Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes. - Institut Pasteur Access content directly
Journal Articles Cell Year : 2015

Hypervulnerability to Sound Exposure through Impaired Adaptive Proliferation of Peroxisomes.

Didier Dulon

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

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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⟩
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