Mitochondria are central eukaryotic organelles with activities in a number of pathways key to cellular life, like energy production and control, and with major impacts in diseases, ageing and death. Of endosymbiotic origin, human mitochondria have kept only a limited genome (16,5Kb) coding for a few proteins (13 respiratory chain subunits) and for translation-related RNAs (2 rRNAs and 22 tRNAs). In mammalians, the mutation rate of mitochondrial (mt)-DNA is high, generating the peculiar situation where mt-RNAs are rather degenerated as compared to their bacterial ancestors and yet have to be recognized by nucleus-encoded partner proteins. Over the last years, numerous clinically relevant mutations within mt- and nuclear- genomes affecting molecules implicated in the mitochondrial translation were correlated to a variety of human disorders.
The general objectives of the team are to decipher the structure/function relationships of macromolecules forming the translation machinery in healthy as well as under pathological contexts. Focuses are made on mitochondrial aminoacylation systems [tRNAs and aminoacyl-tRNA synthetases (aaRSs)], using approaches combining mainly biochemistry, molecular and cellular biology, and bioinformatic.
Our work is supported by the LabEx MitoCross
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