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Article

Tmprss3, a transmembrane serine protease deficient in human DFNB8/10 deafness, is critical for cochlear hair cell survival at the onset of hearing.

The Journal of biological chemistry - 13 May 2011

Fasquelle Lydie, Scott Hamish S, Lenoir Marc, Wang Jing, Rebillard Guy, Gaboyard Sophie, Venteo Stéphanie, François Florence, Mausset-Bonnefont Anne-Laure, Antonarakis Stylianos E, Neidhart Elizabeth, Chabbert Christian, Puel Jean-Luc, Guipponi Michel, Delprat Benjamin

Abstract excerpt

Mutations in the type II transmembrane serine protease 3 (TMPRSS3) gene cause non-syndromic autosomal recessive deafness (DFNB8/10), characterized by congenital or childhood onset bilateral profound hearing loss. In order to explore the physiopathology of TMPRSS3 related deafness, we have generated an ethyl-nitrosourea-induced mutant mouse carrying a protein-truncating nonsense mutation in Tmprss3 (Y260X) and...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 21454591
DOI: 10.1074/jbc.M110.190652
PMCID: PMC3089580

Genes

TMPRSS3

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