MGP Database

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MGP005106

UniProt Annotations

Entry Information
Gene Nametyrosyl-tRNA synthetase 2, mitochondrial
Protein EntrySYYM_HUMAN
UniProt IDQ9Y2Z4
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityATP + L-tyrosine + tRNA(Tyr) = AMP + diphosphate + L-tyrosyl-tRNA(Tyr).
DiseaseMyopathy with lactic acidosis and sideroblastic anemia 2 (MLASA2) [MIM:613561]: A rare oxidative phosphorylation disorder specific to skeletal muscle and bone marrow. Affected individuals manifest sideroblastic anemia, progressive lethargy, muscle weakness, and exercise intolerance associated with persistent lactic acidemia. {ECO:0000269|PubMed:20598274, ECO:0000269|PubMed:22504945}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionCatalyzes the attachment of tyrosine to tRNA(Tyr) in a two-step reaction: tyrosine is first activated by ATP to form Tyr- AMP and then transferred to the acceptor end of tRNA(Tyr). {ECO:0000269|PubMed:15779907}.
SimilarityBelongs to the class-I aminoacyl-tRNA synthetase family. {ECO:0000305}.
Subcellular LocationMitochondrion matrix {ECO:0000269|PubMed:15779907}.
SubunitHomodimer. {ECO:0000269|PubMed:15779907}.
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