 39051 - Desoto Mills LLC, Fort Payne, AL; Notice of Negative Determination Regarding Application for...

    Science.gov (United States)

    2010-07-07

    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-73,416] Desoto Mills LLC, Fort... applicable to workers and former workers at Desoto Mills, LLC, a Subsidiary of Fruit of the Loom, Fort Payne... * * * locations outside the Desoto Mills Plant.'' The petitioner compares the situation at this location with...

  9. 77 FR 71636 - Huntington Foam LLC, Fort Smith, AR; Notice of Revised Determination on Reconsideration

    Science.gov (United States)

    2012-12-03

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

  10. Recolonization of gravel habitats on Georges Bank (northwest Atlantic)

    Science.gov (United States)

    Collie, Jeremy S.; Hermsen, Jerome M.; Valentine, Page C.

    2009-09-01

    Gravel habitats on continental shelves around the world support productive fisheries but are also vulnerable to disturbance from bottom fishing. We conducted a 2-year in situ experiment to measure the rate of colonization of a gravel habitat on northern Georges Bank in an area closed to fishing (Closed Area II) since December 1994. Three large (0.25 m 2) sediment trays containing defaunated pebble gravel were deployed at a study site (47 m water depth) in July 1997 and recovered in June 1999. The undersides of the tray lids positioned 56 cm above the trays served as settlement panels over the same time period. We observed rapid colonization of the gravel substrate (56 species) and the settlement panels (35 species), indicating that colonization of gravel in this region is not limited by the supply of colonists. The species composition of the taxa found in the trays was broadly similar to that we collected over a 10-year period (1994-2004) in dredge samples from gravel sediments at the same site. The increase in abundance of animals in the gravel colonization trays was rapid and reached a level in 2 years that took 4.5 years to achieve in the surrounding gravel sediments once fishing had stopped, based on data from dredge sampling at this site. The increase in biomass of animals found in the sediment trays paralleled the trend of biomass increase observed in dredge samples over the same period (1997-1999) but was lower in value. These data suggest that after rapid initial increase in abundance of organisms, succession proceeded by increasing individual body size. A comparison of settlement panel and tray faunas revealed that the mean biomass of structure-forming epifauna (sponges, bryozoans, anemones, hydroids, colonial tube worms) on the panels was 8 times that found on the trays. Structure-forming taxa constituted 29% of the mean biomass of the panel fauna but only 5.5% of the tray fauna. By contrast, the mean biomass of scavengers (crabs, echinoderms, nudibranchs

  11. Recolonization of gravel habitats on Georges Bank (northwest Atlantic)

    Science.gov (United States)

    Collie, Jeremy S.; Hermsen, Jerome M.; Valentine, Page C.

    2009-01-01

    Gravel habitats on continental shelves around the world support productive fisheries but are also vulnerable to disturbance from bottom fishing. We conducted a 2-year in situ experiment to measure the rate of colonization of a gravel habitat on northern Georges Bank in an area closed to fishing (Closed Area II) since December 1994. Three large (0.25 m2) sediment trays containing defaunated pebble gravel were deployed at a study site (47 m water depth) in July 1997 and recovered in June 1999. The undersides of the tray lids positioned 56 cm above the trays served as settlement panels over the same time period. We observed rapid colonization of the gravel substrate (56 species) and the settlement panels (35 species), indicating that colonization of gravel in this region is not limited by the supply of colonists. The species composition of the taxa found in the trays was broadly similar to that we collected over a 10-year period (1994-2004) in dredge samples from gravel sediments at the same site. The increase in abundance of animals in the gravel colonization trays was rapid and reached a level in 2 years that took 4.5 years to achieve in the surrounding gravel sediments once fishing had stopped, based on data from dredge sampling at this site. The increase in biomass of animals found in the sediment trays paralleled the trend of biomass increase observed in dredge samples over the same period (1997-1999) but was lower in value. These data suggest that after rapid initial increase in abundance of organisms, succession proceeded by increasing individual body size. A comparison of settlement panel and tray faunas revealed that the mean biomass of structure-forming epifauna (sponges, bryozoans, anemones, hydroids, colonial tube worms) on the panels was 8 times that found on the trays. Structure-forming taxa constituted 29% of the mean biomass of the panel fauna but only 5.5% of the tray fauna. By contrast, the mean biomass of scavengers (crabs, echinoderms, nudibranchs

  12. Neurosurgical Work during the Napoleonic Wars: George James Guthrie's Experience.

    Science.gov (United States)

    Roux, Franck-Emmanuel

    2016-01-01

    Involved in what is still considered, along with the two world wars of the 20th century, as one of the major conflicts in Europe, George James Guthrie (1785-1856) was the most famous English army surgeon of the Napoleonic wars. After treating the injured throughout the Peninsular Campaign (1808-1814), in 1815 and then in 1842 he published two major books dealing with cranial and brain injuries, among other topics. In these books, we can find, for example, an early description of the plantar reflex further described by Joseph Babinsky, accurate descriptions of the clinical signs of intracranial hypertension, and details of the physiopathology of subdural and epidural haematomas. Skull fractures are also discussed intensively, along with the indications for trepanation, a much-debated issue at the turn of the 19th century. The dura was often the limit of the surgical field for Guthrie. Nevertheless, he tried to rationalize the use of trepanation and favoured its use in two main cases: in cases of depressed skull bones, jammed bone fragments or debris irritating the dura or the brain and in cases of life-threatening cerebral compression caused by supposed blood clots. In their works, Guthrie and his contemporaries did not address neurosurgery in the modern sense of the word, but rather 'cranial surgery' in most cases. Guthrie, who saw so many patients with brain injuries and amputations, failed to understand that cerebral functions could be localized to the cortex and neglected to describe the phantom limb phenomenon, as did most of his contemporaries. © 2016 S. Karger AG, Basel.

  13. Residential architecture of George Pavlovic Kovalevsky in interwar Belgrade

    Directory of Open Access Journals (Sweden)

    Vuksanović-Macura Zlata

    2016-01-01

    Full Text Available George Pavlovic Kovalevsky, architect and urban planner, belongs to the most significant authors who created in the period between the Two World Wars in Belgrade. Yet, his work and life have rarely been the subject of scholarly literature, while the study of his housing projects has been overlooked. This paper discusses housing projects designed by Kovalevsky mainly in collaboration with another Russian architect Vladimir Bilinski. The analysis of the projects is based on original archival plans, documents and photographs. Residential buildings designed during 1921-1923 by these two architects include small single-storey houses, multi-storey building and spacious villas. Although of different surface, volume and shape, they have some common characteristics such as: classical construction, equable aesthetic quality that followed the spirit of the late Secession or the modernized Academicism, and were designed in line with the requirements of the property-owners. Buildings created by Kovalevsky and Bilinski cannot be considered among the most important achievements of the interwar Belgrade residential architecture, but their study is important because it contributes to a better understanding and evaluation of the work of these authors, as well as the overall architectural production of that period. In a broader sense, this paper builds upon general rise in interest in the revaluation of cultural, social and historical values of residential architecture in Belgrade that was created in the late 19th and the first half of the 20th century, as an important segment of the material and immaterial cultural heritage and the identity of the city and contributes to its more adequate interpretation and preservation.

  14. Philosophy of War in Georges Bataille’s Works

    Directory of Open Access Journals (Sweden)

    Zygmont Aleksei

    2017-03-01

    Full Text Available The paper considers various conceptualisations of war in works by the French philosopher Georges Bataille as well as their relationship with the notion of the sacred. It is suggested that this philosopher was the first to propose such formulation of the problem that emerged at the intersection of two interrelated views, namely the one about the decline of the sacred in the modern world and about the essential difference between World War ΙΙ and all previous wars. The latter view had made possible speculations as to the “sacred” character of this war. Tracing the development of the philosopher’s views on this subject from the 1930s to 1960s, the paper suggests that he consistently condemns war as an armed political conflict and contrasted it with religious perception of life and with free movement of violence in the forms of revolution, tragedy, and sacrifi ce, thus denying the sacred character of the war. Nevertheless, arguing against the so-called “modern” war, which appears pragmatic, mechanised, profane and threatening humankind with complete devastation, he contrasts it with two other — sacred — types of war. The first is “archaic” war. Its significance is much closer to that of sacrifi ce. It transfigures the human being by putting the latter in the proximity to death. The second is “spiritual fight”, the battle against his or her own subjectivity. Bataille borrowed this conception from Catholic mysticism of the Middle Ages. These different views on war arise from the multifaceted personal experience of the philosopher himself.

  15. Georges Marinesco (1863-1938): neurologist, neurohistologist and neuropathologist.

    Science.gov (United States)

    Catala, M; Poirier, J

    2012-01-01

    Gheorghe Marinescu (Georges Marinesco, in French) is a Romanian physician, founder of the School of Neurology in that country. He begins his medical studies in Bucharest, then has the opportunity to reach Paris and join the School of Neurology in La Salpêtrière Hospital, lead by Jean-Martin Charcot. This trip will forever imprint the mind of Marinescu, a great friend of France, a respectful student of Charcot and a friendly colleague of many Parisian neurologists. Marinescu's works are multiple and very important. He describes the succulent hand in syringomyelia and the palmar-jaw reflex. Marinescu is also one of the first to use the cinema for medical purposes. His work as an anatomo-clinician, a method developed by Charcot, is important. We denote the description of the locus niger affected by tuberculosis in a case of parkinsonism (this description paving Etienne Brissaud's way to highlight the anatomical origin of Parkinson's disease), the original clinical description of Marinesco-Sjögren syndrome, and that of medullomyoblastoma. Marinescu is also a famous neurocytologist as evidenced by his work, La Cellule Nerveuse, published in 1909. The first volume of the book is devoted to the aspects of the normal nervous tissue: the neurofibrillar network, the chromatophilic elements, and the paranucleolar corpuscles (now known as Marinescu's bodies). The second volume of the book is almost related to features revealed by experimental lesions: chromatolysis and neuronophagia. Furthermore, Marinescu describes with Oscar Blocq, small nodules than are now regarded as the first description of senile plaques.

  16. 77 FR 9960 - Final Environmental Impact Statement for Extension of F-Line Streetcar Service to Fort Mason...

    Science.gov (United States)

    2012-02-21

    ... Environmental Impact Statement for Extension of F-Line Streetcar Service to Fort Mason Center, San Francisco, CA... Environmental Impact Statement for the Extension of F-Line Streetcar Service to Fort Mason Center, San Francisco... the extension of the historic streetcar F-line from Fisherman's Wharf to the Fort Mason Center, in San...

  17. 78 FR 28622 - Notice of Approval of Record of Decision for Extending F-Line Streetcar Service to Fort Mason...

    Science.gov (United States)

    2013-05-15

    ...] Notice of Approval of Record of Decision for Extending F-Line Streetcar Service to Fort Mason Center... Environmental Impact Statement (Final EIS) for extending the F-Line historic streetcar service to Fort Mason... turnaround terminus at the Fort Mason Center; and installing appurtenant features such as signals, crossings...

  18. 77 FR 58354 - Bend-Fort Rock Ranger District; Oregon; Withdrawal of Notice for Preparation of an Environmental...

    Science.gov (United States)

    2012-09-20

    ...-Fort Rock Ranger District; Oregon; Withdrawal of Notice for Preparation of an Environmental Impact... Administration, USDOT. ACTION: Notice of withdrawal. SUMMARY: The Bend-Fort Rock Ranger District and FHWA are..., Project Leader, Bend- Fort Rock Ranger District, 63095 Deschutes Market Road, Bend, OR 97701, phone 541...

  19. Revisiting "Narrow Bipolar Event" intracloud lightning using the FORTE satellite

    Science.gov (United States)

    Jacobson, A. R.; Light, T. E. L.

    2012-02-01

    The lightning stroke called a "Narrow Bipolar Event", or NBE, is an intracloud discharge responsible for significant charge redistribution. The NBE occurs within 10-20 μs, and some associated process emits irregular bursts of intense radio noise, fading at shorter timescales, sporadically during the charge transfer. In previous reports, the NBE has been inferred to be quite different from other forms of lightning strokes, in two ways: First, the NBE has been inferred to be relatively dark (non-luminous) compared to other lightning strokes. Second, the NBE has been inferred to be isolated within the storm, usually not participating in flashes, but when it is in a flash, the NBE has been inferred to be the flash initiator. These two inferences have sufficiently stark implications for NBE physics that they should be subjected to further independent test, with improved statistics. We attempt such a test with both optical and radio data from the FORTE satellite, and with lightning-stroke data from the Los Alamos Sferic Array. We show rigorously that by the metric of triggering the PDD optical photometer aboard the FORTE satellite, NBE discharges are indeed less luminous than ordinary lightning. Referred to an effective isotropic emitter at the cloud top, NBE light output is inferred to be less than ~3 × 108 W. To address isolation of NBEs, we first expand the pool of geolocated intracloud radio recordings, by borrowing geolocations from either the same flash's or the same storm's other recordings. In this manner we generate a pool of ~2 × 105 unique and independent FORTE intracloud radio recordings, whose slant range from the satellite can be inferred. We then use this slant range to calculate the Effective Radiated Power (ERP) at the radio source, in the passband 26-49 MHz. Stratifying the radio recordings by ERP into eight bins, from a lowest bin (140 kW), we document a trend for the radio recordings to become more isolated in time as the ERP increases. The highest

  20. Georg R. R. Martin: "Jää ja tule laul" filmi ei mahu / intervjueerinud Hendrik Alla

    Index Scriptorium Estoniae

    Martin, George R. R., 1948-

    2011-01-01

    USA telekanali HBO telesarjast "Troonide mäng", George R. R. Martini "Jää ja tule laulu" sarja esimese raamatu "Troonide mäng" teleekraniseeringust. Sarja esimene osa esilinastus USA-s 17. aprillil

  1. Surface and Bottom Boundar Layer Dynamics on a Shallow Submarine Bank: Southern Flank of Georges Bank

    National Research Council Canada - National Science Library

    Werner, Sandra

    1999-01-01

    The thesis investigates the circulation at a 75-meter deep study site on the southern flank of Georges Bank, a shallow submarine bank located between the deeper Gulf of Maine and the continental slope...

  2. Adequacy of environmental information for outer continental shelf oil and gas decisions: Georges Bank. Final report

    International Nuclear Information System (INIS)

    1991-01-01

    Georges Bank, a large, shallow marine bank with important fishery resources and possibly important oil and gas resources, lies east of Massachusetts in the territorial waters of both the United States and Canada. The Department of the Interior has planned since 1974 to lease parts of the north Atlantic outer continental shelf (OCS)--including part of Georges Bank--for oil and gas exploration. As a result of public concern about the environmental impacts of oil and gas production on the U.S. OCS, Congress declared a moratorium on drilling on Georges Bank and an area to the southwest. The report--by the NRC's Committee to Review the Outer Continental Shelf Environmental Studies Program and its panels on physical oceanography, ecology, and socioeconomics--reviews the adequacy of information bearing on the potential environmental impacts of OCS oil and gas activities for the Georges Bank sale area

  3. Eesti kriminaalõiguse arenguteed : kolmveerand sajandit esimese kriminaalseadustiku vastuvõtmisest / Georg Ambach

    Index Scriptorium Estoniae

    Ambach, Georg, 1952-2006

    2004-01-01

    Karl Saarmannist ja tema teesidest seoses karistusseadustikuga ja tegevusest kriminaalreformi läbiviimisel. Karistusseadustiku eelnõu väljatöötamisest. Mag. iur. Georg Ambach on professor Karl Saarmanni Seltsi juhatuse esimees

  4. Raamidest välja mõtlemine pole piisav / Gabor George Burt ; interv. Harli Uljas

    Index Scriptorium Estoniae

    Burt, Gabor George

    2006-01-01

    Juhtimisstrateegia Blue Ocean Strategy eestkõneleja Gabor George Burti sõnul tuleb edu saavutamiseks keskenduda kliendile ning tegeleda personaalselt kliendi jaoks sobiva lahenduse loomisega. Kommenteerib Priit Põldoja

  5. Muusika : Viiulivirtuoos Kanadast. Holland Music Sessionsi stipendiaadid. "Jazzkaar" Georg Otsa jälgedes

    Index Scriptorium Estoniae

    2002-01-01

    Estonia kontserdisaalis esineb ERSO ees tippviiuldaja Lara St. John. Holland Music Sessions'i meistrikursustele pääses konkursi korras kuus eesti noort muusikut. Vene Draamateatris 17. III toimuvast kontserdist "Georg Otsa jälgedes"

  6. Vanim humanistlik album amicorum Eestis : Alciato "Embleemid" Georg Locameruse reisialbumina / Katre Kaju, Kristi Viiding

    Index Scriptorium Estoniae

    Kaju, Katre, 1974-

    2014-01-01

    Varastest reisialbumitest. Georg Locamerusest ja reisialbumi sisseseadmisest. Locameruse reisialbumi raamatuloolistest ja paleograafilistest aspektidest. Reisialbumist kui selektiivse ja idealiseeritud sotsiaalse võrgustiku peegeldajast. Albumi keelelisest ja kirjanduslikust profiilist. Reisialbumi teekonnast Tartu ülikooli raamatukokku. Editeerimispõhimõtetest

  7. St. George Feast: Relation to Eastern Legends and Cults [In Bulgarian

    Directory of Open Access Journals (Sweden)

    S. Rulinski

    2016-07-01

    Full Text Available The article examines George as a counterpart to the Sumerian god Ninurta. Feast St. George boasts Georgy feat - creation of the world. The date of the feast – 6th of May, is commented in its relation to the astronomical arguments and Sumerian ancient texts. The main rituals of the feast are described. The most typical folk songs devoted to St. Georgy are listed in the paper appendix.

  8. On Some Philosophical Aspects of the Background to Georg Cantor’s theory of sets

    OpenAIRE

    Tapp, Christian

    2011-01-01

    Georg Cantor a cherché à assurer les fondements de sa théorie des ensembles. Cet article présente les differentiations cantoriennes concernant la notion d’infinité et une perspective historique de l’émergence de sa notion d’ensemble. Georg Cantor sought secure foundations for his set theory. This article presents an account of Cantor’s differentiations concerning the notion of infinity and a tentative historical parsepctive on his notion of set.

  9. Noonan's and DiGeorge syndromes with monosomy 22q11.

    OpenAIRE

    Wilson, D I; Britton, S B; McKeown, C; Kelly, D; Cross, I E; Strobel, S; Scambler, P J

    1993-01-01

    A boy with the dysmorphic features of Noonan's syndrome and pulmonary valve stenosis who had evidence of hypoparathyroidism and abnormal T lymphocyte numbers in the neonatal period is reported. He had a normal karyotype but molecular analysis revealed a submicroscopic deletion within chromosome 22q11, the region deleted in DiGeorge syndrome. Thus this child has both Noonan's syndrome and DiGeorge syndrome; 22q11 is a candidate region for a gene defective in Noonan's syndrome.

  10. EAARL Topography - George Washington Birthplace National Monument 2008

    Science.gov (United States)

    Brock, John C.; Nayegandhi, Amar; Wright, C. Wayne; Stevens, Sara; Yates, Xan

    2009-01-01

    These remotely sensed, geographically referenced elevation measurements of Lidar-derived bare earth (BE) and first surface (FS) topography were produced as a collaborative effort between the U.S. Geological Survey (USGS), Florida Integrated Science Center (FISC), St. Petersburg, FL; the National Park Service (NPS), Northeast Coastal and Barrier Network, Kingston, RI; and the National Aeronautics and Space Administration (NASA), Wallops Flight Facility, VA. This project provides highly detailed and accurate datasets of the George Washington Birthplace National Monument in Virginia, acquired on March 26, 2008. The datasets are made available for use as a management tool to research scientists and natural resource managers. An innovative airborne Lidar instrument originally developed at the NASA Wallops Flight Facility, and known as the Experimental Advanced Airborne Research Lidar (EAARL) was used during data acquisition. The EAARL system is a raster-scanning, waveform-resolving, green-wavelength (532-nanometer) Lidar designed to map near-shore bathymetry, topography, and vegetation structure simultaneously. The EAARL sensor suite includes the raster-scanning, water-penetrating full-waveform adaptive Lidar, a down-looking red-green-blue (RGB) digital camera, a high-resolution multi-spectral color infrared (CIR) camera, two precision dual-frequency kinematic carrier-phase GPS receivers, and an integrated miniature digital inertial measurement unit, which provide for submeter georeferencing of each laser sample. The nominal EAARL platform is a twin-engine Cessna 310 aircraft, but the instrument may be deployed on a range of light aircraft. A single pilot, a Lidar operator, and a data analyst constitute the crew for most survey operations. This sensor has the potential to make significant contributions in measuring sub-aerial and submarine coastal topography within cross-environmental surveys. Elevation measurements were collected over the survey area using the EAARL

  11. Modern sedimentation patterns in Potter Cove, King George Island, Antarctica

    Science.gov (United States)

    Hass, H. Christian; Kuhn, Gerhard; Wölfl, Anne-Cathrin; Wittenberg, Nina; Betzler, Christian

    2013-04-01

    IMCOAST among a number of other initiatives investigates the modern and the late Holocene environmental development of south King George Island with a strong emphasis on Maxwell Bay and its tributary fjord Potter Cove (maximum water depth: about 200 m). In this part of the project we aim at reconstructing the modern sediment distribution in the inner part of Potter Cove using an acoustic ground discrimination system (RoxAnn) and more than136 ground-truth samples. Over the past 20 years the air temperatures in the immediate working area increased by more than 0.6 K (Schloss et al. 2012) which is less than in other parts of the West Antarctic Peninsula (WAP) but it is still in the range of the recovery of temperatures from the Little Ice Age maximum to the beginning of the 20th century. Potter Cove is a small fjord characterized by a series of moraine ridges produced by a tidewater glacier (Fourcade Glacier). Presumably, the farthest moraine is not much older than about 500 years (LIA maximum), hence the sediment cover is rather thin as evidenced by high resolution seismic data. Since a few years at least the better part of the tidewater glacier retreated onto the island's mainland. It is suggested that such a fundamental change in the fjord's physiography has also changed sedimentation patterns in the area. Potter Cove is characterized by silty-clayey sediments in the deeper inner parts of the cove. Sediments are coarser (fine to coarse sands and boulders) in the shallower areas; they also coarsen from the innermost basin to the mouth of the fjord. Textural structures follow the seabed morphology, i.e. small v-shaped passages through the moraine ridges. The glacier still produces large amounts of turbid melt waters that enter the cove at various places. We presume that very fine-grained sediments fall out from the meltwater plumes and are distributed by mid-depth or even bottom currents, thus suggesting an anti-estuarine circulation pattern. Older sediments that are

  12. Preplaced aggregate concrete application on Fort St. Vrain PCRV construction

    International Nuclear Information System (INIS)

    Ople, F.S. Jr.

    1976-01-01

    Two distinct concreting methods were employed in the construction of the prestressed concrete reactor vessel (PCRV) of the Fort St. Vrain (FSV) Nuclear Generating Station, a 330 MW(e) High Temperature Gas-Cooled Reactor installation near Denver, Colorado. Preplaced aggregate concrete (PAC) techniques were employed in the PCRV bottom head and the core support floor; conventional job-mixed concrete was used in the PCRV sidewall and top head regions. This paper describes the successful application of PAC techniques utilized primarily in solving construction difficulties associated with confined and heavily congested regions of the PCRV. The PAC technique consists of placing coarse aggregate inside the forms, followed by injection of grout under pressure through embedded pipes to fill the interstices in the aggregate mass. Details of the PAC construction method including grout mix development, grouting equipment, grout pipe layout, grouting sequence, grout level monitoring, concrete temperature control, and pre-construction mockups are described. (author)

  13. Analysis of natural gas supply strategies at Fort Drum

    International Nuclear Information System (INIS)

    Stucky, D.J.; Shankle, S.A.; Anderson, D.M.

    1992-07-01

    This analysis investigates strategies for Fort Drum to acquire a reliable natural gas supply while reducing its gas supply costs. The purpose of this study is to recommend an optimal supply mix based on the life-cycle costs of each strategy analyzed. In particular, this study is intended to provide initial guidance as to whether or not the building and operating of a propane-air mixing station is a feasible alternative to the current gas acquisition strategy. The analysis proceeded by defining the components of supply (gas purchase, gas transport, supplemental fuel supply); identifying alternative options for each supply component; constructing gas supply strategies from different combinations of the options available for each supply component and calculating the life-cycle costs of each supply strategy under a set of different scenarios reflecting the uncertainty of future events

  14. Depressurization accident analyses for the Fort St. Vrain Reactor

    International Nuclear Information System (INIS)

    Paul, D.D.

    1976-01-01

    Design-basis depressurization accident analyses for the Fort St. Vrain reactor were performed using the FLODIS (Ref. 4) code. The FLODIS code models the active core, side reflector, gas annulus between the core barrel and the PCRV liner, and the PCRV cooling system. Results are presented for the Pelton circulators operating at 10,550, 8800, and 7000 rpm. Maximum temperatures of selected components are plotted as a function of time during the transient. None of the components studied exceeded the temperature at which failure or damage may occur. However, there must be sufficient mixing of the outlet gas in the lower plenum to insure the integrity of the steel liners of the steam generator inlet ducts

  15. Fort St. Vrain reactor performance and operation to full power

    International Nuclear Information System (INIS)

    Simon, W.A.; Bramblett, G.C.

    1982-01-01

    The Fort St. Vrain Nuclear Generating Station, powered by a high-temperature gas-cooled reactor (HTGR), has now been tested to full thermal power. Testing was conducted for the dual purposes of demonstrating component and system capability as a part of the rise-to-power program and determining core fluctuation/redistribution behavior under full power conditions. Both objectives were met. Full power performance of all major components and the achievement of nearly all design objectives has been verified. In addition, the tests showed that the fluctuation phenomenon has been corrected. Core region outlet temperature redistributions have been characterized, related to a physical mechanism, and shown to be inconsequential for overall plant operation

  16. Fort St. Vrain graphite site mechanical separation concept selection

    International Nuclear Information System (INIS)

    Berry, S.M.

    1993-09-01

    One of the alternatives to the disposal of the Fort St. Vrain (FSV) reactor spent nuclear fuel involves the separation of the fuel rods composed of compacts from the graphite fuel block assembly. After the separation of these two components, the empty graphite fuel blocks would be disposed of as a low level waste (provided the appropriate requirements are met) and the fuel compacts would be treated as high level waste material. This report deals with the mechanical separation aspects concerning physical disassembly of the FSV graphite fuel element into the empty graphite fuel blocks and fuel compacts. This report recommends that a drilling technique is the preferred choice for accessing the, fuel channel holes and that each hole is drilled separately. This report does not cover any techniques or methods to separate the triso fuel particles from the graphite matrix of the fuel compacts

  17. Exploration Drilling and Technology Demonstration At Fort Bliss

    Energy Technology Data Exchange (ETDEWEB)

    Barker, Ben; Moore, Joe [EGI; Segall, Marylin; Nash, Greg; Simmons, Stuart; Jones, Clay; Lear, Jon; Bennett, Carlon

    2014-02-26

    The Tularosa-Hueco basin in south-central New Mexico has long been known as an extensional area of high heat flow. Much of the basin is within the Fort Bliss military reservation, which is an exceptionally high value customer for power independent of the regional electric grid and for direct use energy in building climate control. A series of slim holes drilled in the 1990s established the existence of a thermal anomaly but not its practical value. This study began in 2009 with a demonstration of new exploration drilling technology. The subsequent phases reported here delivered a useful well, comparative exploration data sets and encouragement for further development. A production-size well, RMI56-5, was sited after extensive study of archival and newly collected data in 2010-2011. Most of 2012 was taken up with getting state and Federal authorities to agree on a lead agency for permitting purposes, getting a drilling permit and redesigning the drilling program to suit available equipment. In 2013 we drilled, logged and tested a 924 m well on the McGregor Range at Fort Bliss using a reverse circulation rig. Rig tests demonstrated commercial permeability and the well has a 7-inch slotted liner for use either in production or injection. An August 2013 survey of the completed well showed a temperature of 90 C with no reversal, the highest such temperature in the vicinity. The well’s proximity to demand suggests a potentially valuable resource for direct use heat and emergency power generation. The drilling produced cuttings of excellent size and quality. These were subjected to traditional analyses (thin sections, XRD) and to the QEMScan™ for comparison. QEMScan™ technology includes algorithms for determining such properties of rocks as density, mineralogy, heavy/light atoms, and porosity to be compared with direct measurements of the cuttings. In addition to a complete cuttings set, conventional and resistivity image logs were obtained in the open hole before

  18. The bystander effect model of Brenner and Sachs fitted to lung cancer data in 11 cohorts of underground miners, and equivalence of fit of a linear relative risk model with adjustment for attained age and age at exposure

    International Nuclear Information System (INIS)

    Little, M P

    2004-01-01

    Bystander effects following exposure to α-particles have been observed in many experimental systems, and imply that linearly extrapolating low dose risks from high dose data might materially underestimate risk. Brenner and Sachs (2002 Int. J. Radiat. Biol. 78 593-604; 2003 Health Phys. 85 103-8) have recently proposed a model of the bystander effect which they use to explain the inverse dose rate effect observed for lung cancer in underground miners exposed to radon daughters. In this paper we fit the model of the bystander effect proposed by Brenner and Sachs to 11 cohorts of underground miners, taking account of the covariance structure of the data and the period of latency between the development of the first pre-malignant cell and clinically overt cancer. We also fitted a simple linear relative risk model, with adjustment for age at exposure and attained age. The methods that we use for fitting both models are different from those used by Brenner and Sachs, in particular taking account of the covariance structure, which they did not, and omitting certain unjustifiable adjustments to the miner data. The fit of the original model of Brenner and Sachs (with 0 y period of latency) is generally poor, although it is much improved by assuming a 5 or 6 y period of latency from the first appearance of a pre-malignant cell to cancer. The fit of this latter model is equivalent to that of a linear relative risk model with adjustment for age at exposure and attained age. In particular, both models are capable of describing the observed inverse dose rate effect in this data set

  19. Electricity Generation from Geothermal Resources on the Fort Peck Reservation in Northeast Montana

    Energy Technology Data Exchange (ETDEWEB)

    Carlson, Garry J. [Gradient Geophysics Inc., Missoula, MT (United States); Birkby, Jeff [Birkby Consulting LLC, Missoula, MT (United States)

    2015-05-12

    Tribal lands owned by Assiniboine and Sioux Tribes on the Fort Peck Indian Reservation, located in Northeastern Montana, overlie large volumes of deep, hot, saline water. Our study area included all the Fort Peck Reservation occupying roughly 1,456 sq miles. The geothermal water present in the Fort Peck Reservation is located in the western part of the Williston Basin in the Madison Group complex ranging in depths of 5500 to 7500 feet. Although no surface hot springs exist on the Reservation, water temperatures within oil wells that intercept these geothermal resources in the Madison Formation range from 150 to 278 degrees F.

  20. Anatomy of the Le Fort I segment: Are arterial variations a potential risk factor for avascular bone necrosis in Le Fort I osteotomies?

    Science.gov (United States)

    Bruneder, Simon; Wallner, Jürgen; Weiglein, Andreas; Kmečová, Ĺudmila; Egger, Jan; Pilsl, Ulrike; Zemann, Wolfgang

    2018-05-02

    Osteotomies of the Le Fort I segment are routine operations with low complication rates. Ischemic complications are rare, but can have severe consequences that may lead to avascular bone necrosis of the Le Fort I segment. Therefore the aim of this study was to investigate the blood supply and special arterial variants of the Le Fort I segment responsible for arterial hypoperfusion or ischemic avascular necrosis after surgery. The arterial anatomy of the Le Fort I segment's blood supply using 30 halved human cadaver head specimens was analyzed after complete dissection until the submicroscopic level. In all specimens the arterial variants of the Le Fort I segment and also the arterial diameters measured at two points were evaluated. The typical known vascularization pattern was apparent in 90% of all specimens, in which the ascending palatine (D1: 1,2 mm ± 0,34 mm; D2: 0,8 mm ± 0,34 mm) and ascending pharyngeal artery (D1: 1,3 mm ± 0,58 mm; D2: avascular segment necrosis after surgery. An individualized operation plan may prevent ischemic complications in at-risk patients. Copyright © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

  1. Geoscience Education and Cognition Research at George Mason University

    Science.gov (United States)

    Mattietti, G. K.; Peters, E. E.; Verardo, S.

    2009-12-01

    Cognition research in Geoscience is the focus of a small group of faculty from the College of Science and the College of Education and Human Development at George Mason University. We approached this research when we were involved in an Institution-wide effort to assess critical thinking, one of the competencies mandated for evaluation by the State Council of Higher Education of Virginia. Our group started spontaneously and informally from personal interests and enthusiasm for what and how our students are learning about Geology and in general about science. We want to understand what our students bring to the course, their attitude towards science, their knowledge of the scientific enterprise and preconceived ideas—and what our students take away from the course, beyond the course content. We believe that, with the support of cognitive science, we can improve the learning experience and therefore enhance the learning outcomes for science and non-science majors alike. Our Institution offers introductory Physical and Historical Geology classes populated primarily by non-science-major undergraduates. Geology lectures range in size from 90 to over 220 students per session per semester, with laboratory sessions averaging 27 students per session. With this large student population, it is necessary to use research tools that give us valuable information about student cognition, while being efficient in terms of time use and logistics. Some examples of our work include critical readings on Geoscience topics, surveys on students’ understanding of science as a way of knowing, exercises with built-in self-efficacy assessments, and concept mapping. The common denominator among these tools is that they are calibrated to address one or more of the higher levels in the revised Bloom’s Taxonomy of the Cognitive Domain, which form a complex assessment of student learning processes. These tools, once refined, can provide us with a better view of how our students learn in

  2. 2009 Federal Emergency Management Agency (FEMA) Topographic LiDAR: Fort Kent, Maine

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Camp Dresser McKee Inc. contracted with Sanborn Map Company to provide LiDAR mapping services for Fort Kent, Maine. Utilizing multi-return systems, Light Detection...

  3. Design Schematics for a Sustainable Parking Lot: Building 2-2332, ENRD Classroom, Fort Bragg, NC

    National Research Council Canada - National Science Library

    Stumpf, Annette

    2003-01-01

    ...) was tasked with planning a sustainable design "charrette" to explore and develop alternative parking lot designs that would meet Fort Bragg's parking needs, as well as its need to meet sustainable...

  4. Vendor Payments-Operation Mongoose, Fort Belvoir Defense Accounting Office and Rome Operating Location

    National Research Council Canada - National Science Library

    Lane, F

    1996-01-01

    .... Due to the impending closure of the Defense Accounting Office at Fort Belvoir and the anticipated consolidation to the Rome Operating Location, New York, we did not perform a review of the management...

  5. Final Sampling and Analysis Plan for Background Sampling, Fort Sheridan, Illinois

    National Research Council Canada - National Science Library

    1995-01-01

    .... This Background Sampling and Analysis Plan (BSAP) is designed to address this issue through the collection of additional background samples at Fort Sheridan to support the statistical analysis and the Baseline Risk Assessment (BRA...

  6. Freight Advanced Traveler Information System (FRATIS) - Dallas-Fort Worth (DFW) prototype : final report.

    Science.gov (United States)

    This is the Final Report for the FRATIS Dallas-Fort Worth DFW prototype system. The FRATIS prototype in : DFW consisted of the following components: optimization algorithm, terminal wait time, route specific : navigation/traffic/weather, and advanced...

  7. Notification: Hotline Complaint – Drinking Water Treatment Plant at the Fort Belknap Indian Community

    Science.gov (United States)

    Project #OA-FY13-0076, November 13, 2012. On March 22, 2012, the Office of Inspector General (OIG) received a hotline complaint on the construction of the Drinking Water Treatment Plant (DWTP) at the Fort Belknap Indian Community.

  8. Biological Assessment of Streams Associated with the Northern Training Complex at Fort knox, Kentucky, August 2000

    National Research Council Canada - National Science Library

    Payne, Berry

    2001-01-01

    .... The benthic macroinvertebrate aspect of the U.S. Environmental Protection Agency's Rapid Bioassessment Protocol was applied in August 2000 to selected streams likely to be affected by proposed improvements of training facilities on Fort Knox...

  9. 75 FR 24930 - Fort Bliss (Texas) Army Growth and Force Structure Realignment Final Environmental Impact...

    Science.gov (United States)

    2010-05-06

    ...-PWE, Building 624, Taylor Road, Fort Bliss, TX 79916-6812; e- mail: [email protected] . FOR... Regional Branch Library, 551 Redd Road. In Las Cruces (NM), the New Mexico State University Zuhl Library...

  10. Feasibility Study for an Off-Post, Primary Care Clinic at Fort Campbell, Kentucky

    National Research Council Canada - National Science Library

    Kvalevog, Kristen J

    2005-01-01

    .... Over 90,679 beneficiaries currently live in -the-Fort Campbell-catchment area and receive primary care at Blanchfield Army Community Hospital through the Red, White, Blue, Gold, and Young Eagle Clinics...

  11. Analyzing Street Art to Present the Heritage of George Town, Malaysia

    Directory of Open Access Journals (Sweden)

    Seyedehelham Sadatiseyedmahalleh

    2015-08-01

    Full Text Available Street art comprises all kinds of art developed in public locations. This form of art has spread to all heritage streets in George Town, the capital of Penang Island, which is also a known UNESCO World Heritage Site. However, instead of its heritage, food, and interesting past life and structure, the rapid multiplication of street art in George Town has created a new and unusual art that attracts tourists. Street art has spread to small roads and heritage streets in the city. This art increases the awareness of spectators by introducing them to the rich history of these streets and inducing a lively concept in the inner city of George Town. Accordingly, the impact of iron rod sculptures and murals, which comprises approximately 90% of street art in George Town, were determined to explore and identify the effects of this street art on this world heritage site. The findings were obtained from 263 out of 306 questionnaires accomplished by tourists in George Town. The results confirm that tourists prefer murals over iron rod sculptures. Iron rod sculptures are designed to tell the history of the street, whereas murals are designed to create aesthetic value. The findings indicated that similar to iron rod sculptures, more control should be exercised on the subjects of murals.

  12. Prader-Willi syndrome and Tay-Sachs disease in association with mixed maternal uniparental isodisomy and heterodisomy 15 in a girl who also had isochromosome Xq.

    Science.gov (United States)

    Zeesman, Susan; McCready, Elizabeth; Sadikovic, Bekim; Nowaczyk, Małgorzata Jm

    2015-01-01

    Malsegregation of chromosomes during reproduction can result in uniparental disomy when associated with trisomy rescue, monosomy rescue or gamete complementation. Pathogenicity stemming from uniparental disomy in liveborns results from imprinting disorders or autozygosity for autosomal recessive disorders. We report on a girl with Prader-Willi syndrome and Tay-Sachs disease resulting from maternal uniparental disomy of chromosome 15. The child also had an isochromosome Xq. To further characterize the etiology of the aberrant chromosome 15 and the isochromosome Xq, SNP loci from both chromosomes were assessed in the proband and parents, and genome-wide DNA methylation analysis was performed. SNP and DNA methylation analysis confirmed maternal uniparental heterodisomy around the Prader-Willi locus, while the region around the HEXA locus showed maternal uniparental isodisomy. This result is consistent with trisomy rescue of a maternal meiosis l error in a chromosome 15 with two meiotic recombinations. SNP analysis of the X chromosomes is consistent with a maternal origin for the isochromosome. © 2014 Wiley Periodicals, Inc.

  13. Molecular analysis of HEXA gene in Argentinean patients affected with Tay-Sachs disease: possible common origin of the prevalent c.459+5A>G mutation.

    Science.gov (United States)

    Zampieri, Stefania; Montalvo, Annalisa; Blanco, Mariana; Zanin, Irene; Amartino, Hernan; Vlahovicek, Kristian; Szlago, Marina; Schenone, Andrea; Pittis, Gabriela; Bembi, Bruno; Dardis, Andrea

    2012-05-15

    Tay-Sachs disease (TSD) is a recessively inherited disorder caused by the deficient activity of hexosaminidase A due to mutations in the HEXA gene. Up to date there is no information regarding the molecular genetics of TSD in Argentinean patients. In the present study we have studied 17 Argentinean families affected by TSD, including 20 patients with the acute infantile form and 3 with the sub-acute form. Overall, we identified 14 different mutations accounting for 100% of the studied alleles. Eight mutations were novel: 5 were single base changes leading to drastic residue changes or truncated proteins, 2 were small deletions and one was an intronic mutation that may cause a splicing defect. Although the spectrum of mutations was highly heterogeneous, a high frequency of the c.459+5G>A mutation, previously described in different populations was found among the studied cohort. Haplotype analysis suggested that in these families the c.459+5G>A mutation might have arisen by a single mutational event. Copyright © 2012 Elsevier B.V. All rights reserved.

  14. Site Investigations with the Site Characterization and Analysis Penetrator System at Fort Dix, New Jersey

    Science.gov (United States)

    1993-07-01

    rod system or through a tremie tube ; both procedures were used interchangeably at Fort Dix to demon- strate the efficiency and effectiveness of each...allows delivery through either a l/4-in.-diam grout tube or a 3/8-in.-diam rout tube . The grout used at Fort Dix consisted of a mixture of water and... microfine , blended Portland cement (Lehigh Geocem’, Leeds, Alabama). The grout is a suspension of a uniformly produced cement clinker interground with

  15. Cannon Fire Soon to Accompany Bugle Call at Fort Detrick | Poster

    Science.gov (United States)

    Beginning June 14, the familiar bugle calls at Fort Detrick will be joined by a special percussion instrument: a cannon. A single cannon shot will be fired on the first note of “Reveille,” which signals the start of each day and is accompanied by the raising of the American flag. “Reveille” sounds at 6:30 a.m. At 5 p.m., Fort Detrick plays “Retreat,” which alerts the post that

  16. Inpatient Behavioral Health Recapture A Busiess Case Analysis at Evans Army Community Hospital Fort Carson, Colorado

    Science.gov (United States)

    2009-07-20

    and Obstetrics /Gynecology. Inpatient care includes Obstetrics , Intensive Care, and Post Anesthesia Care/Same Day Surgery. EACH Mission: Delivering...charged with murder in Iraq shooting deaths, 2009). EACH Inpt Psych 13 Fort Carson has not been immune to the increase in suicides and violence among...to identify Soldiers with PTSD symptoms. In 2008, however, attention returned to Fort Carson as a number of local homicides and other violence tied

  17. How Do Le Fort-Type Fractures Present in a Pediatric Cohort?

    Science.gov (United States)

    Macmillan, Alexandra; Lopez, Joseph; Luck, J D; Faateh, Muhammad; Manson, Paul; Dorafshar, Amir H

    2018-05-01

    Le Fort-type fractures are very rare in children, and there is a paucity of literature presenting their frequency and characteristics. The purpose of this study was to determine the etiology, frequency, and fracture patterns of children with severe facial trauma associated with pterygoid plate fractures in a pediatric cohort. We performed a retrospective cohort study of all children aged younger than 16 years with pterygoid plate and facial fractures who presented to our institute between 1990 and 2010. Patient charts and radiologic records were reviewed for demographic and fracture characteristics. Patients were categorized into 2 groups as per facial fracture pattern: non-Le Fort-type fractures (group A) and Le Fort-type fractures (group B). Other variables including dentition age, frontal sinus development, mechanism of injury, injury severity, and concomitant injuries were recorded. Univariate methods were used to compare groups. We identified 24 children; 25% were girls, and 20.8% were of nonwhite race. Most presented with Le Fort-type fracture patterns (group B, 66.7%). Age was significantly different between group A and group B (mean, 5.9 years and 9.9 years, respectively; P = .009). No significant differences in Injury Severity Score, rate of operative repair, and length of stay were found between groups. Most children with severe facial fractures and pterygoid plate fractures presented with Le Fort-type fracture patterns in our cohort. The mean age of children with Le Fort-type fractures was greater than in those with non-Le Fort-type patterns. However, Le Fort-type fractures did occur in younger children with deciduous and mixed dentition. Copyright © 2017 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  18. Rumlige fortællinger fra mobilt og web-baseret GIS

    DEFF Research Database (Denmark)

    Møller-Jensen, Lasse

    2009-01-01

    Denne artikel handler om begrebet rumlige fortællinger med anvendelse af fortællingshenvisninger, og disses potentielle rolle ved implementation af fleksible og tematiske turistinformationssystemer. Artiklen fokuserer på brugen af mobile, positionsbekendte enheder, såsom visse PDA'er og smartphon......, samt på web-gis. Der præsenteres to anvendelseseksempler: et fra det centrale København og et fra et område nær Accra, Ghana....

  19. Habitat Restoration/Enhancement Fort Hall Reservation : 2008 Annual Report.

    Energy Technology Data Exchange (ETDEWEB)

    Osborne, Hunter [Shoshone Bannock Tribes

    2009-07-23

    Habitat enhancement, protection and monitoring were the focus of the Resident Fisheries Program during 2008. Enhancement and protection included sloping, fencing and planting wetlands plugs at sites on Spring Creek (Head-waters). Many previously constructed instream structures (rock barbs and wing dams) were repaired throughout the Fort Hall Indian Reservation (Reservation). Physical sampling during 2008 included sediment and depth measurements (SADMS) in Spring Creek at the Car Removal site. SADMS, used to track changes in channel morphology and specifically track movements of silt through Bottoms stream systems were completed for 5 strata on Spring Creek. Water temperature and chemistry were monitored monthly on Spring Creek, Clear Creek, Diggie Creek, and Portneuf (Jimmy Drinks) and Blackfoot rivers. Fish population densities and biomass were sampled in five reservation streams which included nine sites. Sampling protocols were identical to methods used in past years. Numbers of fish in Spring Creek series remained relatively low, however, there was an increase of biomass overall since 1993. Salmonid fry densities were monitored near Broncho Bridge and were similar to 2006, and 2007, however, as in years past, high densities of macrophytes make it very difficult to see fry in addition to lack of field technicians. Mean catch rate by anglers on Bottoms streams stayed the same as 2007 at 1.5/hr. Numbers of fish larger than 18-inches caught by anglers increased from 2007 at .20 to .26/hr.

  20. Fate of TCE in heated Fort Lewis soil.

    Science.gov (United States)

    Costanza, Jed; Fletcher, Kelly E; Löffler, Frank E; Pennell, Kurt D

    2009-02-01

    This study explores the transformation of trichloroethene (TCE) caused by heating contaminated soil and groundwater samples obtained from the East Gate Disposal Yard (EGDY) located in Fort Lewis, WA. After field samples transferring into glass ampules and introducing 1.5 micromol of TCE, the sealed ampules were incubated at temperatures of 25, 50, and 95 degrees C for periods of up to 95.5 days. Although TCE was completely transformed into cis-1,2-dichloroethene (cis-DCE) after 42 days at 25 degrees C by microbial activity, this transformation was not observed at 50 or 95 degrees C. Chloride levels increased after 42 days at 25 degrees C corresponding to the mass of TCE transformed to cis-DCE, were constant at 50 degrees C, and increased at 95 degrees C yielding a TCE degradation half-life of 1.6-1.9 years. These findings indicate that indigenous microbes contribute to the partial dechlorination of TCE to cis-DCE at temperatures of less than 50 degrees C, whereas interphase mass transfer and physical recovery of TCE will predominate over in situ degradation processes at temperatures of greater than 50 degrees C during thermal treatment at the EGDY site.