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Article

Progressive Hearing Loss in Mice Carrying a Mutation in Usp53.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 25 Nov 2015

Kazmierczak Marcin, Harris Suzan L, Kazmierczak Piotr, Shah Prahar, Starovoytov Valentin, Ohlemiller Kevin K, Schwander Martin

Abstract excerpt

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 contains a catalytically inactive ubiquitin-specific protease domain and is...

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Source

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Identifiers

PMID: 26609154
DOI: 10.1523/JNEUROSCI.1965-15.2015
PMCID: PMC4659823

Genes

USP53
TJP1
TJP2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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