tRNA modification landscape selectively controls mitochondrial translation efficiency in MERRF. tRNA modification landscape selectively controls mitochondrial translation efficiency in MERRF

We identified a novel N1-methyladenosine (m1A) modification in tRNALys with distinct effects on the synthesis and stabilities of the 13 mitochondrial proteins. Moreover, we observed regulated RNA modifications of mitochondrial tRNAs for the control of mitochondrial gene expression. Collectively, our data provide unprecedented insight into the regulation of mitochondrial tRNAs and reveal greater complexity to the molecular pathogenesis of MERRF. Overall design: Comprehensive biochemical and sequencing analysis of wild-type and MERRF samples treated or not with AlkB demethylase (DM)