Gene:
phenylalanine-tRNA synthetase 2 (mitochondrial)
Description:
Aminoacyl-tRNA synthetases are a class of enzymes that charge tRNAs with their cognate amino acids. FARS1 encodes a mitochondrially-located phenylalanine-tRNA synthetase (PheRS) which consists of a single polypeptide chain unlike the (alpha-beta)2 structure of the prokaryotic and eukaryotic cytoplasmic forms of PheRS. Structure analysis and catalytic properties indicate mitochondrial PheRSs may constitute a class of PheRS distinct from the enzymes found in prokaryotes and the eukaryotic cytoplasm.