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收藏Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism揭示Otogl突变小鼠听觉超兴奋性，阐明了听觉神经病变机制
Gagliardini, M; Mechaussier, S; Pina, CC; Morais, M; Postal, O; Jean, P; Dupont, T; Singh-Estivalet, A; Udugampolage, S; Scandola, C; Verpy, E; Libé-Philippot, B; Inbar, TC; Schwenkgrub, J; Spinola, CMB; Etournay, R; El-Amraoui, A; Bathellier, B; Mallet, A; Delmaghani, S; Giraudet, F; Petit, C; Gourévitch, B; Avan, P; Michalski, N 分享 收藏 分享 收藏 Clarin-2 gene supplementation durably preserves hearing in a model of progressive hearing lossMendia, Clara; Peineau, Thibault; Zamani, Mina; Felgerolle, Chloe; Yahiaoui, Nawal; Christophersen, Nele; Papal, Samantha; Maudoux, Audrey; Maroo, Reza; Patni, Pranav; Nouaille, Sylvie; Bowl, Michael R.; Delmaghani, Sedigheh; Galehdari, Hamid; Vona, Barbara; Dulon, Didier; Vitry, Sandrine; El-Amraoui, Aziz 分享 收藏 分享 收藏 A biallelic variant in CLRN2 causes non-syndromic hearing loss in humansCLRN2中的双等位基因变体导致人类非综合征性听力损失Vona, Barbara; Mazaheri, Neda; Lin, Sheng-Jia; Dunbar, Lucy A.; Maroofian, Reza; Azaiez, Hela; Booth, Kevin T.; Vitry, Sandrine; Rad, Aboulfazl; Rueschendorf, Franz; Varshney, Pratishtha; Fowler, Ben; Beetz, Christian; Alagramam, Kumar N.; Murphy, David; Shariati, Gholamreza; Sedaghat, Alireza; Houlden, Henry; Petree, Cassidy; VijayKumar, Shruthi; Smith, Richard J. H.; Haaf, Thomas; El-Amraoui, Aziz; Bowl, Michael R.; Varshney, Gaurav K.; Galehdari, Hamid 分享 收藏 分享 收藏 Clarin-2 is essential for hearing by maintaining stereocilia integrity and functionDunbar, Lucy A.; Patni, Pranav; Aguilar, Carlos; Mburu, Philomena; Corns, Laura; Wells, Helena R. R.; Delmaghani, Sedigheh; Parker, Andrew; Johnson, Stuart; Williams, Debbie; Esapa, Christopher T.; Simon, Michelle M.; Chessum, Lauren; Newton, Sherylanne; Dorning, Joanne; Jeyarajan, Prashanthini; Morse, Susan; Lelli, Andrea; Codner, Gemma F.; Peineau, Thibault; Gopal, Suhasini R.; Alagramam, Kumar N.; Hertzano, Ronna; Dulon, Didier; Wells, Sara; Williams, Frances M.; Petit, Christine; Dawson, Sally J.; Brown, Steve D. M.; Marcotti, Walter; El-Amraoui, Aziz; Bowl, Michael R. 分享 收藏 Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndromeDulon, Didier; Papal, Samantha; Patni, Pranav; Cortese, Matteo; Vincent, Philippe F. Y.; Tertrais, Margot; Emptoz, Alice; Tlili, Abdelaziz; Bouleau, Yohan; Michel, Vincent; Delmaghani, Sedigheh; Aghaie, Alain; Pepermans, Elise; Alegria-Prevot, Olinda; Akil, Omar; Lustig, Lawrence; Avan, Paul; Safieddine, Saaid; Petit, Christine; El-Amraoui, Aziz 分享 收藏 Cone degeneration is triggered by the absence of USH1 proteins but prevented by antioxidant treatmentsTrouillet, Alix; Dubus, Elisabeth; Degardin, Julie; Estivalet, Amrit; Ivkovic, Ivana; Godefroy, David; Garcia-Ayuso, Diego; Simonutti, Manuel; Sahly, Iman; Sahel, Jose A.; El-Amraoui, Aziz; Petit, Christine; Picaud, Serge 分享 收藏 CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survivalMichel, Vincent; Booth, Kevin T.; Patni, Pranav; Cortese, Matteo; Azaiez, Hela; Bahloul, Amel; Kahrizi, Kimia; Labbe, Menelik; Emptoz, Alice; Lelli, Andrea; Degardin, Julie; Dupont, Typhaine; Aghaie, Asadollah; Oficjalska-Pham, Danuta; Picaud, Serge; Najmabadi, Hossein; Smith, Richard J.; Bowl, Michael R.; Brown, Steven D. M.; Avan, Paul; Petit, Christine; El-Amraoui, Aziz 分享 收藏 分享 收藏 Conformational switch of harmonin, a submembrane scaffold protein of the hair cell mechanoelectrical transduction machineryBahloul, Amel; Pepermans, Elise; Raynal, Bertrand; Wolff, Nicolas; Cordier, Florence; England, Patrick; Nouaille, Sylvie; Baron, Bruno; El-Amraoui, Aziz; Hardelin, Jean-Pierre; Durand, Dominique; Petit, Christine 分享 收藏 Usher syndrome type 1-associated cadherins shape the photoreceptor outer segmentSchietroma, Cataldo; Parain, Karine; Estivalet, Amrit; Aghaie, Asadollah; de Monvel, Jacques Boutet; Picaud, Serge; Sahel, Jose-Alain; Perron, Muriel; El-Amraoui, Aziz; Petit, Christine 分享 收藏 分享 收藏 Novel gene function revealed by mouse mutagenesis screens for models of age-related diseasePotter, Paul K.; Bowl, Michael R.; Jeyarajan, Prashanthini; Wisby, Laura; Blease, Andrew; Goldsworthy, Michelle E.; Simon, Michelle M.; Greenaway, Simon; Michel, Vincent; Barnard, Alun; Aguilar, Carlos; Agnew, Thomas; Banks, Gareth; Blake, Andrew; Chessum, Lauren; Dorning, Joanne; Falcone, Sara; Goosey, Laurence; Harris, Shelley; Haynes, Andy; Heise, Ines; Hillier, Rosie; Hough, Tertius; Hoslin, Angela; Hutchison, Marie; King, Ruairidh; Kumar, Saumya; Lad, Heena V.; Law, Gemma; MacLaren, Robert E.; Morse, Susan; Nicol, Thomas; Parker, Andrew; Pickford, Karen; Sethi, Siddharth; Starbuck, Becky; Stelma, Femke; Cheeseman, Michael; Cross, Sally H.; Foster, Russell G.; Jackson, Ian J.; Peirson, Stuart N.; Thakker, Rajesh V.; Vincent, Tonia; Scudamore, Cheryl; Wells, Sara; El-Amraoui, Aziz; Petit, Christine; Acevedo-Arozena, Abraham; Nolan, Patrick M.; Cox, Roger; Mallon, Anne-Marie; Brown, Steve D. M. 分享 收藏 Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growthLelli, Andrea; Miche, Vincent; de Monve, Jacques Boutet; Cortese, Matteo; Bosch-Grau, Montserrat; Aghaie, Asadollah; Perfettini, Isabelle; Dupont, Typhaine; Avan, Paul; El-Amraoui, Aziz; Petit, Christine 分享 收藏 An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cellsKamiya, Kazusaku; Michel, Vincent; Giraudet, Fabrice; Riederer, Brigitte; Foucher, Isabelle; Papal, Samantha; Perfettini, Isabelle; Le Gal, Sebastien; Verpy, Elisabeth; Xia, Weiliang; Seidler, Ursula; Georgescu, Maria-Magdalena; Avan, Paul; El-Amraoui, Aziz; Petit, Christine 分享 收藏 Successful Gene Therapy in the RPGRIP1-deficient Dog: a Large Model of Cone-Rod DystrophyLheriteau, Elsa; Petit, Lolita; Weber, Michel; Le Meur, Guylene; Deschamps, Jack-Yves; Libeau, Lyse; Mendes-Madeira, Alexandra; Guihal, Caroline; Francois, Achille; Guyon, Richard; Provost, Nathalie; Lemoine, Francoise; Papal, Samantha; El-Amraoui, Aziz; Colle, Marie-Anne; Moullier, Philippe; Rolling, Fabienne 分享 收藏 The giant spectrin V couples the molecular motors to phototransduction and Usher syndrome type I proteins along their trafficking routePapal, Samantha; Cortese, Matteo; Legendre, Kirian; Sorusch, Nasrin; Dragavon, Joseph; Sahly, Iman; Shorte, Spencer; Wolfrum, Uwe; Petit, Christine; El-Amraoui, Aziz 分享 收藏 
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