description
Mitochondrial tRNA synthetases act in the mitochondrial matrix to catalyze the reactions of tRNAs encoded in the mitochondrial genome, their cognate amino acids, and ATP to form aminoacyl-tRNAs, AMP, and pyrophosphate (Schneider et al. 2000). The synthetase enzymes that catalyze these reactions are all encoded in the nuclear genome. In three cases, glycine, lysine, and glutamine, a single gene encodes two enzyme isoforms, one cytosolic and one mitochondrial. All other mitochondrial tRNA synthetases are encoded by genes different from the ones encoding the corresponding cytosolic enzymes

external resources
NCBI:1269711
REACTOME:R-HSA-379726
PUBMED:11121736

genes
GARS , KARS , QARS , WARS2 , FARS2 , LARS2 , HARS2 , PARS2 , PPA2 , YARS2 , SARS2 , DARS2 , IARS2 , RARS2 , VARS2 , AARS2 , CARS2 , NARS2 , TARS2 , MARS2 , EARS2 ,