104 results on '"Faure, Julien"'
Search Results
2. Gene therapies for RyR1-related myopathies
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Marty, Isabelle, Beaufils, Mathilde, Fauré, Julien, and Rendu, John
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- 2023
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3. Local and global aspects of the linear MRI in accretion disks
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Latter, Henrik N., Fromang, Sebastien, and Faure, Julien
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Astrophysics - High Energy Astrophysical Phenomena - Abstract
We revisit the linear MRI in a cylindrical model of an accretion disk and uncover a number of attractive results overlooked in previous treatments. In particular, we elucidate the connection between local axisymmetric modes and global modes, and show that a local channel flow corresponds to the evanescent part of a global mode. In addition, we find that the global problem reproduces the local dispersion relation without approximation, a result that helps explain the success the local analysis enjoys in predicting global growth rates. MRI channel flows are nonlinear solutions to the governing equations in the local shearing box. However, only a small subset of MRI modes share the same property in global disk models, providing further evidence that the prominence of channels in local boxes is artificial. Finally, we verify our results via direct numerical simulations with the Godunov code RAMSES., Comment: 13 pages, 9 figures, MNRAS accepted
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- 2015
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4. The explosion mechanism of core-collapse supernovae: progress in supernova theory and experiments
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Foglizzo, Thierry, Kazeroni, Rémi, Guilet, Jérôme, Masset, Frédéric, González, Matthias, Krueger, Brendan K., Novak, Jérôme, Oertel, Micaela, Margueron, Jérôme, Faure, Julien, Martin, Noël, Blottiau, Patrick, Peres, Bruno, and Durand, Gilles
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Astrophysics - High Energy Astrophysical Phenomena ,Physics - Fluid Dynamics - Abstract
The explosion of core-collapse supernova depends on a sequence of events taking place in less than a second in a region of a few hundred kilometers at the center of a supergiant star, after the stellar core approaches the Chandrasekhar mass and collapses into a proto-neutron star, and before a shock wave is launched across the stellar envelope. Theoretical efforts to understand stellar death focus on the mechanism which transforms the collapse into an explosion. Progress in understanding this mechanism is reviewed with particular attention to its asymmetric character. We highlight a series of successful studies connecting observations of supernova remnants and pulsars properties to the theory of core-collapse using numerical simulations. The encouraging results from first principles models in axisymmetric simulations is tempered by new puzzles in 3D. The diversity of explosion paths and the dependence on the pre-collapse stellar structure is stressed, as well as the need to gain a better understanding of hydrodynamical and MHD instabilities such as SASI and neutrino-driven convection. The shallow water analogy of shock dynamics is presented as a comparative system where buoyancy effects are absent. This dynamical system can be studied numerically and also experimentally with a water fountain. The potential of this complementary research tool for supernova theory is analyzed. We also review its potential for public outreach in science museums., Comment: 19 pages, 6 figures, invited review accepted for publication in PASA
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- 2015
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5. An empirical exploration of the subjectivity problem of information qualities.
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Van der Sluis, Frans, Faure, Julien, and Homnual, Sofie Phutachard
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INTELLECT , *MEDICAL quality control , *EMPIRICAL research , *INFORMATION resources , *DESCRIPTIVE statistics , *THEMATIC analysis , *ODDS ratio , *CONCEPTUAL structures , *RESEARCH methodology , *HEALTH information systems , *QUALITY assurance , *JUDGMENT (Psychology) , *COMPARATIVE studies , *USER interfaces ,RESEARCH evaluation - Abstract
Information qualities such as usefulness, accuracy, and comprehensiveness are to some extent subjective. Information resources have different meanings to different people and at different moments. This apparent subjectivity hinders indexing based on qualities for retrieval and filtering purposes. We conceptualize this as the subjectivity problem and address it through two studies. Study One explores whether, on public fora, people consider qualities as claims they should agree upon. Study Two explores, through a vignettes study, which conditions foster this inter‐subjective validity of quality claims. We conclude that information qualities become agreeable given the right set of conditions. We discuss the need for transparency about information qualities and quality considerations in order to offer these conditions to end users. [ABSTRACT FROM AUTHOR]
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- 2024
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6. Variations in the TRPV1 gene are associated to exertional heat stroke
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Bosson, Caroline, Rendu, John, Pelletier, Laurent, Abriat, Amandine, Chatagnon, Amandine, Brocard, Julie, Brocard, Jacques, Figarella-Branger, Dominique, Ducreux, Sylvie, van Coppenolle, Fabien, Sagui, Emmanuel, Marty, Isabelle, Roux-Buisson, Nathalie, and Faure, Julien
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- 2020
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7. Vortex cycles at the inner edges of dead zones in protoplanetary disks
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Faure, Julien, Fromang, Sébatien, latter, Henrik, and Meheut, Heloise
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Astrophysics - Earth and Planetary Astrophysics - Abstract
In protoplanetary disks, the inner boundary between the turbulent and laminar regions is a promising site for planet formation because solids may become trapped at the interface itself or in vortices generated by the Rossby wave instability. The disk thermodynamics and the turbulent dynamics at that location are entwined because of the importance of turbulent dissipation on thermal ionization and, conversely, of thermal ionisation on the turbulence. However, most previous work has neglected this dynamical coupling and have thus missed a key element of the physics in this region. In this paper, we aim to determine how the the interplay between ionization and turbulence impacts on the formation and evolution of vortices at the interface between the active and the dead zones. Using the Godunov code RAMSES, we have performed a 3D magnetohydrodynamic global numerical simulation of a cylindrical model of an MRI--turbulent protoplanetary disk, including thermodynamical effects as well as a temperature-dependant resistivity. The comparison with an analogous 2D viscous simulation has been extensively used to help identify the relevant physical processes and the disk's long-term evolution. We find that a vortex formed at the interface, due to Rossby wave instability, migrates inward and penetrates the active zone where it is destroyed by turbulent motions. Subsequently, a new vortex emerges a few tens of orbits later at the interface, and the new vortex migrates inward too. The sequence repeats itself, resulting in cycles of vortex formation, migration, and disruption. This behavior is successfully reproduced using two different codes. In this paper, we characterize this vortex life cycle and discuss its implications for planet formation at the dead/active interface. Our simulations highlight the importance of thermodynamical processes for the vortex evolution at the dead zone inner edge., Comment: 13 pages, 15 figures
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- 2014
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8. Thermodynamics of the dead-zone inner edge in protoplanetary disks
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Faure, Julien, Fromang, Sebastien, and Latter, Henrik N.
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Astrophysics - Earth and Planetary Astrophysics - Abstract
In protoplanetary disks, the inner boundary between the turbulent and laminar regions could be a promising site for planet formation, thanks to the trapping of solids at the boundary itself or in vortices generated by the Rossby wave instability. At the interface, the disk thermodynamics and the turbulent dynamics are entwined because of the importance of turbulent dissipation and thermal ionization. Numerical models of the boundary, however, have neglected the thermodynamics, and thus miss a part of the physics. The aim of this paper is to numerically investigate the interplay between thermodynamics and dynamics in the inner regions of protoplanetary disks by properly accounting for turbulent heating and the dependence of the resistivity on the local temperature. Using the Godunov code RAMSES, we performed a series of 3D global numerical simulations of protoplanetary disks in the cylindrical limit, including turbulent heating and a simple prescription for radiative cooling. We find that waves excited by the turbulence significantly heat the dead zone, and we subsequently provide a simple theoretical framework for estimating the wave heating and consequent temperature profile. In addition, our simulations reveal that the dead-zone inner edge can propagate outward into the dead zone, before staling at a critical radius that can be estimated from a mean-field model. The engine driving the propagation is in fact density wave heating close to the interface. A pressure maximum appears at the interface in all simulations, and we note the emergence of the Rossby wave instability in simulations with extended azimuth. Our simulations illustrate the complex interplay between thermodynamics and turbulent dynamics in the inner regions of protoplanetary disks. They also reveal how important activity at the dead-zone interface can be for the dead-zone thermodynamic structure., Comment: 16 pages, 16 figures. Accepted in Astronomy and Astrophysics
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- 2014
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9. Diagnostic biologique des angioedèmes bradykiniques : les recommandations du CREAK
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Bouillet, Laurence, Defendi, Frederica, Hardy, Gaelle, Cesbron, Jean Yves, Boccon-Gibod, Isabelle, Deroux, Alban, Mansard, Catherine, Launay, David, Gompel, Anne, Floccard, Bernard, Jaussaud, Roland, Beaudouin, Etienne, Armengol, Guillaume, Olliver, Yann, Gayet, Stephane, Du Than, Aureli, Sailler, Laurent, Guez, Stephane, Sarrat, Anne, Sorin, Lucile, de Moreuil, Claire, Pelletier, Fabien, Javaud, Nicolas, Marmion, Nicolas, Fain, Olivier, Fauré, Julien, and Dumestre-Pérard, Chantal
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- 2019
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10. Rocket dust storms and detached dust layers in the Martian atmosphere
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Spiga, Aymeric, Faure, Julien, Madeleine, Jean-Baptiste, Määttänen, Anni, and Forget, François
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Astrophysics - Earth and Planetary Astrophysics ,Physics - Atmospheric and Oceanic Physics ,Physics - Fluid Dynamics ,Physics - Geophysics - Abstract
Airborne dust is the main climatic agent in the Martian environment. Local dust storms play a key role in the dust cycle; yet their life cycle is poorly known. Here we use mesoscale modeling that includes the transport of radiatively active dust to predict the evolution of a local dust storm monitored by OMEGA on board Mars Express. We show that the evolution of this dust storm is governed by deep convective motions. The supply of convective energy is provided by the absorption of incoming sunlight by dust particles, rather than by latent heating as in moist convection on Earth. We propose to use the terminology "rocket dust storm", or conio-cumulonimbus, to describe those storms in which rapid and efficient vertical transport takes place, injecting dust particles at high altitudes in the Martian troposphere (30 to 50 km). Combined to horizontal transport by large-scale winds, rocket dust storms produce detached layers of dust reminiscent of those observed with Mars Global Surveyor and Mars Reconnaissance Orbiter. Since nighttime sedimentation is less efficient than daytime convective transport, and the detached dust layers can convect during the daytime, these layers can be stable for several days. The peak activity of rocket dust storms is expected in low-latitude regions at clear seasons (late northern winter to late northern summer), which accounts for the high-altitude tropical dust maxima unveiled by Mars Climate Sounder. Dust-driven deep convection have strong implications for the Martian dust cycle, thermal structure, atmospheric dynamics, cloud microphysics, chemistry, and robotic and human exploration., Comment: 33 pages, 13 figures, accepted for Journal of Geophysical Research (Planets)
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- 2012
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11. Tubulin Tyrosination Is a Major Factor Affecting the Recruitment of CAP-Gly Proteins at Microtubule Plus Ends
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Peris, Leticia, Fauré, Julien, Saoudi, Yasmina, Lafanechère, Laurence, Galjart, Niels, Bornens, Michel, Wordeman, Linda, Wehland, Juergen, Andrieux, Annie, and Job, Didier
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- 2006
12. Role of LBPA and Alix in Multivesicular Liposome Formation and Endosome Organization
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Matsuo, Hirotami, Chevallier, Julien, Mayran, Nathalie, Le Blanc, Isabelle, Ferguson, Charles, Fauré, Julien, Blanc, Nathalie Sartori, Matile, Stefan, Dubochet, Jacques, Sadoul, Rémy, Parton, Robert G., Vilbois, Francis, and Gruenberg, Jean
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- 2004
13. Common and variable clinical, histological, and imaging findings of recessive RYR1-related centronuclear myopathy patients
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Abath Neto, Osorio, Moreno, Cristiane de Araújo Martins, Malfatti, Edoardo, Donkervoort, Sandra, Böhm, Johann, Guimarães, Júlio Brandão, Foley, A. Reghan, Mohassel, Payam, Dastgir, Jahannaz, Bharucha-Goebel, Diana Xerxes, Monges, Soledad, Lubieniecki, Fabiana, Collins, James, Medne, Līvija, Santi, Mariarita, Yum, Sabrina, Banwell, Brenda, Salort-Campana, Emmanuelle, Rendu, John, Fauré, Julien, Yis, Uluc, Eymard, Bruno, Cheraud, Chrystel, Schneider, Raphaël, Thompson, Julie, Lornage, Xaviere, Mesrob, Lilia, Lechner, Doris, Boland, Anne, Deleuze, Jean-François, Reed, Umbertina Conti, Oliveira, Acary Souza Bulle, Biancalana, Valérie, Romero, Norma B., Bönnemann, Carsten G., Laporte, Jocelyn, and Zanoteli, Edmar
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- 2017
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14. Quality Conversations and Considerations on Reddit
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Van Der Sluis, Frans, primary, Faure, Julien, additional, and Phutachard Homnual, Sofie, additional
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- 2023
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15. Diagnostic work-up and phenotypic characteristics of a family with variable severity of distal arthrogryposis type 2B (Sheldon-Hall syndrome) and TNNT3 pathogenic variant
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Dabaj, Ivana, primary, Carlier, Robert Y., additional, Dieterich, Klaus, additional, Desguerre, Isabelle, additional, Faure, Julien, additional, Romero, Norma B., additional, Trang, Wenting, additional, Quijano-Roy, Susana, additional, and Germain, Dominique P., additional
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- 2023
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16. Ryanodine Receptor 1 and Associated Pathologies
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Fauré, Julien, Lunardi, Joël, Monnier, Nicole, Marty, Isabelle, Weiss, Norbert, editor, and Koschak, Alexandra, editor
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- 2014
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17. The clinical and genetic spectrum of autosomal-recessive TOR1A-related disorders
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Saffari, Afshin, primary, Lau, Tracy, additional, Tajsharghi, Homa, additional, Karimiani, Ehsan Ghayoor, additional, Kariminejad, Ariana, additional, Efthymiou, Stephanie, additional, Zifarelli, Giovanni, additional, Sultan, Tipu, additional, Toosi, Mehran Beiraghi, additional, Sedighzadeh, Sahar, additional, Siu, Victoria Mok, additional, Ortigoza-Escobar, Juan Darío, additional, AlShamsi, Aisha M, additional, Ibrahim, Shahnaz, additional, Al-Sannaa, Nouriya Abbas, additional, Al-Hertani, Walla, additional, Sandra, Whalen, additional, Tarnopolsky, Mark, additional, Alavi, Shahryar, additional, Li, Chumei, additional, Day-Salvatore, Debra-Lynn, additional, Martínez-González, Maria Jesús, additional, Levandoski, Kristin M, additional, Bedoukian, Emma, additional, Madan-Khetarpal, Suneeta, additional, Idleburg, Michaela J, additional, Menezes, Minal Juliet, additional, Siddharth, Aishwarya, additional, Platzer, Konrad, additional, Oppermann, Henry, additional, Smitka, Martin, additional, Collins, Felicity, additional, Lek, Monkol, additional, Shahrooei, Mohmmad, additional, Ghavideldarestani, Maryam, additional, Herman, Isabella, additional, Rendu, John, additional, Faure, Julien, additional, Baker, Janice, additional, Bhambhani, Vikas, additional, Calderwood, Laurel, additional, Akhondian, Javad, additional, Imannezhad, Shima, additional, Mirzadeh, Hanieh Sadat, additional, Hashemi, Narges, additional, Doosti, Mohammad, additional, Safi, Mojtaba, additional, Ahangari, Najmeh, additional, Torbati, Paria Najarzadeh, additional, Abedini, Soheila, additional, Salpietro, Vincenzo, additional, Gulec, Elif Yilmaz, additional, Eshaghian, Safieh, additional, Ghazavi, Mohammadreza, additional, Pascher, Michael T, additional, Vogel, Marina, additional, Abicht, Angela, additional, Moutton, Sébastien, additional, Bruel, Ange-Line, additional, Rieubland, Claudine, additional, Gallati, Sabina, additional, Strom, Tim M, additional, Lochmüller, Hanns, additional, Mohammadi, Mohammad Hasan, additional, Alvi, Javeria Raza, additional, Zackai, Elaine H, additional, Keena, Beth A, additional, Skraban, Cara M, additional, Berger, Seth I, additional, Andrew, Erin H, additional, Rahimian, Elham, additional, Morrow, Michelle M, additional, Wentzensen, Ingrid M, additional, Millan, Francisca, additional, Henderson, Lindsay B, additional, Dafsari, Hormos Salimi, additional, Jungbluth, Heinz, additional, Gomez-Ospina, Natalia, additional, McRae, Anne, additional, Peter, Merlene, additional, Veltra, Danai, additional, Marinakis, Nikolaos M, additional, Sofocleous, Christalena, additional, Ashrafzadeh, Farah, additional, Pehlivan, Davut, additional, Lemke, Johannes R, additional, Melki, Judith, additional, Benezit, Audrey, additional, Bauer, Peter, additional, Weis, Denisa, additional, Lupski, James R, additional, Senderek, Jan, additional, Christodoulou, John, additional, Chung, Wendy K, additional, Goodchild, Rose, additional, Offiah, Amaka C, additional, Moreno-De-Luca, Andres, additional, Suri, Mohnish, additional, Ebrahimi-Fakhari, Darius, additional, Houlden, Henry, additional, and Maroofian, Reza, additional
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- 2023
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18. Gene therapy strategies using CRISPR/Cas9 for RyR1-related myopathies
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Beaufils, Mathilde, primary, Brocard, Julie, additional, Faure, Julien, additional, Rendu, John, additional, and Marty, Isabelle, additional
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- 2023
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19. 'Le Tombeau de Couperin' de Maurice Ravel: Desde la obra para piano hasta la obra para orquesta
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Neder Kalil Faure, Julien and Krasutskaya, Alena
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Impressionism ,Classical music ,Orquestación ,Piano ,Maurice Ravel ,Orchestration ,Orchestral music ,Música clásica ,Harpsichord ,Arte barroco ,Baroque ,789 - Compositores y tradiciones de la música [780 - Música] ,Música orquestal ,Impresionismo ,Musical form ,Composición ,Forma musical ,Barroco ,clavecín ,Baroque art - Abstract
Este trabajo está dirigido principalmente a estudiantes y profesionales de la música, y tiene por objeto presentar un análisis comparativo de los recursos utilizados en la composición para piano y posterior orquestación de la obra “Le Tombeau de Couperin” de Maurice Ravel. De esta forma, se busca facilitar la comprensión de los procedimientos usados por el compositor, de manera que se favorezca una adecuada interpretación de la obra. En su primer capítulo se aborda el análisis de los elementos utilizados por Ravel en las cuatro piezas de la obra para piano que fueron orquestadas (Prélude, Forlane, Menuet y Rigaudon). En su segundo capítulo, muestra el estudio exhaustivo de los procedimientos utilizados por el compositor para trasladar esta obra pianística al plano orquestal. This work is mainly intended for music students and professionals and aims to present a comparative analysis of the resources used in the composition for piano and the subsequent orchestration of the work “Le Tombeau de Couperin” by Maurice Ravel. By these means, it seeks to facilitate the understanding of the procedures used by the composer, in such a way that an adequate interpretation of the work is favored. The first chapter deals with the analysis of the elements used by Ravel in the four pieces from the piano work that were orchestrated (Prélude, Forlane, Menuet, and Rigaudon). In its second chapter, it shows the exhaustive study of the procedures used by the composer to transfer this pianistic work to the orchestral language. Maestría
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- 2021
20. Pathogenic Variants in the Myosin Chaperone UNC-45B Cause Progressive Myopathy with Eccentric Cores
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Donkervoort, Sandra, Kutzner, Carl E., Hu, Ying, Lornage, Xaviere, Rendu, John, Stojkovic, Tanya, Baets, Jonathan, Neuhaus, Sarah B., Tanboon, Jantima, Maroofian, Reza, Bolduc, Veronique, Mroczek, Magdalena, Conijn, Stefan, Kuntz, Nancy L., Topf, Ana, Monges, Soledad, Lubieniecki, Fabiana, McCarty, Riley M., Chao, Katherine R., Governali, Serena, Bohm, Johann, Boonyapisit, Kanokwan, Malfatti, Edoardo, Sangruchi, Tumtip, Horkayne-Szakaly, Iren, Hedberg-Oldfors, Carola, Efthymiou, Stephanie, Noguchi, Satoru, Djeddi, Sarah, Iida, Aritoshi, di Rosa, Gabriella, Fiorillo, Chiara, Salpietro, Vincenzo, Darin, Niklas, Faure, Julien, Houlden, Henry, Oldfors, Anders, Nishino, Ichizo, de Ridder, Willem, Straub, Volker, Pokrzywa, Wojciech, Laporte, Jocelyn, Foley, A. Reghan, Romero, Norma B., Ottenheijm, Coen, Hoppe, Thorsten, Boennemann, Carsten G., Donkervoort, Sandra, Kutzner, Carl E., Hu, Ying, Lornage, Xaviere, Rendu, John, Stojkovic, Tanya, Baets, Jonathan, Neuhaus, Sarah B., Tanboon, Jantima, Maroofian, Reza, Bolduc, Veronique, Mroczek, Magdalena, Conijn, Stefan, Kuntz, Nancy L., Topf, Ana, Monges, Soledad, Lubieniecki, Fabiana, McCarty, Riley M., Chao, Katherine R., Governali, Serena, Bohm, Johann, Boonyapisit, Kanokwan, Malfatti, Edoardo, Sangruchi, Tumtip, Horkayne-Szakaly, Iren, Hedberg-Oldfors, Carola, Efthymiou, Stephanie, Noguchi, Satoru, Djeddi, Sarah, Iida, Aritoshi, di Rosa, Gabriella, Fiorillo, Chiara, Salpietro, Vincenzo, Darin, Niklas, Faure, Julien, Houlden, Henry, Oldfors, Anders, Nishino, Ichizo, de Ridder, Willem, Straub, Volker, Pokrzywa, Wojciech, Laporte, Jocelyn, Foley, A. Reghan, Romero, Norma B., Ottenheijm, Coen, Hoppe, Thorsten, and Boennemann, Carsten G.
- Abstract
The myosin-directed chaperone UNC-45B is essential for sarcomeric organization and muscle function from Caenorhabditis elegans to humans. The pathological impact of UNC-45B in muscle disease remained elusive. We report ten individuals with bi-allelic variants in UNC45B who exhibit childhood-onset progressive muscle weakness. We identified a common UNC45B variant that acts as a complex hypomorph splice variant. Purified UNC-45B mutants showed changes in folding and solubility. In situ localization studies further demonstrated reduced expression of mutant UNC-45B in muscle combined with abnormal localization away from the A-band towards the Z-disk of the sarcomere. The physiological relevance of these observations was investigated in C. elegans by transgenic expression of conserved UNC-45 missense variants, which showed impaired myosin binding for one and defective muscle function for three. Together, our results demonstrate that UNC-45B impairment manifests as a chaperonopathy with progressive muscle pathology, which discovers the previously unknown conserved role of UNC-45B in myofibrillar organization.
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- 2020
21. Response to Hall et al.
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Chong, Jessica X., primary, Talbot, Jared C., additional, Teets, Emily M., additional, Previs, Samantha, additional, Martin, Brit L., additional, Shively, Kathryn M., additional, Marvin, Colby T., additional, Aylsworth, Arthur S., additional, Saadeh-Haddad, Reem, additional, Schatz, Ulrich A., additional, Inzana, Francesca, additional, Ben-Omran, Tawfeg, additional, Almusafri, Fatima, additional, Al-Mulla, Mariam, additional, Buckingham, Kati J., additional, Harel, Tamar, additional, Mor-Shaked, Hagar, additional, Radhakrishnan, Periyasamy, additional, Girisha, Katta M., additional, Nayak, Shalini S., additional, Shukla, Anju, additional, Dieterich, Klaus, additional, Faure, Julien, additional, Rendu, John, additional, Capri, Yline, additional, Latypova, Xenia, additional, Nickerson, Deborah A., additional, Warshaw, David, additional, Janssen, Paul M., additional, Amacher, Sharon L., additional, and Bamshad, Michael J., additional
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- 2020
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22. Mechanism of NADPH oxidase activation by the Rac/Rho-GDI complex
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Di-Poi, Nicolas, Faure, Julien, Grizot, Sylvestre, Molnar, Gergery, Pick, Edgar, and Dagher, Marie-Claire
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Biochemistry -- Research ,Oxidases -- Physiological aspects ,Gene mutations -- Physiological aspects ,Carrier proteins -- Physiological aspects ,Biological sciences ,Chemistry - Abstract
Research has been conducted on the Rac/Rho-GDI complex. The production of the characterized and new mutants of the prenylated or not Rac1 in understanding the mechanism of NADPH oxidation activation by the Rac/Rho-GDI complex in a cell-free system has been carried out and described.
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- 2001
23. Mutations in MYLPF Cause a Novel Segmental Amyoplasia that Manifests as Distal Arthrogryposis
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Chong, Jessica X., primary, Talbot, Jared C., additional, Teets, Emily M., additional, Previs, Samantha, additional, Martin, Brit L., additional, Shively, Kathryn M., additional, Marvin, Colby T., additional, Aylsworth, Arthur S., additional, Saadeh-Haddad, Reem, additional, Schatz, Ulrich A., additional, Inzana, Francesca, additional, Ben-Omran, Tawfeg, additional, Almusafri, Fatima, additional, Al-Mulla, Mariam, additional, Buckingham, Kati J., additional, Harel, Tamar, additional, Mor-Shaked, Hagar, additional, Radhakrishnan, Periyasamy, additional, Girisha, Katta M., additional, Nayak, Shalini S., additional, Shukla, Anju, additional, Dieterich, Klaus, additional, Faure, Julien, additional, Rendu, John, additional, Capri, Yline, additional, Latypova, Xenia, additional, Nickerson, Deborah A., additional, Warshaw, David M., additional, Janssen, Paul M.L., additional, Amacher, Sharon L., additional, and Bamshad, Michael J., additional
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- 2020
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24. Prenatal diagnosis of Lowe syndrome in a male fetus with isolated bilateral cataract
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Rouxel, Flavien, Fauré, Julien, Faure, Jean-Michel, Deschamps, Françoise, Burlet, Gilles, Flandrin, Anaig, Couture, Alain, Prodhomme, Olivier, Rendu, John, and Willems, Marjolaine
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- 2022
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25. Null Mutations Causing Depletion of the Type 1 Ryanodine Receptor (RYR1) Are Commonly Associated With Recessive Structural Congenital Myopathies With Cores
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Monnier, Nicole, Marty, Isabelle, Faure, Julien, Castiglioni, Claudia, Desnuelle, Claude, Sacconi, Sabrina, Estournet, Brigitte, Ferreiro, Ana, Romero, Norma, Laquerriere, Annie, Lazaro, Leila, Martin, Jean-Jacques, Morava, Eva, Rossi, Annick, Van der Kooi, Anneke, de Visser, Marianne, Verschuuren, Corien, Lunardi, Joël, and Férec, Claude
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- 2008
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26. TRPV1 variants impair intracellular Ca2+ signaling and may confer susceptibility to malignant hyperthermia
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Vanden Abeele, Fabien, Lotteau, Sabine, Ducreux, Sylvie, Dubois, charlotte, Monnier, Nicole, Amy, Hanna, Gkika, Dimitra, Romestaing, Caroline, Lucile, Noyer, Flourakis, Matthieu, Tessier, Nolwenn, Al-Mawla, Ribal, Chouabe, Christophe, Lefai, Etienne, Joël, Lunardi, Hamilton, Susan, Faure, Julien, Van Coppenolle, Fabien, Prevarskaya, Natalia, Laboratory of Excellence, Ion Channels Science and Therapeutics, Université de Lille, Sciences et Technologies, Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), School of Biomedical Sciences, University of Leeds, Biochimie et Genetique Moleculaire, CHU Grenoble, Department of Medicine and Department of Molecular Physiology & Biophysics, Baylor College of Medecine, Laboratoire de Physiologie Cellulaire : Canaux ioniques, inflammation et cancer - U1003 (PHYCELL), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Laboratoire d'Ecologie des Hydrosystèmes Naturels et Anthropisés (LEHNA), Université de Lyon-Université de Lyon-École Nationale des Travaux Publics de l'État (ENTPE)-Centre National de la Recherche Scientifique (CNRS), Grenoble Institut des Neurosciences (GIN), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Grenoble-Université Joseph Fourier - Grenoble 1 (UJF), Université Joseph Fourier - Grenoble 1 (UJF)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Universitaire [Grenoble] (CHU), LabEx ICST, Laboratoire d'excellence Canaux Ioniques d'Intérêt Thérapeutique, Equipe Labélisée par la Ligue Nationale Contre le Cancer, SIRIC ONCOLille, Université des Sciences et Technologies (Lille 1) (USTL), UMR 1397 CarMeN Cardiovasculaire, Métabolisme, Diabétologie et Nutrition. Centre de recherche Lyon-Grenoble-Auvergne-Rhône-Alpes, Institut National de la Recherche Agronomique (INRA), Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, School of Biomedical Sciences, University of Ulster, Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), IBP, Laboratoire de Biochimie Génétique et Moléculaire, Department of Molecular Physiology and Biophysics, Baylor College of Medicine (BCM), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-École Nationale des Travaux Publics de l'État (ENTPE), Centre National de la Recherche Scientifique (CNRS), École Nationale des Travaux Publics de l'État (ENTPE), GIN Grenoble Institut des Neurosciences, Université de Grenoble, French Ministere de l'Education Nationale, Institut National de la Sante et de la Recherche Medicale (Inserm), ANR program within the 'Investissements d'Avenir' [ANR-10-IBHU-0004], Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National de la Recherche Agronomique (INRA), Physiologie Cellulaire (PHYCEL) - U1003 (PHYCEL), Université de Lille-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Lille Nord de France (COMUE), Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Joseph Fourier - Grenoble 1 (UJF)-Centre Hospitalier Universitaire [Grenoble] (CHU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de Physiologie Cellulaire : Canaux ioniques, inflammation et cancer - U 1003 (PHYCELL), and ProdInra, Archive Ouverte
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Male ,mice ,[SDV]Life Sciences [q-bio] ,Gene Expression ,TRPV Cation Channels ,hereditary diseases ,Hereditary disease ,souris ,maladie héréditaire ,Article ,Malignant hyperthermia ,TRP channel ,Animals ,Homeostasis ,Humans ,Genetic Predisposition to Disease ,Calcium Signaling ,Gene Knock-In Techniques ,Muscle, Skeletal ,Anesthetics ,hyperthermie maligne ,[SDV] Life Sciences [q-bio] ,Mice, Inbred C57BL ,TRPV1 ,Disease Models, Animal ,HEK293 Cells ,Female ,Calcium ,[SDE.BE]Environmental Sciences/Biodiversity and Ecology - Abstract
International audience; Purpose: Malignant hyperthermia (MH) is a pharmacogenetic disorder arising from uncontrolled muscle calcium release due to an abnormality in the sarcoplasmic reticulum (SR) calcium-release mechanism triggered by halogenated inhalational anesthetics. However, the molecular mechanisms involved are still incomplete.Methods: We aimed to identify transient receptor potential vanilloid 1 (TRPV1) variants within the entire coding sequence in patients who developed sensitivity to MH of unknown etiology. In vitro and in vivo functional studies were performed in heterologous expression system, trpv1−/− mice, and a murine model of human MH.Results: We identified TRPV1 variants in two patients and their heterologous expression in muscles of trpv1−/− mice strongly enhanced calcium release from SR upon halogenated anesthetic stimulation, suggesting they could be responsible for the MHphenotype. We confirmed the in vivo significance by using mice with a knock-in mutation (Y524S) in the type I ryanodine receptor (Ryr1), a mutation analogous to the Y522S mutation associated with MH in humans. We showed that the TRPV1 antagonist capsazepine slows the heat-induced hypermetabolic response in this model.Conclusion: We propose that TRPV1 contributes to MH and could represent an actionable therapeutic target for prevention of the pathology and also be responsible for MH sensitivity when mutated.Genetics in Medicine (2019) 21:441–450; https://doi.org/10.1038/s41436- 018-0066-9Keywords:TRP channel; TRPV1; Calcium; Hereditary disease; Malignant hyperthermia
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- 2019
27. International Triadin Knockout Syndrome Registry
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Clemens, Daniel J., primary, Tester, David J., additional, Giudicessi, John R., additional, Bos, J. Martijn, additional, Rohatgi, Ram K., additional, Abrams, Dominic J., additional, Balaji, Seshadri, additional, Crotti, Lia, additional, Faure, Julien, additional, Napolitano, Carlo, additional, Priori, Silvia G., additional, Probst, Vincent, additional, Rooryck-Thambo, Caroline, additional, Roux-Buisson, Nathalie, additional, Sacher, Frederic, additional, Schwartz, Peter J., additional, Silka, Michael J., additional, Walsh, Mark A., additional, and Ackerman, Michael J., additional
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- 2019
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28. Caveolin 3 is associated with the calcium release complex and is modified via in vivo triadin modification
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Vassilopoulos, Stephane, Oddoux, Sarah, Groh, Severine, Cacheux, Marine, Faure, Julien, Brocard, Julie, Campbell, Kevin P., and Marty, Isabelle
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Adenoviruses -- Physiological aspects ,Adenoviruses -- Genetic aspects ,Calcium-binding proteins -- Chemical properties ,Calcium-binding proteins -- Structure ,Gene expression -- Analysis ,Muscles -- Physiological aspects ,Biological sciences ,Chemistry - Abstract
Adenovirus-mediated gene transfer in skeletal muscle of newborn mice was used to overexpress the triadin isoforms Trisk 95 or Trisk 51 and study the specific role of these important components of the skeletal muscle calcium release complex in muscle physiology. The results demonstrated that Trisk 95 and Trisk 51 play an instrumental role in the regulation of interaction between the functionally linke caveolin 3-containing membrane domains and the calcium release complex, integrity of which might be crucial for muscle physiology.
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- 2010
29. An investigation of temporal feature integration for a low-latency classification with application to speech/music/mix classification
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Flocon-Cholet, Joachim, Guérin, Alexandre, Faure, Julien, Scalart, Pascal, Orange Labs [Lannion], France Télécom, Orange Labs [Cesson-Sévigné], Orange Labs, Energy Efficient Computing ArchItectures with Embedded Reconfigurable Resources (CAIRN), Inria Rennes – Bretagne Atlantique, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-ARCHITECTURE (IRISA-D3), Institut de Recherche en Informatique et Systèmes Aléatoires (IRISA), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Université de Bretagne Sud (UBS)-École normale supérieure - Rennes (ENS Rennes)-Institut National de Recherche en Informatique et en Automatique (Inria)-Télécom Bretagne-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Université de Bretagne Sud (UBS)-École normale supérieure - Rennes (ENS Rennes)-Institut National de Recherche en Informatique et en Automatique (Inria)-Télécom Bretagne-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche en Informatique et Systèmes Aléatoires (IRISA), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Université de Bretagne Sud (UBS)-École normale supérieure - Rennes (ENS Rennes)-Télécom Bretagne-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS), AES, Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université de Bretagne Sud (UBS)-École normale supérieure - Rennes (ENS Rennes)-Institut National de Recherche en Informatique et en Automatique (Inria)-Télécom Bretagne-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université de Bretagne Sud (UBS)-École normale supérieure - Rennes (ENS Rennes)-Institut National de Recherche en Informatique et en Automatique (Inria)-Télécom Bretagne-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche en Informatique et Systèmes Aléatoires (IRISA), and Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université de Bretagne Sud (UBS)-École normale supérieure - Rennes (ENS Rennes)-Télécom Bretagne-CentraleSupélec-Centre National de la Recherche Scientifique (CNRS)
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SVM Support Vector Machines ,Temporal Integration ,[INFO.INFO-TS]Computer Science [cs]/Signal and Image Processing ,Audio classification - Abstract
International audience; In this study, we propose several methodologies for the use of feature integration and evaluate them in a low-latency classification framework. These general methodologies are based on three key aspects that will be assessed in this study: the selection of the features which have to be temporally integrated, the choice of the integration techniques, i.e. how the temporal information is extracted, and the size of the integration window. The experiments carried out for the speech/music/mix classification task show that the different methodologies have a significant impact on the global performance. Compared to the state of the art procedures, the methodologies we proposed achieved good performance, even with the low-latency constraints.
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- 2014
30. Thermodynamique du bord interne de la zone morte dans les disques protoplanétaires
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Faure, Julien, Département d'Astrophysique (ex SAP) (DAP), Institut de Recherches sur les lois Fondamentales de l'Univers (IRFU), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay, Université Paris Sud - Paris XI, and Sébastien Fromang
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Protoplanetary disk ,Planet formation ,Disque protoplanétaire ,Formation planétaire ,[SDU.ASTR.EP]Sciences of the Universe [physics]/Astrophysics [astro-ph]/Earth and Planetary Astrophysics [astro-ph.EP] ,Thermodynamics ,Zone morte ,Dead zone ,[PHYS.ASTR.SR]Physics [physics]/Astrophysics [astro-ph]/Solar and Stellar Astrophysics [astro-ph.SR] ,Thermodynamique ,Vortex - Abstract
The dead zone, a quiescent region enclosed in the turbulent flow of a protoplanetary disk, seems to be a promising site for planet formation. Indeed, the development of a density maximum at the dead zone inner edge, that has the property to trap the infalling dust, is a natural outcome of the accretion mismatch at this interface. Moreover, the flow here may be unstable and organize itself into vortical structures that efficiently collect dust grains. The inner edge location is however loosely constrained. In particular, it depends on the thermodynamical prescriptions of the disk model that is considered. It has been recently proposed that the inner edge is not static and that the variations of young stars accretion luminosity are the signature of this interface displacements. This thesis address the question of the impact of the gas thermodynamics onto its dynamics around the dead zone inner edge. MHD simulations including the complex interplay between thermodynamical processes and the dynamics confirmed the dynamical behaviour of the inner edge. A first measure of the interface velocity has been realised. This result has been compared to the predictions of a mean field model. It revealed the crucial role of the energy transport by density waves excited at the interface. These simulations also exhibit a new intriguing phenomenon: vortices forming at the interface follow a cycle of formation-migration-destruction. This vortex cycle may compromise the formation of planetesimals at the inner edge. This thesis claims that thermodynamical processes are at the heart of how the region around the dead zone inner edge in protoplanetary disks works.; La zone morte, région laminaire confinée au coeur des disques protoplanétaires dont la turbulence de l'écoulement à petite échelle explique l'accrétion de matière sur l'étoile en formation, semble être un lieu propice à la formation planétaire. En effet, au bord interne de la zone morte la différence d'accrétion entraîne le développement d'une sur-densité capable de piéger les grains de poussière qui dérivent vers l'étoile. L'écoulement à cet endroit est de plus potentiellement instable. Le cas échéant, il s'organise en structures tourbillonnaires appelées ''vortex'' qui collectent efficacement la poussière. La position du bord interne est toutefois très incertaine et dépend en particulier de la thermodynamique du modèle de disque considéré. Récemment, le déplacement du bord interne a été envisagé pour expliquer la variabilité de l'accrétion des étoiles jeunes. Cette thèse aborde le problème posé par l'influence de la thermodynamique sur la dynamique du bord interne de la zone morte. Des simulations MHD qui incluent le couplage entre les processus thermodynamiques avec la dynamique de l'écoulement ont tout d'abord permis de confirmer le comportement dynamique du bord interne ainsi que de réaliser la mesure inédite de sa vitesse typique de déplacement. La comparaison de ces résultats avec les prédictions données par un modèle de champ moyen a révélé le rôle du transport d'énergie par des ondes excitées au bord interne de la zone morte. Ces simulations présentent de plus un phénomène nouveau: les vortex formés à l'interface suivent un cycle de formation-migration-destruction. Cette découverte est susceptible de modifier notre vision du scénario de formation planétaire. En résumé, cette thèse met en évidence le fait que les processus thermodynamiques sont au coeur du fonctionnement de la région du bord interne de la zone morte dans les disques protoplanétaires.
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- 2014
31. 217th ENMC International Workshop: RYR1-related myopathies, Naarden, The Netherlands, 29–31 January 2016
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Jungbluth, Heinz, primary, Dowling, James J., additional, Ferreiro, Ana, additional, Muntoni, Francesco, additional, Bönnemann, Carsten, additional, Dirksen, Robert, additional, Faure, Julien, additional, Hamilton, Susan, additional, Hopkins, Phil, additional, Marks, Andrew, additional, Marty, Isabelle, additional, Meilleur, Katy, additional, Riazi, Sheila, additional, Sewry, Caroline, additional, Treves, Susan, additional, Voermans, Nicol, additional, and Zorzato, Francesco, additional
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- 2016
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32. Planet filtering at the inner edges of dead zones in protoplanetary disks
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Faure, Julien, primary and Nelson, Richard P., additional
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- 2016
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33. Dynamics of the inner edge of the dead zone in protoplanetaty disks
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Latter Henrik, Fromang Sebastien, and Faure Julien
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Physics ,Equation of state ,Accretion (meteorology) ,Turbulence ,Young stellar object ,QC1-999 ,Rossby wave ,Mechanics ,Astrophysics ,Instability ,Front velocity ,Critical radius ,Astrophysics::Earth and Planetary Astrophysics - Abstract
In protoplanetary disks, the inner boundary between an MRI active and inactive region has recently been suggested to be a promising site for planet formation. A set of numerical simulations has indeed shown that vortex formation mediated by the Rossby wave instability is a natural consequence of the disk dynamics at that location. However, such models have so far considered only the case of an isothermal equation of state, while the more complex thermodynamics of this region may have strong consequences on disk properties because of thermal ionization. Gas is heated by turbulent dissipation and radiatively cools on long timescales because disks are optically thick. Using a mean field model of the dynamics of that boundary, Latter and Balbus (2012) have shown that this complexity can lead to situations in which the active/dead interface moves systematically inward or outward, depending on the initial conditions. This is because turbulent activity is controlled by ohmic resistivity that is itself a sensitive function of temperature. Such a behavior suggests, as observed in young stellar object, a nonsteady accretion onto the central star. Using the Godunov code Ramses, we have performed 3D global numerical simulations of protoplanetary disks that relax the isothermal hypothesis in order to check the above scenario. We confirm the existence of such MRI fronts, thus validating the mean field approach described above. As shown by Latter and Balbus (2012), MRI fronts tend to stop at a critical radius. We argue that the typical front velocity crucially depends on turbulent diffusion of temperature. The diffusivity of temperature due to turbulence is measured to be order of H2/Ω where Ω is the local orbital time and H the typical height of the disk.
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- 2013
34. Exertional Heat Stroke and Susceptibility to Malignant Hyperthermia in an Athlete: Evidence for a Link?
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Poussel, Mathias, primary, Guerci, Philippe, primary, Kaminsky, Pierre, primary, Heymonet, Marie, primary, Roux-Buisson, Nathalie, primary, Faure, Julien, primary, Fronzaroli, Emilien, primary, and Chenuel, Bruno, primary
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- 2015
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35. Local and global aspects of the linear MRI in accretion discs
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Latter, Henrik N., primary, Fromang, Sebastien, additional, and Faure, Julien, additional
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- 2015
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36. Packet-loss concealment technology advances in EVS
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Lecomte, Jeremie, primary, Vaillancourt, Tommy, additional, Bruhn, Stefan, additional, Sung, Hosang, additional, Peng, Ke, additional, Kikuiri, Kei, additional, Wang, Bin, additional, Subasingha, Shaminda, additional, and Faure, Julien, additional
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- 2015
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37. Overview of the EVS codec architecture
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Dietz, Martin, primary, Multrus, Markus, additional, Eksler, Vaclav, additional, Malenovsky, Vladimir, additional, Norvell, Erik, additional, Pobloth, Harald, additional, Miao, Lei, additional, Wang, Zhe, additional, Laaksonen, Lasse, additional, Vasilache, Adriana, additional, Kamamoto, Yutaka, additional, Kikuiri, Kei, additional, Ragot, Stephane, additional, Faure, Julien, additional, Ehara, Hiroyuki, additional, Rajendran, Vivek, additional, Atti, Venkatraman, additional, Sung, Hosang, additional, Oh, Eunmi, additional, Yuan, Hao, additional, and Zhu, Changbao, additional
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- 2015
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38. The Explosion Mechanism of Core-Collapse Supernovae: Progress in Supernova Theory and Experiments
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Foglizzo, Thierry, primary, Kazeroni, Rémi, additional, Guilet, Jérôme, additional, Masset, Frédéric, additional, González, Matthias, additional, Krueger, Brendan K., additional, Novak, Jérôme, additional, Oertel, Micaela, additional, Margueron, Jérôme, additional, Faure, Julien, additional, Martin, Noël, additional, Blottiau, Patrick, additional, Peres, Bruno, additional, and Durand, Gilles, additional
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- 2015
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39. Vortex cycles at the inner edges of dead zones in protoplanetary disks
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Faure, Julien, primary, Fromang, Sébastien, additional, Latter, Henrik, additional, and Meheut, Heloise, additional
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- 2015
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40. Modèle hybride pour l'évaluation de la qualité vocale sans référence, appliqué à la téléphonie
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Leman, Adrien, Faure, Julien, Parizet, Etienne, Leclere, Quentin, Société Française d'Acoustique - SFA, Orange Labs [Lannion], France Télécom, Laboratoire Vibrations Acoustique (LVA), Institut National des Sciences Appliquées de Lyon (INSA Lyon), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)
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[SPI.ACOU]Engineering Sciences [physics]/Acoustics [physics.class-ph] ,[SPI.ACOU] Engineering Sciences [physics]/Acoustics [physics.class-ph] ,Qualité vocale ,Hybride ,Sans référence ,[PHYS.MECA.ACOU] Physics [physics]/Mechanics [physics]/Acoustics [physics.class-ph] ,[PHYS.MECA.ACOU]Physics [physics]/Mechanics [physics]/Acoustics [physics.class-ph] ,Multidimensionnel - Abstract
National audience; L'évaluation de la qualité vocale est un phénomène multidimensionnel faisant intervenir certains attributs perceptifs dépendant des dégradations induites par le type de communication utilisé (RTC / RNIS / VoIP / GSM). Le modèle proposé est basé sur un espace perceptif multidimensionnel dont les dimensions se combinent pour fournir un indicateur global de qualité. L'identification des dimensions a permis de révéler trois attributs perceptifs prépondérants : la bruyance, la coloration, et la continuité. Chacune des dimensions est estimée à partir d'indicateurs paramétriques (issues des statistiques réseaux) ou basés sur le signal sonore (indicateurs phycoacoustiques, indicateurs physiques). Cette approche hybride avec un cœur commun et des estimateurs pouvant s'adapter selon l'information accessible permet à la fois d'optimiser les performances et la mise en œuvre du modèle.
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- 2010
41. Apport d'un outil d'optimisation globale à l'ajustement des modèles statistiques
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Cabarbaye, André, Faure, Julien, and Jds2009, Conférence
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[MATH.MATH-ST] Mathematics [math]/Statistics [math.ST] - Abstract
Les modèles paramétriques ne peuvent représenter la réalité que si leurs paramètres ont été correctement ajustés à partir de données de retour d'expérience. La méthode d'ajustement la plus utilisée est la méthode du maximum de vraisemblance qui ne peut se suffire d'une simple technique d'optimisation locale quand les optima sont multiples. C'est la raison pour laquelle les outils existants donnent parfois des résultats erronés. Aussi avons-nous tenté d'ajuster différents modèles utilisés en fiabilité au moyen d'un outil d'optimisation globale associant Algorithmes Génétiques, Evolution Différentielle et Simplexe non linéaire. Nous nous sommes d'abord intéressés aux processus de renouvellement généralisé (GRP), pour lesquels les actions de maintenance ont un certain effet de rajeunissement. Nous avons ensuite traité le modèle de BERTHOLON, utilisé dans le domaine ferroviaire, qui associe une exponentielle à une Weibull pour modéliser globalement la deuxième et la troisième partie de la courbe en baignoire (défaillances précoces, défaillances occasionnelles puis usure). Ce modèle est susceptible d'être largement utilisé à l' avenir pour caractériser la fiabilité des composants électroniques dont l'intégration croissante devrait conduire à des limitations en durée de vie de plus en plus sévères. Mais son ajustement présentait jusqu'alors des difficultés. Pour considérer globalement les trois phases de la courbe en baignoire, nous avons enfin proposé un modèle plus complet à 7 paramètres que nous avons pu également ajuster sans difficulté en retrouvant approximativement les paramètres utilisés pour simuler préalablement un jeu d'une centaine de données expérimentales.
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- 2009
42. Effects of the window mounting conditions on the auditory perception of transmitted environmental noises
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Trollé, Arnaud, Marquis-Favre, Catherine, and Faure, Julien
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- 2005
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43. A robust howling detection algorithm based on a statistical approach
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Flocon-Cholet, Joachim, primary, Faure, Julien, additional, Guerin, Alexandre, additional, and Scalart, Pascal, additional
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- 2014
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44. Thermodynamics of the dead-zone inner edge in protoplanetary disks
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Faure, Julien, primary, Fromang, Sébastien, additional, and Latter, Henrik, additional
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- 2014
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45. Mild clinical presentation in KLHL40-related nemaline myopathy (NEM 8)
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Seferian, Andreea M., Malfatti, Edoardo, Bosson, Caroline, Pelletier, Laurent, Taytard, Jessica, Forin, Veronique, Gidaro, Teresa, Gargaun, Elena, Carlier, Pierre, Fauré, Julien, Romero, Norma B., Rendu, John, and Servais, Laurent
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- 2016
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46. 217th ENMC International Workshop: RYR1-related myopathies, Naarden, The Netherlands, 29–31 January 2016
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Bönnemann, Carsten, Dirksen, Robert, Faure, Julien, Hamilton, Susan, Hopkins, Phil, Marks, Andrew, Marty, Isabelle, Meilleur, Katy, Riazi, Sheila, Sewry, Caroline, Treves, Susan, Voermans, Nicol, Zorzato, Francesco, Jungbluth, Heinz, Dowling, James J., Ferreiro, Ana, and Muntoni, Francesco
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- 2016
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47. Dynamics of the inner edge of the dead zone in protoplanetary disks
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Faure, Julien, primary, Fromang, Sebastien, additional, and Latter, Henrik, additional
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- 2013
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48. Rocket dust storms and detached dust layers in the Martian atmosphere
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Spiga, Aymeric, primary, Faure, Julien, additional, Madeleine, Jean-Baptiste, additional, Määttänen, Anni, additional, and Forget, François, additional
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- 2013
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49. Triadin and CLIMP-63 form a link between triads and microtubules in muscle cells.
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Osseni, Alexis, Se'bastien, Muriel, Sarrault, Oriana, Baudet, Mathieu, Coute', Yohann, Faure', Julien, Fourest-Lieuvin, Anne, and Marty, Isabelle
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MEMBRANE proteins ,MUSCLE cells ,MICROTUBULES ,SKELETAL muscle ,SARCOPLASMIC reticulum ,RETICULUM (Ruminants) - Abstract
In skeletal muscle, the triad is a structure comprising a transverse (T)- tubule and sarcoplasmic reticulum (SR) cisternae. Triads constitute the basis of excitation-contraction coupling as the cradle of the Ca
2+ release complex. We have shown previously that triadin, a member of this complex, has shaping properties on reticulum membrane and is indirectly involved in a link between triads and microtubules. We have identified here that CLIMP-63 (also known as CKAP4), as the partner of triadin, is responsible for this association of triads and microtubules. Triadin and CLIMP-63 interact through their respective luminal domains and the shaping properties of triadin depend on the capacity of CLIMP-63 to bind microtubules with its cytosolic portion. In skeletal muscle, CLIMP-63 is localized in the SR, including triads, and is associated with the Ca2+ release complex through its interaction with triadin. Knockout of triadin in muscles results in the delocalization of CLIMP-63 from triads, its dissociation from the Ca2+ release complex and a disorganization of the microtubule network. Our results suggest that the association of triadin and CLIMP-63 could be involved in the shaping of SR terminal cisternae and in the guidance of microtubules close to the triads. [ABSTRACT FROM AUTHOR]- Published
- 2016
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50. A non-intrusive signal-based model for speech quality evaluation using automatic classification of background noises
- Author
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Leman, Adrien, primary, Faure, Julien, additional, and Parizet, Etienne, additional
- Published
- 2009
- Full Text
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