Ontology highlight
ABSTRACT:
SUBMITTER: Tischner C
PROVIDER: S-EPMC4380071 | biostudies-literature | 2015 Apr
REPOSITORIES: biostudies-literature
Tischner Christin C Hofer Annette A Wulff Veronika V Stepek Joanna J Dumitru Iulia I Becker Lore L Haack Tobias T Kremer Laura L Datta Alexandre N AN Sperl Wolfgang W Floss Thomas T Wurst Wolfgang W Chrzanowska-Lightowlers Zofia Z De Angelis Martin Hrabe MH Klopstock Thomas T Prokisch Holger H Wenz Tina T
Human molecular genetics 20141230 8
Mitochondrial diseases often exhibit tissue-specific pathologies, but this phenomenon is poorly understood. Here we present regulation of mitochondrial translation by the Mitochondrial Translation Optimization Factor 1, MTO1, as a novel player in this scenario. We demonstrate that MTO1 mediates tRNA modification and controls mitochondrial translation rate in a highly tissue-specific manner associated with tissue-specific OXPHOS defects. Activation of mitochondrial proteases, aberrant translation ...[more]