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

More general terms

• amino acyl tRNA synthetase-1
• mitochondrial protein
• oligomerizing protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=51067
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:51067
• OMIM: https://mirror.omim.org/entry/610957
• OMIM: https://mirror.omim.org/entry/613561
• UniProt: http://www.uniprot.org/uniprot/Q9Y2Z4.html