Article
Mature middle and inner ears express Chd7 and exhibit distinctive pathologies in a mouse model of CHARGE syndrome.
Hearing research - 1 Dec 2011
Hurd Elizabeth A, Adams Meredith E, Layman Wanda S, Swiderski Donald L, Beyer Lisa A, Halsey Karin E, Benson Jennifer M, Gong Tzy-Wen, Dolan David F, Raphael Yehoash, Martin Donna M
Abstract excerpt
Heterozygous mutations in the gene encoding chromodomain-DNA-binding-protein 7 (CHD7) cause CHARGE syndrome, a multiple anomaly condition which includes vestibular dysfunction and hearing loss. Mice with heterozygous Chd7 mutations exhibit semicircular canal dysgenesis and abnormal inner ear neurogenesis, and are an excellent model of CHARGE syndrome. Here we characterized Chd7 expression in mature middle and...
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