Article
Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness.
Human molecular genetics - 1 May 2003
Kudo Takayuki, Kure Shigeo, Ikeda Katsuhisa, Xia An-Ping, Katori Yukio, Suzuki Masaaki, Kojima Kanako, Ichinohe Akiko, Suzuki Yoichi, Aoki Yoko, Kobayashi Toshimitsu, Matsubara Yoichi
Abstract excerpt
Hereditary deafness affects about 1 in 2000 children and mutations in the GJB2 gene are the major cause in various ethnic groups. GJB2 encodes connexin26, a putative channel component in cochlear gap junction. However, the pathogenesis of hearing loss caused by the GJB2 mutations remains obscure. The generation of a mouse model to study the function of connexin26 during hearing has been hampered by the fact that...
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