Structural highlights
Disease
[SYAM_HUMAN] Combined oxidative phosphorylation defect type 8;Hereditary diffuse leukoencephalopathy with axonal spheroids and pigmented glia;Ovarioleukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.
Function
[SYAM_HUMAN] 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.[HAMAP-Rule:MF_03133]
