Show simple item record Delmaghani S Defourny J Aghaie A Beurg M Dulon D Thelen N Perfettini I Zelles, Tibor Aller M Meyer A Emptoz A Giraudet F Leibovici M Dartevelle S Soubigou G Thiry M Vizi, E. Szilveszter Safieddine S Hardelin JP Avan P Petit C 2018-09-26T09:29:52Z 2018-09-26T09:29:52Z 2015
dc.identifier 84946234112
dc.identifier.citation pagination=894-906; journalVolume=163; journalIssueNumber=4; journalTitle=CELL;
dc.identifier.uri doi:10.1016/j.cell.2015.10.023
dc.description.abstract A deficiency in pejvakin, a protein of unknown function, causes a strikingly heterogeneous form of human deafness. Pejvakin-deficient (Pjvk(-/-)) mice also exhibit variable auditory phenotypes. Correlation between their hearing thresholds and the number of pups per cage suggest a possible harmful effect of pup vocalizations. Direct sound or electrical stimulation show that the cochlear sensory hair cells and auditory pathway neurons of Pjvk(-/-) mice and patients are exceptionally vulnerable to sound. Subcellular analysis revealed that pejvakin is associated with peroxisomes and required for their oxidative-stress-induced proliferation. Pjvk(-/-) cochleas display features of marked oxidative stress and impaired antioxidant defenses, and peroxisomes in Pjvk(-/-) hair cells show structural abnormalities after the onset of hearing. Noise exposure rapidly upregulates Pjvk cochlear transcription in wild-type mice and triggers peroxisome proliferation in hair cells and primary auditory neurons. Our results reveal that the antioxidant activity of peroxisomes protects the auditory system against noise-induced damage.
dc.relation.ispartof urn:issn:0092-8674
dc.title Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes
dc.type Journal Article 2018-08-31T12:07:45Z
dc.language.rfc3066 en
dc.identifier.mtmt 2976639
dc.identifier.wos 000364829700015
dc.identifier.pubmed 26544938
dc.contributor.department SE/AOK/I/Farmakológiai és Farmakoterápiás Intézet
dc.contributor.institution Semmelweis Egyetem

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