Hypervulnerability to sound-exposure through impaired adaptive proliferation of peroxisomes

ABSTRACT: A deficiency of pejvakin, a protein of unknown function, causes a strikingly heterogeneous form of deafness. Pejvakin-deficient (Pjvk-/-) mice also exhibited variable auditory phenotypes. Correlation between their hearing thresholds and the number of pups per cage suggested a possible harmful effect of pup vocalizations. Direct sound or electrical stimulation showed that the cochlear sensory hair cells and auditory pathway neurons of Pjvk-/- mice and patients were exceptionally vulnerable to sound. Pjvk-/- cochleas displayed features of marked oxidative stress and impaired anti-oxidant defenses. We showed that pejvakin is associated with peroxisomes, and is required for the oxidative stress-induced proliferation of these organelles. In Pjvk-/- hair cells, peroxisomes displayed structural a

ORGANISM(S): Mus musculus

SUBMITTER: Sedigheh Delmaghani 

PROVIDER: E-GEOD-72722 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress