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A deafness mechanism of digenic Cx26 (GJB2) and Cx30 (GJB6) mutations: Reduction of endocochlear potential by impairment of heterogeneous gap junctional function in the cochlear lateral wall

Digenic Connexin26 (Cx26, GJB2) and Cx30 (GJB6) heterozygous mutations are the second most frequent cause of recessive deafness in humans. However, the underlying deafness mechanism remains unclear. In this study, we created different double Cx26 and Cx30 heterozygous (Cx26+/−/Cx30+/−) mouse models...

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Principais autores: Ling Mei, Jin Chen, Liang Zong, Yan Zhu, Chun Liang, Raleigh O. Jones, Hong-Bo Zhao
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier 2017-12-01
coleção:Neurobiology of Disease
Assuntos:
Gap junction
Connexin
Cx26
Cx30
Endocochlear potential
Deafness
Acesso em linha:http://www.sciencedirect.com/science/article/pii/S0969996117301857
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