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.
