Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome

Clarin-1, a tetraspan-like membrane protein defective in Usher syndrome type IIIA (USH3A), is essential for hair bundle morphogenesis in auditory hair cells. We report a new synaptic role for clarin-1 in mouse auditory hair cells elucidated by characterization of Clrn1 total (Clrn1(ex4–/–)) and post...

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Detalhes bibliográficos
Publicado no:J Clin Invest
Main Authors: Dulon, 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
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2018
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6063508/
https://ncbi.nlm.nih.gov/pubmed/29985171
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI94351
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