Reactome: A Curated Pathway Database

Mitochondrial tRNA aminoacylation (R-HSA-379726)

Species Homo sapiens


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.

Locations in the PathwayBrowser
Additional Information
Compartment mitochondrial matrix
GO Biological Process tRNA aminoacylation for protein translation (0006418)
Literature References
pubMedId Title Journal Year
11121736 Mitochondrial tRNA import: are there distinct mechanisms? Trends Cell Biol 2000