Search Results - "PETIT, Christine"

Pejvakin-mediated pexophagy protects auditory hair cells against noise-induced damage by Defourny, Jean, Aghaie, Alain, Perfettini, Isabelle, Avan, Paul, Delmaghani, Sedigheh, Petit, Christine

“…Noise overexposure causes oxidative stress, leading to auditory hair cell damage. Adaptive peroxisome proliferation involving pejvakin, a peroxisome-associated…”
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The Auditory Hair Cell Ribbon Synapse: From Assembly to Function by SAFIEDDINE, Saaid, EL-AMRAOUI, Aziz, PETIT, Christine

“…Cochlear inner hair cells (IHCs), the mammalian auditory sensory cells, encode acoustic signals with high fidelity by Graded variations of their membrane…”
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Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model by Akil, Omar, Dyka, Frank, Calvet, Charlotte, Emptoz, Alice, Lahlou, Ghizlene, Nouaille, Sylvie, de Monvel, Jacques Boutet, Hardelin, Jean-Pierre, Hauswirth, William W., Avan, Paul, Petit, Christine, Safieddine, Saaid, Lustig, Lawrence R.

“…Autosomal recessive genetic forms (DFNB) account for most cases of profound congenital deafness. Adeno-associated virus (AAV)-based gene therapy is a promising…”
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Emerging low-molecular weight nucleopeptide-based hydrogels: state of the art, applications, challenges and perspectives by Giraud, Tristan, Hoschtettler, Paul, Pickaert, Guillaume, Averlant-Petit, Marie-Christine, Stefan, Loic

“…Over the last twenty years, low-molecular weight gelators and, in particular, peptide-based hydrogels, have drawn great attention from scientists thanks to…”
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Otogelin, otogelin-like, and stereocilin form links connecting outer hair cell stereocilia to each other and the tectorial membrane by Avan, Paul, Le Gal, Sébastien, Michel, Vincent, Dupont, Typhaine, Hardelin, Jean-Pierre, Petit, Christine, Verpy, Elisabeth

“…The function of outer hair cells (OHCs), the mechanical actuators of the cochlea, involves the anchoring of their tallest stereocilia in the tectorial membrane…”
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Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes by Delmaghani, Sedigheh, Defourny, Jean, Aghaie, Asadollah, Beurg, Maryline, Dulon, Didier, Thelen, Nicolas, Perfettini, Isabelle, Zelles, Tibor, Aller, Mate, Meyer, Anaïs, Emptoz, Alice, Giraudet, Fabrice, Leibovici, Michel, Dartevelle, Sylvie, Soubigou, Guillaume, Thiry, Marc, Vizi, E. Sylvester, Safieddine, Saaid, Hardelin, Jean-Pierre, Avan, Paul, Petit, Christine

“…A deficiency in pejvakin, a protein of unknown function, causes a strikingly heterogeneous form of human deafness. Pejvakin-deficient (Pjvk−/−) mice also…”
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How the genetics of deafness illuminates auditory physiology by Richardson, Guy P, de Monvel, Jacques Boutet, Petit, Christine

“…Although the basic principles underlying the function of the peripheral auditory system have been known for many years, the molecules required for hearing have…”
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Usher syndrome type 1-associated cadherins shape the photoreceptor outer segment by Schietroma, Cataldo, Parain, Karine, Estivalet, Amrit, Aghaie, Asadollah, Boutet de Monvel, Jacques, Picaud, Serge, Sahel, José-Alain, Perron, Muriel, El-Amraoui, Aziz, Petit, Christine

“…Usher syndrome type 1 (USH1) causes combined hearing and sight defects, but how mutations in USH1 genes lead to retinal dystrophy in patients remains elusive…”
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Phylogenetic analysis of Harmonin homology domains by Colcombet-Cazenave, Baptiste, Druart, Karen, Bonnet, Crystel, Petit, Christine, Spérandio, Olivier, Guglielmini, Julien, Wolff, Nicolas

“…Harmonin Homogy Domains (HHD) are recently identified orphan domains of about 70 residues folded in a compact five alpha-helix bundle that proved to be…”
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Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation by Corns, Laura F., Johnson, Stuart L., Roberts, Terri, Ranatunga, Kishani M., Hendry, Aenea, Ceriani, Federico, Safieddine, Saaid, Steel, Karen P., Forge, Andy, Petit, Christine, Furness, David N., Kros, Corné J., Marcotti, Walter

“…In the adult auditory organ, mechanoelectrical transducer (MET) channels are essential for transducing acoustic stimuli into electrical signals. In the absence…”
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Different CaV1.3 Channel Isoforms Control Distinct Components of the Synaptic Vesicle Cycle in Auditory Inner Hair Cells by Vincent, Philippe F Y, Bouleau, Yohan, Charpentier, Gilles, Emptoz, Alice, Safieddine, Saaid, Petit, Christine, Dulon, Didier

“…The mechanisms orchestrating transient and sustained exocytosis in auditory inner hair cells (IHCs) remain largely unknown. These exocytotic responses are…”
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Otoferlin, Defective in a Human Deafness Form, Is Essential for Exocytosis at the Auditory Ribbon Synapse by Roux, Isabelle, Safieddine, Saaid, Nouvian, Régis, Grati, M'hamed, Simmler, Marie-Christine, Bahloul, Amel, Perfettini, Isabelle, Le Gall, Morgane, Rostaing, Philippe, Hamard, Ghislaine, Triller, Antoine, Avan, Paul, Moser, Tobias, Petit, Christine

“…The auditory inner hair cell (IHC) ribbon synapse operates with an exceptional temporal precision and maintains a high level of neurotransmitter release…”
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Ciliary proteins link basal body polarization to planar cell polarity regulation by Yoder, Bradley K, Banizs, Boglarka, Foucher, Isabelle, Petit, Christine, Chen, Ping, Qian, Dong, Roper, Venus C, Jones, Chonnettia

“…Planar cell polarity (PCP) refers to coordinated polarization of cells within the plane of a cell sheet. A conserved signaling pathway is required for the…”
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Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice by Sahly, Iman, Dufour, Eric, Schietroma, Cataldo, Michel, Vincent, Bahloul, Amel, Perfettini, Isabelle, Pepermans, Elise, Estivalet, Amrit, Carette, Diane, Aghaie, Asadollah, Ebermann, Inga, Lelli, Andrea, Iribarne, Maria, Hardelin, Jean-Pierre, Weil, Dominique, Sahel, José-Alain, El-Amraoui, Aziz, Petit, Christine

“…The mechanisms underlying retinal dystrophy in Usher syndrome type I (USH1) remain unknown because mutant mice lacking any of the USH1 proteins-myosin VIIa,…”
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Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth by Lelli, Andrea, Michel, Vincent, Boutet de Monvel, Jacques, Cortese, Matteo, Bosch-Grau, Montserrat, Aghaie, Asadollah, Perfettini, Isabelle, Dupont, Typhaine, Avan, Paul, El-Amraoui, Aziz, Petit, Christine

“…The precise architecture of hair bundles, the arrays of mechanosensitive microvilli-like stereocilia crowning the auditory hair cells, is essential to hearing…”
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Consequences of Isostructural Main-Chain Modifications for the Design of Antimicrobial Foldamers: Helical Mimics of Host-Defense Peptides Based on a Heterogeneous Amide/Urea Backbone by Claudon, Paul, Violette, Aude, Lamour, Karen, Decossas, Marion, Fournel, Sylvie, Heurtault, Béatrice, Godet, Julien, Mély, Yves, Jamart-Grégoire, Brigitte, Averlant-Petit, Marie-Christine, Briand, Jean-Paul, Duportail, Guy, Monteil, Henri, Guichard, Gilles

“…Fraternal twins: Oligoureas and γ‐peptides are isosteric, quasi‐isostructural helical foldamers endowed with distinct biomolecular recognition properties…”
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Control of exocytosis by synaptotagmins and otoferlin in auditory hair cells by Beurg, Maryline, Michalski, Nicolas, Safieddine, Saaid, Bouleau, Yohan, Schneggenburger, Ralf, Chapman, Edwin R, Petit, Christine, Dulon, Didier

“…In pre-hearing mice, vesicle exocytosis at cochlear inner hair cell (IHC) ribbon synapses is triggered by spontaneous Ca(2+) spikes. At the onset of hearing,…”
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Defect in the gene encoding the EAR/EPTP domain-containing protein TSPEAR causes DFNB98 profound deafness by DELMAGHANI, Sedigheh, AGHAIE, Asadollah, MICHALSKI, Nicolas, BONNET, Crystel, WEIL, Dominique, PETIT, Christine

“…We report a consanguineous Iranian family affected by congenital profound sensorineural deafness segregating in an autosomal recessive mode. Auditory tests…”
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Rheological investigation of supramolecular physical gels in water/dimethylsulfoxide mixtures by lysine derivatives by Rangel Euzcateguy, Géraldine, Parajua‐Sejil, Caroline, Marchal, Philippe, Chapron, David, Averlant‐Petit, Marie‐Christine, Stefan, Loïc, Pickaert, Guillaume, Durand, Alain

“…Two derivatives of lysine were synthesized by acylating the Nα with dodecanoic acid starting from commercial precursors in which the Nε was already acylated by…”
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Mutations in apoptosis-inducing factor cause X-linked recessive auditory neuropathy spectrum disorder by Zong, Liang, Guan, Jing, Ealy, Megan, Zhang, Qiujing, Wang, Dayong, Wang, Hongyang, Zhao, Yali, Shen, Zhirong, Campbell, Colleen A, Wang, Fengchao, Yang, Ju, Sun, Wei, Lan, Lan, Ding, Dalian, Xie, Linyi, Qi, Yue, Lou, Xin, Huang, Xusheng, Shi, Qiang, Chang, Suhua, Xiong, Wenping, Yin, Zifang, Yu, Ning, Zhao, Hui, Wang, Jun, Wang, Jing, Salvi, Richard J, Petit, Christine, Smith, Richard J H, Wang, Qiuju

“…Auditory neuropathy spectrum disorder (ANSD) is a form of hearing loss in which auditory signal transmission from the inner ear to the auditory nerve and brain…”
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