serine-tRNA ligase, mitochondrial; SerRSmt; mitochondrial Seryl-tRNA synthetase; SerRS; Seryl-tRNA(Ser/sec) synthetase (SARS2, SARSM)

Function

• catalyzes the attachment of serine to tRNA(Ser)
• also able to aminoacylate tRNA(sec) with serine, to form the misacylated tRNA L-seryl-tRNA(sec), which will be further converted into selenocysteinyl-tRNA(sec)
• aminoacyl-tRNA biosynthesis
  • selenocysteinyl-tRNA(sec) biosynthesis
  • L-seryl-tRNA(sec) from L-serine & tRNA(sec): step 1/1
• homodimer: the tRNA molecule binds across the dimer

ATP + L-serine + tRNA(Ser)
<-->
AMP + diphosphate + L-seryl-tRNA(Ser)

Structure

• consists of two distinct domains
  • catalytic core
  • N-terminal extension that is involved in tRNA binding
• belongs to the class-II aminoacyl-tRNA synthetase family, type-1 seryl-tRNA synthetase subfamily

Compartment

mitochondrial matrix

Pathology

• defects in SARS2 are the cause of hyperuricemia pulmonary hypertension renal failure & alkalosis (HUPRA)

More general terms

• amino acyl tRNA synthetase-2
• mitochondrial protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=54938
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:54938
• OMIM: https://mirror.omim.org/entry/612804
• OMIM: https://mirror.omim.org/entry/613845
• UniProt: http://www.uniprot.org/uniprot/Q9NP81.html