Article
Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic emission.
Neuroscience - 15 Dec 2009
Minekawa A, Abe T, Inoshita A, Iizuka T, Kakehata S, Narui Y, Koike T, Kamiya K, Okamura H-O, Shinkawa H, Ikeda K
Abstract excerpt
Mutations in the connexin26 gene (GJB2) are the most common genetic cause of congenital bilateral non-syndromic sensorineural hearing loss. Transgenic mice were established carrying human Cx26 with the R75W mutation that was identified in a deaf family with autosomal dominant negative inheritance [Kudo T et al. (2003) Hum Mol Genet 12:995-1004]. A dominant-negative Gjb2 R75W transgenic mouse model shows...
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