arginine-tRNA ligase, mitochondrial; mitochondrial Arginyl-tRNA synthetase; ArgRS (RARS2 RARSL)

Function

-..........-

ATP + L-arginine + tRNA(Arg)
         <-->
AMP + diphosphate + L-arginyl-tRNA(Arg)

Structure

• belongs to the class-I aminoacyl-tRNA synthetase family

Compartment

mitochondrial matrix

Pathology

• defects in RARS2 are the cause of pontocerebellar hypoplasia type 6

More general terms

• amino acyl tRNA synthetase-2
• mitochondrial protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=57038
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:57038
• OMIM: https://mirror.omim.org/entry/611523
• OMIM: https://mirror.omim.org/entry/611524
• UniProt: http://www.uniprot.org/uniprot/Q5T160.html