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

@article{Fasquelle2011Tmprss3AT,
  title={Tmprss3, a transmembrane serine protease deficient in human DFNB8/10 deafness, is critical for cochlear hair cell survival at the onset of hearing.},
  author={Lydie Fasquelle and Hamish S. Scott and Marc Lenoir and Jing Wang and Guy Rebillard and Sophie Gaboyard and St{\'e}phanie Vent{\'e}o and Florence François and Anne-Laure Mausset-Bonnefont and Stylianos E. Antonarakis and Elizabeth Neidhart and Christian Chabbert and Jean-Luc Puel and Michel Guipponi and Benjamin Delprat},
  journal={The Journal of biological chemistry},
  year={2011},
  volume={286 19},
  pages={
          17383-97
        }
}
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 characterized the functional and histological consequences of Tmprss3 deficiency… CONTINUE READING

Citations

Publications citing this paper.
SHOWING 1-10 OF 20 CITATIONS

Similar Papers

Loading similar papers…