AARS2 Proteins

Protein Information

The protein encoded by this gene belongs to the class-II aminoacyl-tRNA synthetase family. Aminoacyl-tRNA synthetases play critical roles in mRNA translation by charging tRNAs with their cognate amino acids. The encoded protein is a mitochondrial enzyme that specifically aminoacylates alanyl-tRNA. Mutations in this gene are a cause of combined oxidative phosphorylation deficiency 8. Catalyzes the attachment of alanine to tRNA(Ala) in a two-step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain.

Synonyms

AARS2; AARSL; alanine tRNA ligase 2, mitochondrial (putative); Alanine--tRNA ligase, mitochondrial; alanine--tRNA ligase, mitochondrial {ECO:0000255; Alanyl-tRNA synthetase; alanyl-tRNA synthetase {ECO:0000255; alanyl-tRNA synthetase 2, mitochondrial; alanyl-tRNA synthetase 2, mitochondrial (putative); alanyl-tRNA synthetase like; alanyl-tRNA synthetase, mitochondrial; AlaRS; alaRS {ECO:0000255; bA444E17.1; COXPD8; Gm89; HAMAP-Rule:MF_03133}; Kiaa1270; LKENP; MTALARS; MT-ALARS; probable alanyl-tRNA synthetase, mitochondrial