Article
Modeling and Preventing Progressive Hearing Loss in Usher Syndrome III.
Scientific reports - 18 Oct 2017
Geng Ruishuang, Omar Akil, Gopal Suhasini R, Chen Daniel H-C, Stepanyan Ruben, Basch Martin L, Dinculescu Astra, Furness David N, Saperstein David, Hauswirth William, Lustig Lawrence R, Alagramam Kumar N
Abstract excerpt
Usher syndrome type III (USH3) characterized by progressive loss of vision and hearing is caused by mutations in the clarin-1 gene (CLRN1). Clrn1 knockout (KO) mice develop hair cell defects by postnatal day 2 (P2) and are deaf by P21-P25. Early onset profound hearing loss in KO mice and lack of information about the cochlear cell type that requires Clrn1 expression pose challenges to therapeutic investigation....
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