Stria Vascularis Dysfunction in a Mouse Model of Mitochondrial Hearing Loss
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Authors
McKay, Sharen
Yan, Wayne
Song, Lei
Raimundo, Nuno
Santos-Sacchi, Joseph
Shadel, Gerald S.
Issue Date
2014-03-28
Type
Presentation
Language
en_US
Keywords
Faculty research day , Health sciences , Mitochondrial hearing loss
Alternative Title
Abstract
We recently described a transgenic mouse model of hearing loss induced by over-expression of the mitochondrial ribosomal RNA (rRNA) methyltransferase, TFB1M (Tg-TFB1M). These mice recapitulate maternally inherited deafness caused by the human A1555G mtDNA mutation, which results in increased methylation of the 12S rRNA in mitochondrial ribosomes and tissue-specific susceptibility to apoptosis. The present study aims to identify the specific cellular and tissue based pathologies underlying this form of deafness.