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.
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Protein Aliases: AARS2; alanine tRNA ligase 2, mitochondrial (putative); Alanine--tRNA ligase, mitochondrial; Alanyl-tRNA synthetase; alanyl-tRNA synthetase 2, mitochondrial (putative); alanyl-tRNA synthetase like; AlaRS; bA444E17.1; probable alanyl-tRNA synthetase, mitochondrial
Gene Aliases: AARS2; AARSL; COXPD8; KIAA1270; LKENP; MT-ALARS; MTALARS
UniProt ID: (Human) Q5JTZ9
Entrez Gene ID: (Human) 57505
Molecular Function: aminoacyl-tRNA synthetase