Article
Cochlear Cell Modeling Using Disease-Specific iPSCs Unveils a Degenerative Phenotype and Suggests Treatments for Congenital Progressive Hearing Loss.
Cell reports - 3 Jan 2017
Hosoya Makoto, Fujioka Masato, Sone Takefumi, Okamoto Satoshi, Akamatsu Wado, Ukai Hideki, Ueda Hiroki R, Ogawa Kaoru, Matsunaga Tatsuo, Okano Hideyuki
Abstract excerpt
Hearing impairments are the most common symptom of congenital defects, and they generally remain intractable to treatment. Pendred syndrome, the most frequent syndromic form of hereditary hearing loss, is associated with mutations in the anion exchanger pendrin. Loss of pendrin function as an anion exchanger is thought to be causative, but rodent models do not exhibit progressive deafness. Here, we report a...
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