Tmc1 is necessary for normal functional maturation and survival of inner and outer hair cells in the mouse cochlea.

@article{Marcotti2006Tmc1IN,
  title={Tmc1 is necessary for normal functional maturation and survival of inner and outer hair cells in the mouse cochlea.},
  author={Walter Marcotti and Alexandra Erven and Stuart L Johnson and Karen P. Steel and Corn{\'e} J Kros},
  journal={The Journal of physiology},
  year={2006},
  volume={574 Pt 3},
  pages={
          677-98
        }
}
The deafness (dn) and Beethoven (Bth) mutant mice are models for profound congenital deafness (DFNB7/B11) and progressive hearing loss (DFNA36), respectively, caused by recessive and dominant mutations of transmembrane cochlear-expressed gene 1 (TMC1), which encodes a transmembrane protein of unknown function. In the mouse cochlea Tmc1 is expressed in both outer (OHCs) and inner (IHCs) hair cells from early stages of development. Immature hair cells of mutant mice seem normal in appearance and… CONTINUE READING

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