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dc.contributor.author Delmaghani S
dc.contributor.author Defourny J
dc.contributor.author Aghaie A
dc.contributor.author Beurg M
dc.contributor.author Dulon D
dc.contributor.author Thelen N
dc.contributor.author Perfettini I
dc.contributor.author Zelles, Tibor
dc.contributor.author Aller M
dc.contributor.author Meyer A
dc.contributor.author Emptoz A
dc.contributor.author Giraudet F
dc.contributor.author Leibovici M
dc.contributor.author Dartevelle S
dc.contributor.author Soubigou G
dc.contributor.author Thiry M
dc.contributor.author Vizi, E. Szilveszter
dc.contributor.author Safieddine S
dc.contributor.author Hardelin JP
dc.contributor.author Avan P
dc.contributor.author Petit C
dc.date.accessioned 2018-09-26T09:29:52Z
dc.date.available 2018-09-26T09:29:52Z
dc.date.issued 2015
dc.identifier 84946234112
dc.identifier.citation pagination=894-906; journalVolume=163; journalIssueNumber=4; journalTitle=CELL;
dc.identifier.uri http://repo.lib.semmelweis.hu//handle/123456789/6306
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
dc.date.updated 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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