Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes. - Institut Pasteur Accéder directement au contenu
Article Dans Une Revue Cell Année : 2015

Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes.

Didier Dulon

Résumé

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.
Fichier principal
Vignette du fichier
Pejvakin Manuscript Delmaghani et al CELL2015.pdf (7.54 Mo) Télécharger le fichier
Origine : Fichiers produits par l'(les) auteur(s)
Loading...

Dates et versions

pasteur-01230439 , version 1 (18-11-2015)

Licence

Copyright (Tous droits réservés)

Identifiants

Citer

Sedigheh Delmaghani, Jean Defourny, Asadollah Aghaie, Maryline Beurg, Didier Dulon, et al.. Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes.. Cell, 2015, 163 (4), pp.894-906. ⟨10.1016/j.cell.2015.10.023⟩. ⟨pasteur-01230439⟩
850 Consultations
382 Téléchargements

Altmetric

Partager

Gmail Facebook X LinkedIn More