Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes.

[en] 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.

Disciplines :

Anatomy (cytology, histology, embryology...) & physiology

Author, co-author :

Delmaghani, Sedigheh

Defourny, Jean ; Université de Liège - ULiège > Département des sciences de la vie > GIGA-R : Biologie cellulaire

Aghaie, Asadollah

Beurg, Maryline

Dulon, Didier

Thelen, Nicolas ; Université de Liège - ULiège > Département des sciences de la vie > GIGA-R : Biologie cellulaire

Perfettini, Isabelle

Zelles, Tibor

Aller, Mate

Meyer, Anais

More authors (11 more)

Language :

English

Title :

Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes.

Publication date :

November 2015

Journal title :

Cell

ISSN :

0092-8674

eISSN :

1097-4172

Publisher :

Cell Press, United States - Massachusetts

Volume :

163

Issue :

Pages :

894-906

Peer reviewed :

Peer Reviewed verified by ORBi

Commentary :

Available on ORBi :

since 04 January 2016

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Scopus citations^®

136

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without self-citations

125

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129

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