Institution
Pierre-and-Marie-Curie University
Education•Paris, France•
About: Pierre-and-Marie-Curie University is a education organization based out in Paris, France. It is known for research contribution in the topics: Population & Raman spectroscopy. The organization has 34448 authors who have published 56139 publications receiving 2392398 citations.
Topics: Population, Raman spectroscopy, Catalysis, Context (language use), Gene
Papers published on a yearly basis
Papers
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TL;DR: A novel 40-amino acid toxin from tarantula venom is described, which potently blocks a particular subclass of ASIC channels that are highly expressed in both central nervous system neurons and sensory neurons from dorsal root ganglia.
440 citations
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TL;DR: Au catalysts with different metallic particle sizes and supported on silica, alumina, titania, zirconia, ceria, and niobia were prepared, and the reduced catalysts were characterized by EXAFS spectroscopy.
440 citations
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TL;DR: Results demonstrate that in the immature rat more than 50% of PCs are each innervated by at least two distinct CFs; later on, the disappearance of the supernumerary synapses between CF and PC leads, as early as day 15, to the one-to-one relationship betweenCF and PC.
Abstract: Climbing fiber (CF)–-Purkinje cell (PC) relationships were studied electrophysiologically on the cerebellum of 8 to 15 day old rats. Some animals were rendered agranular by x-irradiation from birth; some others were treated with 3-acetyl pyridine 3 days before study to selectively destroy the CF. Unitary extracellular recordings in 8–9 day old normal rats revealed that more than 50μ of the PC units each exhibited either two types of all-or-none climbing fiber responses (CFR) or stepwise graded CFRs. The other PC units only presented one type of all-or-none CFR. These activities were entirely mediated via CF since they persisted at the same age in x-irradiated rats, but were absent in animals treated with 3-acetyl pyridine. Interaction experiments were performed between juxtafastigial and Inferior Olive stimulations on 49 PC units in 8–9 day old normal rats. Collisions between impulses set up in CFs were disclosed in 21 out of the 24 PCs which exhibited only one type of CFR. In the three others and in each of the 25 PCs that displayed two types of all-or-none CFRs, or CFRs graded by steps, no collision was detected. Moreover intracellular recordings of epsp's mediated via CFs in PCs of 8–9 day old normal rats revealed that they frequently fluctuated in stepwise fashion. These results demonstrate that in the immature rat more than 50μ of PCs are each innervated by at least two distinct CFs; later on, the disappearance of the supernumerary synapses between CF and PC leads, as early as day 15, to the one-to-one relationship between CF and PC.
438 citations
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University of Manchester1, George Washington University2, Bradford Royal Infirmary3, Université libre de Bruxelles4, Hospital Italiano de Buenos Aires5, Kaiser Permanente6, Technion – Israel Institute of Technology7, University of Barcelona8, University of Pavia9, Marshfield Clinic10, University of Toronto11, University of Paris12, University Hospitals Coventry and Warwickshire NHS Trust13, University of Angers14, University of Pisa15, University of Liverpool16, McGill University17, French Institute of Health and Medical Research18, University of Oxford19, University of Santiago de Compostela20, St Mary's Hospital21, University of Colorado Boulder22, NHS Ayrshire and Arran23, University of Udine24, University Hospitals of Leicester NHS Trust25, University of Sydney26, Katholieke Universiteit Leuven27, Istituto Giannina Gaslini28, Monash University29, University of Brescia30, Leeds General Infirmary31, Belfast Health and Social Care Trust32, University of Nantes33, Kocaeli University34, Temple University35, Boston Children's Hospital36, University of Paris-Sud37, University of Greifswald38, HealthPartners39, Guy's and St Thomas' NHS Foundation Trust40, University of Helsinki41, Royal Children's Hospital42, University of São Paulo43, Pierre-and-Marie-Curie University44, Princess Margaret Hospital for Children45, Amrita Vishwa Vidyapeetham46, Aarhus University47, University of British Columbia48, Rikshospitalet–Radiumhospitalet49, University of Milan50, University of Liège51, Mater Dei Hospital52, Karolinska Institutet53, Tel Aviv University54, University of Utah55, Nottingham University Hospitals NHS Trust56, University of Basel57, University of Melbourne58, University Hospital of Wales59, Christian Medical College & Hospital60, Casa Sollievo della Sofferenza61, Ghent University62, VU University Amsterdam63, Mount Sinai St. Luke's and Mount Sinai Roosevelt64, University of Nottingham65, McMaster University66, University of Glasgow67
TL;DR: A robust relationship between mutations in all seven genes with increased type I interferon activity in cerebrospinal fluid and serum, and the increased expression of interferOn‐stimulated gene transcripts in peripheral blood is observed.
Abstract: Aicardi-Goutieres syndrome is an inflammatory disease occurring due to mutations in any of TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR or IFIH1. We report on 374 patients from 299 families with mutations in these seven genes. Most patients conformed to one of two fairly stereotyped clinical profiles; either exhibiting an in utero disease-onset (74 patients; 22.8% of all patients where data were available), or a post-natal presentation, usually within the first year of life (223 patients; 68.6%), characterized by a sub-acute encephalopathy and a loss of previously acquired skills. Other clinically distinct phenotypes were also observed; particularly, bilateral striatal necrosis (13 patients; 3.6%) and non-syndromic spastic paraparesis (12 patients; 3.4%). We recorded 69 deaths (19.3% of patients with follow-up data). Of 285 patients for whom data were available, 210 (73.7%) were profoundly disabled, with no useful motor, speech and intellectual function. Chilblains, glaucoma, hypothyroidism, cardiomyopathy, intracerebral vasculitis, peripheral neuropathy, bowel inflammation and systemic lupus erythematosus were seen frequently enough to be confirmed as real associations with the Aicardi-Goutieres syndrome phenotype. We observed a robust relationship between mutations in all seven genes with increased type I interferon activity in cerebrospinal fluid and serum, and the increased expression of interferon-stimulated gene transcripts in peripheral blood. We recorded a positive correlation between the level of cerebrospinal fluid interferon activity assayed within one year of disease presentation and the degree of subsequent disability. Interferon-stimulated gene transcripts remained high in most patients, indicating an ongoing disease process. On the basis of substantial morbidity and mortality, our data highlight the urgent need to define coherent treatment strategies for the phenotypes associated with mutations in the Aicardi-Goutieres syndrome-related genes. Our findings also make it clear that a window of therapeutic opportunity exists relevant to the majority of affected patients and indicate that the assessment of type I interferon activity might serve as a useful biomarker in future clinical trials.
437 citations
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TL;DR: A review of available information from the literature on the relationship between developmental instability and various fitness components such as growth, fecundity, and longevity suggests that there indeed is a general negative relationship.
Abstract: Developmental stability reflects the ability of individuals to undergo stable development of their phenotype under a range of environmental conditions. Developmental instability is measured in terms of fluctuating asymmetry or phenodeviance. A negative relationship between developmental instability and fitness has figured as a prominent untested assumption in the literature. A review of available information from the literature on the relationship between developmental instability and various fitness components such as growth, fecundity, and longevity suggests that there indeed is a general negative relationship. Symmetrical individuals do generally have faster growth, higher fecundity, and better survival than do more asymmetrical individuals. These differences appear partially to arise from lower competitive ability and higher risks of predation and parasitism of asymmetrical individuals compared with more symmetrical conspecifics. The relationship between developmental stability and fitness ma...
437 citations
Authors
Showing all 34671 results
Name | H-index | Papers | Citations |
---|---|---|---|
Zhong Lin Wang | 245 | 2529 | 259003 |
Guido Kroemer | 236 | 1404 | 246571 |
Krzysztof Matyjaszewski | 169 | 1431 | 128585 |
J. E. Brau | 162 | 1949 | 157675 |
E. Hivon | 147 | 403 | 118440 |
Kazuhiko Hara | 141 | 1956 | 107697 |
Simon Prunet | 141 | 434 | 96314 |
H. J. McCracken | 140 | 579 | 71091 |
G. Calderini | 139 | 1734 | 102408 |
Stefano Giagu | 139 | 1651 | 101569 |
Jean-Paul Kneib | 138 | 805 | 89287 |
G. Marchiori | 137 | 1590 | 94277 |
J. Ocariz | 136 | 1562 | 95905 |
Jean-Marie Tarascon | 136 | 853 | 137673 |
Alexis Brice | 135 | 870 | 83466 |