Article
A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth.
Development (Cambridge, England) - 1 Apr 2008
Lefèvre Gaelle, Michel Vincent, Weil Dominique, Lepelletier Léa, Bizard Emilie, Wolfrum Uwe, Hardelin Jean-Pierre, Petit Christine
Abstract excerpt
The planar polarity and staircase-like pattern of the hair bundle are essential to the mechanoelectrical transduction function of inner ear sensory cells. Mutations in genes encoding myosin VIIa, harmonin, cadherin 23, protocadherin 15 or sans cause Usher syndrome type I (USH1, characterized by congenital deafness, vestibular dysfunction and retinitis pigmentosa leading to blindness) in humans and hair bundle...
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