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- Publication . Article . 2016Open Access EnglishAuthors:Besma Aouar; Denisa Kovarova; Sébastien Letard; Albert Font-Haro; Jonathan Florentin; Jan Weber; David Durantel; Laurence Chaperot; Joel Plumas; Katerina Trejbalova; +6 moreBesma Aouar; Denisa Kovarova; Sébastien Letard; Albert Font-Haro; Jonathan Florentin; Jan Weber; David Durantel; Laurence Chaperot; Joel Plumas; Katerina Trejbalova; Jiri Hejnar; Jacques A. Nunès; Daniel Olive; Patrice Dubreuil; Ivan Hirsch; Ruzena Wiersum Stranska;Publisher: HAL CCSDCountry: France
International audience; Crosslinking of regulatory immunoreceptors (RR), such as BDCA-2 (CD303) or ILT7 (CD85g), of plasmacytoid dendritic cells (pDCs) efficiently suppresses production of type-I interferon (IFN)-α/β and other cytokines in response to Toll-like receptor (TLR) 7/9 ligands. This cytokine-inhibitory pathway is mediated by spleen tyrosine kinase (Syk) associated with the ITAM-containing adapter of RR. Here we demonstrate by pharmacological targeting of Syk that in addition to the negative regulation of TLR7/9 signaling via RR, Syk also positively regulates the TLR7/9 pathway in human pDCs. Novel highly specific Syk inhibitor AB8779 suppressed IFN-α, TNF-α and IL-6 production induced by TLR7/9 agonists in primary pDCs and in the pDC cell line GEN2.2. Triggering of TLR9 or RR signaling induced a differential kinetics of phosphorylation at Y352 and Y525/526 of Syk and a differential sensitivity to AB8779. Consistent with the different roles of Syk in TLR7/9 and RR signaling, a concentration of AB8779 insufficient to block TLR7/9 signaling still released the block of IFN-α production triggered via the RR pathway, including that induced by hepatitis B and C viruses. Thus, pharmacological targeting of Syk partially restored the main pDC function-IFN-α production. Opposing roles of Syk in TLR7/9 and RR pathways may regulate the innate immune response to weaken inflammation reaction.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . 2019Open Access EnglishAuthors:Schmidt, Robert; Ruiz, Maria; Kilavik, Bjørg; Lundqvist, X; Philip, X; Starr, A; Aron, Adam;Schmidt, Robert; Ruiz, Maria; Kilavik, Bjørg; Lundqvist, X; Philip, X; Starr, A; Aron, Adam;Publisher: HAL CCSDCountry: FranceProject: EC | HBP SGA2 (785907), NIH | Closed Loop Deep Brain St... (5UH3NS100544-04), NIH | Cortical Circuits for Att... (5R37MH087027-02), EC | HBP SGA1 (720270), NIH | The motor network in Park... (5R01NS090913-06)
International audience; Beta oscillations (ϳ13 to 30 Hz) have been observed during many perceptual, cognitive, and motor processes in a plethora of brain recording studies. Although the function of beta oscillations (hereafter "beta" for short) is unlikely to be explained by any single monolithic description, we here discuss several convergent findings. In prefrontal cortex (PFC), increased beta appears at the end of a trial when working memory information needs to be erased. A similar "clear-out" function might apply during the stopping of action and the stopping of long-term memory retrieval (stopping thoughts), where increased prefrontal beta is also observed. A different apparent role for beta in PFC occurs during the delay period of working memory tasks: it might serve to maintain the current contents and/or to prevent interference from distraction. We confront the challenge of relating these observations to the large literature on beta recorded from sensorimotor cortex. Potentially, the clear-out of working memory in PFC has its counterpart in the postmovement clear-out of the motor plan in sensorimotor cortex. However, recent studies support alternative interpretations. In addition, we flag emerging research on different frequencies of beta and the relationship between beta and single-neuron spiking. We also discuss where beta might be generated: basal ganglia, cortex, or both. We end by considering the clinical implications for adaptive deep-brain stimulation.
- Publication . Article . 2015Open Access EnglishAuthors:Tomas Ekeberg; Martin Svenda; Chantal Abergel; Filipe R. N. C. Maia; Virginie Seltzer; Jean-Michel Claverie; Max F. Hantke; Olof Jönsson; Carl Nettelblad; Gijs van der Schot; +19 moreTomas Ekeberg; Martin Svenda; Chantal Abergel; Filipe R. N. C. Maia; Virginie Seltzer; Jean-Michel Claverie; Max F. Hantke; Olof Jönsson; Carl Nettelblad; Gijs van der Schot; Mengning Liang; Daniel P. DePonte; Anton Barty; M. Marvin Seibert; Bianca Iwan; Inger Andersson; N. Duane Loh; Andrew V. Martin; Henry N. Chapman; Christoph Bostedt; John D. Bozek; Ken R. Ferguson; Jacek Krzywinski; Sascha W. Epp; Daniel Rolles; Artem Rudenko; Robert Hartmann; Nils Kimmel; Janos Hajdu;Publisher: HAL CCSDCountries: Germany, France, France, Sweden
International audience; We present a proof-of-concept three-dimensional reconstruction of the giant mimivirus particle from experimentally measured diffraction patterns from an x-ray free-electron laser. Three-dimensional imaging requires the assembly of many two-dimensional patterns into an internally consistent Fourier volume. Since each particle is randomly oriented when exposed to the x-ray pulse, relative orientations have to be retrieved from the diffraction data alone. We achieve this with a modified version of the expand, maximize and compress algorithm and validate our result using new methods.
Substantial popularitySubstantial popularity In top 1%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2018Closed AccessAuthors:Patrice Bourgeois; Clothilde Esteve; Charlene Chaix; Christophe Béroud; Nicolas Lévy; Alexandre Fabre; Catherine Badens;Patrice Bourgeois; Clothilde Esteve; Charlene Chaix; Christophe Béroud; Nicolas Lévy; Alexandre Fabre; Catherine Badens;
doi: 10.1002/humu.23418
pmid: 29527791
Publisher: Hindawi LimitedCountry: FranceInternational audience; Tricho-Hepato-Enteric syndrome (THES) is a very rare autosomal recessive syndromic enteropathy caused by mutations of either TTC37 or SKIV2L genes. Very little is known of these two gene products in mammals nor of the pathophysiology of the disease. Since the identification of the genes, we have set up the molecular diagnostic of THES in routine, gathering a large cohort with clinical and molecular data. Here, we report the phenotype and genotype analysis of this cohort together with an extensive literature review of THES cases worldwide, that is, 96 individuals harboring mutations in one gene or the other. We set up locus-specific databases for both genes and reviewed the type of mutation aswell as their localization in the proteins. No hot spot is evidenced for any type of mutation. The phenotypic analysis was first made on the whole cohort but is limited due to heterogeneity in clinical descriptions. We then examined the lab diagnostic cohort in detail for clinical manifestations. For the first time, we are able to suggest that patients lacking SKIV2L seem more severely affected than those lacking TTC37, in terms of liver damage and prenatal growth impairment.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2014Restricted EnglishAuthors:Jonathan Romiguier; Philippe Gayral; Marion Ballenghien; Aurélien Bernard; Vincent Cahais; Anne Chenuil; Ylenia Chiari; Rémy Dernat; Laurent Duret; Nicolas Faivre; +11 moreJonathan Romiguier; Philippe Gayral; Marion Ballenghien; Aurélien Bernard; Vincent Cahais; Anne Chenuil; Ylenia Chiari; Rémy Dernat; Laurent Duret; Nicolas Faivre; Etienne Loire; Joao M. Lourenco; Benoit Nabholz; Camille Roux; Georgia Tsagkogeorga; Alexandra Anh-Thu Weber; Lucy A. Weinert; Khalid Belkhir; Nicolas Bierne; Sylvain Glémin; Nicolas Galtier;
doi: 10.1038/nature13685
pmid: 25141177
Publisher: HAL CCSDCountry: FranceInternational audience; Genetic diversity is the amount of variation observed between DNA sequences from distinct individuals of a given species. This pivotal concept of population genetics has implications for species health, domest-ication, management and conservation. Levels of genetic diversity seem to vary greatly in natural populations and species, but the determinants of this variation, and particularly the relative influences of species biology and ecology versus population history, are still largely mysterious 1,2. Here we show that the diversity of a species is predictable , and is determined in the first place by its ecological strategy. We investigated the genome-wide diversity of 76 non-model animal species by sequencing the transcriptome of two to ten individuals in each species. The distribution of genetic diversity between species revealed no detectable influence of geographic range or invasive status but was accurately predicted by key species traits related to parental investment: long-lived or low-fecundity species with brooding ability were genetically less diverse than short-lived or highly fecund ones. Our analysis demonstrates the influence of long-term life-history strategies on species response to short-term environmental perturbations, a result with immediate implications for conservation policies. Since the early studies of evolutionary genetics, there has been no understanding of how and why genetic diversity levels vary between species. This old puzzle, considered four decades ago as 'the central problem in population genetics'
Substantial popularitySubstantial popularity In top 1%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . Other literature type . Preprint . 2012Open Access EnglishAuthors:Emmanouil Papastergis; Andrea Cattaneo; Shan Huang; Riccardo Giovanelli; Martha P. Haynes;Emmanouil Papastergis; Andrea Cattaneo; Shan Huang; Riccardo Giovanelli; Martha P. Haynes;Country: FranceProject: NSF | Harvesting ALFALFA: Track... (1107390), NSF | ALFALFA: The Arecibo Extr... (0607007)
We use both an HI-selected and an optically-selected galaxy sample to directly measure the abundance of galaxies as a function of their "baryonic" mass (stars + atomic gas). Stellar masses are calculated based on optical data from the Sloan Digital Sky Survey (SDSS) and atomic gas masses are calculated using atomic hydrogen (HI) emission line data from the Arecibo Legacy Fast ALFA (ALFALFA) survey. By using the technique of abundance matching, we combine the measured baryonic function (BMF) of galaxies with the dark matter halo mass function in a LCDM universe, in order to determine the galactic baryon fraction as a function of host halo mass. We find that the baryon fraction of low-mass halos is much smaller than the cosmic value, even when atomic gas is taken into account. We find that the galactic baryon deficit increases monotonically with decreasing halo mass, in contrast with previous studies which suggested an approximately constant baryon fraction at the low-mass end. We argue that the observed baryon fractions of low mass halos cannot be explained by reionization heating alone, and that additional feedback mechanisms (e.g. supernova blowout) must be invoked. However, the outflow rates needed to reproduce our result are not easily accommodated in the standard picture of galaxy formation in a LCDM universe. Comment: Accepted to ApJ. 45 pages (aastex), 19 figures; added references and updated fig.18 for version2
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2012Open Access EnglishAuthors:B. Dridi Rezgui; Fabrice Gourbilleau; D. Maestre; Olivier Palais; A. Sibai; Mustapha Lemiti; Georges Bremond;B. Dridi Rezgui; Fabrice Gourbilleau; D. Maestre; Olivier Palais; A. Sibai; Mustapha Lemiti; Georges Bremond;
doi: 10.1063/1.4737579
Publisher: HAL CCSDCountries: Spain, France, France, FranceInternational audience; Experimental investigation of photoconductivity in Si-rich silicon oxide (SRSO)/SiO2 multilayer (ML) structures prepared by magnetron reactive sputtering is reported. Photocurrent (PC) measurements show that the PC threshold increases with decreasing the thickness of SRSO layer. Photo-conduction processes in our samples are shown to be dominated by carrier transport through quantum-confined silicon nanocrystals embedded in the SiO2 host. In addition, the observed bias-dependence of photocurrent intensity is consistent with a model in which carrier transport occurs by both tunneling and hopping through defect states in the silicon oxide matrix. A photocurrent density Jph of 1–2 mA cm−2 is extracted from our results. Although this photocurrent density along the ML absorber film is relatively low, the results presented in this work are believed to be a valuable contribution toward the implementation of all-Si tandem solar cells.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2012Open Access EnglishAuthors:Bloen Metzger; Jason E. Butler;Bloen Metzger; Jason E. Butler;
doi: 10.1063/1.3685537
Publisher: HAL CCSDCountry: FranceInternational audience; We have investigated the time evolution of a cloud of non-Brownian particles sub- jected to a periodic shear flow in an otherwise pure liquid at low Reynolds number. This experiment illustrates the irreversible nature of particulate systems submitted to a shear. When repeating the cycles of shear, we have found that clouds of particles progressively disperse in the flow direction until reaching a threshold critical volume fraction that depends upon the strain amplitude; this critical volume fraction coin- cides with measurements of the threshold for reversibility found from experiments on homogeneous suspensions in periodic shear. Two distinct patterns, including a “galaxy-like” shape, are observed for the evolution of the clouds and the transi- tion between the patterns is identified using a simple scaling analysis. Movies are available with the online version of the paper.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Preprint . Article . Other literature type . 2021 . Embargo End Date: 01 Jan 2021Open AccessAuthors:K. Kiiveri; Daniel Gruen; Alexis Finoguenov; Thomas Erben; L. van Waerbeke; Eli S. Rykoff; Lance Miller; Steffen Hagstotz; R. A. Dupke; J. Patrick Henry; +12 moreK. Kiiveri; Daniel Gruen; Alexis Finoguenov; Thomas Erben; L. van Waerbeke; Eli S. Rykoff; Lance Miller; Steffen Hagstotz; R. A. Dupke; J. Patrick Henry; J. P. Kneib; Ghassem Gozaliasl; C. C. Kirkpatrick; N Cibirka; Nicolas Clerc; M. Costanzi; Eduardo Serra Cypriano; Eduardo Rozo; Huanyuan Shan; P. Spinelli; J. Valiviita; Jochen Weller;Publisher: arXivCountries: France, Switzerland, FinlandProject: AKA | Euclid Cosmology Mission ... (292882), AKA | The COSMOS legacy survey (266918), AKA | Euclid Cosmology Mission ... (295113)
The COnstrain Dark Energy with X-ray clusters (CODEX) sample contains the largest flux limited sample of X-ray clusters at $0.35 = \alpha \mu + \beta$, with $\mu = \ln (M_{200c}/M_{\mathrm{piv}})$, and $M_{\mathrm{piv}} = 10^{14.81} M_{\odot}$. We find a slope $\alpha = 0.49^{+0.20}_{-0.15}$, normalization $ \exp(\beta) = 84.0^{+9.2}_{-14.8}$ and $\sigma_{\ln \lambda | \mu} = 0.17^{+0.13}_{-0.09}$ using CFHT richness estimates. In comparison to other weak lensing richness-mass relations, we find the normalization of the richness statistically agreeing with the normalization of other scaling relations from a broad redshift range ($0.0
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2017Open Access FrenchAuthors:Yinan Zhou; Pierre Kaser;Yinan Zhou; Pierre Kaser;
doi: 10.4000/ideo.673
Publisher: HAL CCSDCountry: FrancePresentation Dernier rejeton d'une longue lignee d'homme de lettres, le jeune Zhang Dai 張岱 (1597-1681) connut l'opulence d'un riche lettre oisif, s'adonnant tout a loisir a sa passion pour la litterature et a son gout tres marque pour le theâtre avant de tout perdre au moment de la chute des Ming 明 (1644). Reste fidele a la defunte dynastie, il brava les interdits en en redigeant l'histoire. Depouille et traque, il eprouva la misere et la clandestinite, ce qui explique que la plupart de ses e...
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product.
11,949 Research products, page 1 of 1,195
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- Publication . Article . 2016Open Access EnglishAuthors:Besma Aouar; Denisa Kovarova; Sébastien Letard; Albert Font-Haro; Jonathan Florentin; Jan Weber; David Durantel; Laurence Chaperot; Joel Plumas; Katerina Trejbalova; +6 moreBesma Aouar; Denisa Kovarova; Sébastien Letard; Albert Font-Haro; Jonathan Florentin; Jan Weber; David Durantel; Laurence Chaperot; Joel Plumas; Katerina Trejbalova; Jiri Hejnar; Jacques A. Nunès; Daniel Olive; Patrice Dubreuil; Ivan Hirsch; Ruzena Wiersum Stranska;Publisher: HAL CCSDCountry: France
International audience; Crosslinking of regulatory immunoreceptors (RR), such as BDCA-2 (CD303) or ILT7 (CD85g), of plasmacytoid dendritic cells (pDCs) efficiently suppresses production of type-I interferon (IFN)-α/β and other cytokines in response to Toll-like receptor (TLR) 7/9 ligands. This cytokine-inhibitory pathway is mediated by spleen tyrosine kinase (Syk) associated with the ITAM-containing adapter of RR. Here we demonstrate by pharmacological targeting of Syk that in addition to the negative regulation of TLR7/9 signaling via RR, Syk also positively regulates the TLR7/9 pathway in human pDCs. Novel highly specific Syk inhibitor AB8779 suppressed IFN-α, TNF-α and IL-6 production induced by TLR7/9 agonists in primary pDCs and in the pDC cell line GEN2.2. Triggering of TLR9 or RR signaling induced a differential kinetics of phosphorylation at Y352 and Y525/526 of Syk and a differential sensitivity to AB8779. Consistent with the different roles of Syk in TLR7/9 and RR signaling, a concentration of AB8779 insufficient to block TLR7/9 signaling still released the block of IFN-α production triggered via the RR pathway, including that induced by hepatitis B and C viruses. Thus, pharmacological targeting of Syk partially restored the main pDC function-IFN-α production. Opposing roles of Syk in TLR7/9 and RR pathways may regulate the innate immune response to weaken inflammation reaction.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . 2019Open Access EnglishAuthors:Schmidt, Robert; Ruiz, Maria; Kilavik, Bjørg; Lundqvist, X; Philip, X; Starr, A; Aron, Adam;Schmidt, Robert; Ruiz, Maria; Kilavik, Bjørg; Lundqvist, X; Philip, X; Starr, A; Aron, Adam;Publisher: HAL CCSDCountry: FranceProject: EC | HBP SGA2 (785907), NIH | Closed Loop Deep Brain St... (5UH3NS100544-04), NIH | Cortical Circuits for Att... (5R37MH087027-02), EC | HBP SGA1 (720270), NIH | The motor network in Park... (5R01NS090913-06)
International audience; Beta oscillations (ϳ13 to 30 Hz) have been observed during many perceptual, cognitive, and motor processes in a plethora of brain recording studies. Although the function of beta oscillations (hereafter "beta" for short) is unlikely to be explained by any single monolithic description, we here discuss several convergent findings. In prefrontal cortex (PFC), increased beta appears at the end of a trial when working memory information needs to be erased. A similar "clear-out" function might apply during the stopping of action and the stopping of long-term memory retrieval (stopping thoughts), where increased prefrontal beta is also observed. A different apparent role for beta in PFC occurs during the delay period of working memory tasks: it might serve to maintain the current contents and/or to prevent interference from distraction. We confront the challenge of relating these observations to the large literature on beta recorded from sensorimotor cortex. Potentially, the clear-out of working memory in PFC has its counterpart in the postmovement clear-out of the motor plan in sensorimotor cortex. However, recent studies support alternative interpretations. In addition, we flag emerging research on different frequencies of beta and the relationship between beta and single-neuron spiking. We also discuss where beta might be generated: basal ganglia, cortex, or both. We end by considering the clinical implications for adaptive deep-brain stimulation.
- Publication . Article . 2015Open Access EnglishAuthors:Tomas Ekeberg; Martin Svenda; Chantal Abergel; Filipe R. N. C. Maia; Virginie Seltzer; Jean-Michel Claverie; Max F. Hantke; Olof Jönsson; Carl Nettelblad; Gijs van der Schot; +19 moreTomas Ekeberg; Martin Svenda; Chantal Abergel; Filipe R. N. C. Maia; Virginie Seltzer; Jean-Michel Claverie; Max F. Hantke; Olof Jönsson; Carl Nettelblad; Gijs van der Schot; Mengning Liang; Daniel P. DePonte; Anton Barty; M. Marvin Seibert; Bianca Iwan; Inger Andersson; N. Duane Loh; Andrew V. Martin; Henry N. Chapman; Christoph Bostedt; John D. Bozek; Ken R. Ferguson; Jacek Krzywinski; Sascha W. Epp; Daniel Rolles; Artem Rudenko; Robert Hartmann; Nils Kimmel; Janos Hajdu;Publisher: HAL CCSDCountries: Germany, France, France, Sweden
International audience; We present a proof-of-concept three-dimensional reconstruction of the giant mimivirus particle from experimentally measured diffraction patterns from an x-ray free-electron laser. Three-dimensional imaging requires the assembly of many two-dimensional patterns into an internally consistent Fourier volume. Since each particle is randomly oriented when exposed to the x-ray pulse, relative orientations have to be retrieved from the diffraction data alone. We achieve this with a modified version of the expand, maximize and compress algorithm and validate our result using new methods.
Substantial popularitySubstantial popularity In top 1%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2018Closed AccessAuthors:Patrice Bourgeois; Clothilde Esteve; Charlene Chaix; Christophe Béroud; Nicolas Lévy; Alexandre Fabre; Catherine Badens;Patrice Bourgeois; Clothilde Esteve; Charlene Chaix; Christophe Béroud; Nicolas Lévy; Alexandre Fabre; Catherine Badens;
doi: 10.1002/humu.23418
pmid: 29527791
Publisher: Hindawi LimitedCountry: FranceInternational audience; Tricho-Hepato-Enteric syndrome (THES) is a very rare autosomal recessive syndromic enteropathy caused by mutations of either TTC37 or SKIV2L genes. Very little is known of these two gene products in mammals nor of the pathophysiology of the disease. Since the identification of the genes, we have set up the molecular diagnostic of THES in routine, gathering a large cohort with clinical and molecular data. Here, we report the phenotype and genotype analysis of this cohort together with an extensive literature review of THES cases worldwide, that is, 96 individuals harboring mutations in one gene or the other. We set up locus-specific databases for both genes and reviewed the type of mutation aswell as their localization in the proteins. No hot spot is evidenced for any type of mutation. The phenotypic analysis was first made on the whole cohort but is limited due to heterogeneity in clinical descriptions. We then examined the lab diagnostic cohort in detail for clinical manifestations. For the first time, we are able to suggest that patients lacking SKIV2L seem more severely affected than those lacking TTC37, in terms of liver damage and prenatal growth impairment.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2014Restricted EnglishAuthors:Jonathan Romiguier; Philippe Gayral; Marion Ballenghien; Aurélien Bernard; Vincent Cahais; Anne Chenuil; Ylenia Chiari; Rémy Dernat; Laurent Duret; Nicolas Faivre; +11 moreJonathan Romiguier; Philippe Gayral; Marion Ballenghien; Aurélien Bernard; Vincent Cahais; Anne Chenuil; Ylenia Chiari; Rémy Dernat; Laurent Duret; Nicolas Faivre; Etienne Loire; Joao M. Lourenco; Benoit Nabholz; Camille Roux; Georgia Tsagkogeorga; Alexandra Anh-Thu Weber; Lucy A. Weinert; Khalid Belkhir; Nicolas Bierne; Sylvain Glémin; Nicolas Galtier;
doi: 10.1038/nature13685
pmid: 25141177
Publisher: HAL CCSDCountry: FranceInternational audience; Genetic diversity is the amount of variation observed between DNA sequences from distinct individuals of a given species. This pivotal concept of population genetics has implications for species health, domest-ication, management and conservation. Levels of genetic diversity seem to vary greatly in natural populations and species, but the determinants of this variation, and particularly the relative influences of species biology and ecology versus population history, are still largely mysterious 1,2. Here we show that the diversity of a species is predictable , and is determined in the first place by its ecological strategy. We investigated the genome-wide diversity of 76 non-model animal species by sequencing the transcriptome of two to ten individuals in each species. The distribution of genetic diversity between species revealed no detectable influence of geographic range or invasive status but was accurately predicted by key species traits related to parental investment: long-lived or low-fecundity species with brooding ability were genetically less diverse than short-lived or highly fecund ones. Our analysis demonstrates the influence of long-term life-history strategies on species response to short-term environmental perturbations, a result with immediate implications for conservation policies. Since the early studies of evolutionary genetics, there has been no understanding of how and why genetic diversity levels vary between species. This old puzzle, considered four decades ago as 'the central problem in population genetics'
Substantial popularitySubstantial popularity In top 1%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . Other literature type . Preprint . 2012Open Access EnglishAuthors:Emmanouil Papastergis; Andrea Cattaneo; Shan Huang; Riccardo Giovanelli; Martha P. Haynes;Emmanouil Papastergis; Andrea Cattaneo; Shan Huang; Riccardo Giovanelli; Martha P. Haynes;Country: FranceProject: NSF | Harvesting ALFALFA: Track... (1107390), NSF | ALFALFA: The Arecibo Extr... (0607007)
We use both an HI-selected and an optically-selected galaxy sample to directly measure the abundance of galaxies as a function of their "baryonic" mass (stars + atomic gas). Stellar masses are calculated based on optical data from the Sloan Digital Sky Survey (SDSS) and atomic gas masses are calculated using atomic hydrogen (HI) emission line data from the Arecibo Legacy Fast ALFA (ALFALFA) survey. By using the technique of abundance matching, we combine the measured baryonic function (BMF) of galaxies with the dark matter halo mass function in a LCDM universe, in order to determine the galactic baryon fraction as a function of host halo mass. We find that the baryon fraction of low-mass halos is much smaller than the cosmic value, even when atomic gas is taken into account. We find that the galactic baryon deficit increases monotonically with decreasing halo mass, in contrast with previous studies which suggested an approximately constant baryon fraction at the low-mass end. We argue that the observed baryon fractions of low mass halos cannot be explained by reionization heating alone, and that additional feedback mechanisms (e.g. supernova blowout) must be invoked. However, the outflow rates needed to reproduce our result are not easily accommodated in the standard picture of galaxy formation in a LCDM universe. Comment: Accepted to ApJ. 45 pages (aastex), 19 figures; added references and updated fig.18 for version2
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2012Open Access EnglishAuthors:B. Dridi Rezgui; Fabrice Gourbilleau; D. Maestre; Olivier Palais; A. Sibai; Mustapha Lemiti; Georges Bremond;B. Dridi Rezgui; Fabrice Gourbilleau; D. Maestre; Olivier Palais; A. Sibai; Mustapha Lemiti; Georges Bremond;
doi: 10.1063/1.4737579
Publisher: HAL CCSDCountries: Spain, France, France, FranceInternational audience; Experimental investigation of photoconductivity in Si-rich silicon oxide (SRSO)/SiO2 multilayer (ML) structures prepared by magnetron reactive sputtering is reported. Photocurrent (PC) measurements show that the PC threshold increases with decreasing the thickness of SRSO layer. Photo-conduction processes in our samples are shown to be dominated by carrier transport through quantum-confined silicon nanocrystals embedded in the SiO2 host. In addition, the observed bias-dependence of photocurrent intensity is consistent with a model in which carrier transport occurs by both tunneling and hopping through defect states in the silicon oxide matrix. A photocurrent density Jph of 1–2 mA cm−2 is extracted from our results. Although this photocurrent density along the ML absorber film is relatively low, the results presented in this work are believed to be a valuable contribution toward the implementation of all-Si tandem solar cells.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2012Open Access EnglishAuthors:Bloen Metzger; Jason E. Butler;Bloen Metzger; Jason E. Butler;
doi: 10.1063/1.3685537
Publisher: HAL CCSDCountry: FranceInternational audience; We have investigated the time evolution of a cloud of non-Brownian particles sub- jected to a periodic shear flow in an otherwise pure liquid at low Reynolds number. This experiment illustrates the irreversible nature of particulate systems submitted to a shear. When repeating the cycles of shear, we have found that clouds of particles progressively disperse in the flow direction until reaching a threshold critical volume fraction that depends upon the strain amplitude; this critical volume fraction coin- cides with measurements of the threshold for reversibility found from experiments on homogeneous suspensions in periodic shear. Two distinct patterns, including a “galaxy-like” shape, are observed for the evolution of the clouds and the transi- tion between the patterns is identified using a simple scaling analysis. Movies are available with the online version of the paper.
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Preprint . Article . Other literature type . 2021 . Embargo End Date: 01 Jan 2021Open AccessAuthors:K. Kiiveri; Daniel Gruen; Alexis Finoguenov; Thomas Erben; L. van Waerbeke; Eli S. Rykoff; Lance Miller; Steffen Hagstotz; R. A. Dupke; J. Patrick Henry; +12 moreK. Kiiveri; Daniel Gruen; Alexis Finoguenov; Thomas Erben; L. van Waerbeke; Eli S. Rykoff; Lance Miller; Steffen Hagstotz; R. A. Dupke; J. Patrick Henry; J. P. Kneib; Ghassem Gozaliasl; C. C. Kirkpatrick; N Cibirka; Nicolas Clerc; M. Costanzi; Eduardo Serra Cypriano; Eduardo Rozo; Huanyuan Shan; P. Spinelli; J. Valiviita; Jochen Weller;Publisher: arXivCountries: France, Switzerland, FinlandProject: AKA | Euclid Cosmology Mission ... (292882), AKA | The COSMOS legacy survey (266918), AKA | Euclid Cosmology Mission ... (295113)
The COnstrain Dark Energy with X-ray clusters (CODEX) sample contains the largest flux limited sample of X-ray clusters at $0.35 = \alpha \mu + \beta$, with $\mu = \ln (M_{200c}/M_{\mathrm{piv}})$, and $M_{\mathrm{piv}} = 10^{14.81} M_{\odot}$. We find a slope $\alpha = 0.49^{+0.20}_{-0.15}$, normalization $ \exp(\beta) = 84.0^{+9.2}_{-14.8}$ and $\sigma_{\ln \lambda | \mu} = 0.17^{+0.13}_{-0.09}$ using CFHT richness estimates. In comparison to other weak lensing richness-mass relations, we find the normalization of the richness statistically agreeing with the normalization of other scaling relations from a broad redshift range ($0.0
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product. - Publication . Article . 2017Open Access FrenchAuthors:Yinan Zhou; Pierre Kaser;Yinan Zhou; Pierre Kaser;
doi: 10.4000/ideo.673
Publisher: HAL CCSDCountry: FrancePresentation Dernier rejeton d'une longue lignee d'homme de lettres, le jeune Zhang Dai 張岱 (1597-1681) connut l'opulence d'un riche lettre oisif, s'adonnant tout a loisir a sa passion pour la litterature et a son gout tres marque pour le theâtre avant de tout perdre au moment de la chute des Ming 明 (1644). Reste fidele a la defunte dynastie, il brava les interdits en en redigeant l'histoire. Depouille et traque, il eprouva la misere et la clandestinite, ce qui explique que la plupart de ses e...
Average popularityAverage popularity In bottom 99%Average influencePopularity: Citation-based measure reflecting the current impact.Average influence In bottom 99%Influence: Citation-based measure reflecting the total impact.add Add to ORCIDPlease grant OpenAIRE to access and update your ORCID works.This Research product is the result of merged Research products in OpenAIRE.
You have already added works in your ORCID record related to the merged Research product.