tyrosine-tRNA ligase, mitochondrial; mitochondrial tyrosyl-tRNA synthetase; TyrRS (YARS2)

Function: - catalyzes the attachment of tyrosine to tRNA(Tyr) in a two-step reaction: tyrosine is first activated by ATP to form Tyr-AMP & then transferred to the acceptor end of tRNA(Tyr) ATP + L-tyrosine + tRNA(Tyr) AMP + diphosphate + L-tyrosyl-tRNA(Tyr) Structure: - homodimer - belongs to the class-I aminoacyl-tRNA synthetase family Compartment: mitochondrial matrix Pathology: - defects in YARS2 are the cause of myopathy with lactic acidosis & sideroblastic anemia type 2

General

amino acyl tRNA synthetase-1 mitochondrial protein oligomerizing protein phosphoprotein

Properties

SIZE: entity length = 477 aa MW = 53 kD COMPARTMENT: mitochondrial matrix MOTIF: "HIGH" region {82-91} "KMSKS" region {281-285} MOTIF: ATP-binding site NAME: ATP-binding site SITE: 284-284 Tyr phosphorylation site {Y426} Ser phosphorylation site {S435}

Database Correlations

OMIM correlations UniProt Q9Y2Z4 Pfam PF00579 Entrez Gene 51067 Kegg hsa:51067 ENZYME 6.1.1.1

References

UniProt :accession Q9Y2Z4