Progressive Hearing Loss in Mice Carrying a Mutation in Usp53.

Abstract

UNLABELLED Disordered protein ubiquitination has been linked to neurodegenerative disease, yet its role in inner ear homeostasis and hearing loss is essentially unknown. Here we show that progressive hearing loss in the ethylnitrosourea-generated mambo mouse line is caused by a mutation in Usp53, a member of the deubiquitinating enzyme family. USP53… (More)
DOI: 10.1523/JNEUROSCI.1965-15.2015

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