Missing mitochondrial Mpv17 gene function induces tissue-specific cell-death pathway in the degenerating inner ear

@article{Gottesberge2012MissingMM,
  title={Missing mitochondrial Mpv17 gene function induces tissue-specific cell-death pathway in the degenerating inner ear},
  author={Angela-Maria Meyer zum Gottesberge and Thomas Massing and Stefan Hansen},
  journal={Cell and Tissue Research},
  year={2012},
  volume={347},
  pages={343-356}
}
The Mpv17 gene encodes a mitochondrial inner-membrane protein that has been implicated in the metabolism of reactive oxygen species. The loss of function in Mpv17-/- mice leads to early sensorineural deafness associated with severe inner ear degeneration and late onset of kidney failure. The present study demonstrates that the onset of the degeneration of the cochlear neuroepithelia is related to the onset of auditory function and appears to be first restricted to the outer hair cells (OHC… CONTINUE READING
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