Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.

@article{Kharkovets2006MiceWA,
  title={Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.},
  author={Tatjana Kharkovets and Karin Dedek and Hannes Maier and Michaela Schweizer and Darina Khimich and R{\'e}gis Nouvian and Vitya Vardanyan and Rudolf Leuwer and Tobias Moser and Thomas J Jentsch},
  journal={The EMBO journal},
  year={2006},
  volume={25 3},
  pages={642-52}
}
KCNQ4 is an M-type K+ channel expressed in sensory hair cells of the inner ear and in the central auditory pathway. KCNQ4 mutations underlie human DFNA2 dominant progressive hearing loss. We now generated mice in which the KCNQ4 gene was disrupted or carried a dominant negative DFNA2 mutation. Although KCNQ4 is strongly expressed in vestibular hair cells, vestibular function appeared normal. Auditory function was only slightly impaired initially. It then declined over several weeks in Kcnq4… CONTINUE READING
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