RARS2, Official Symbol RARS2provided by HGNC Official Full Name arginyl-tRNA synthetase 2, mitochondrialprovided by HGNC Primary source HGNC:HGNC:21406 See related Ensembl:ENSG00000146282 MIM:611524; AllianceGenome:HGNC:21406 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as PCH6; ArgRS; RARSL; DALRD2; PRO1992 Summary This nuclear gene encodes a protein that localizes to the mitochondria, where it catalyzes the transfer of L-arginine to its cognate tRNA, an important step in translation of mitochondrially-encoded proteins. Defects in this gene are a cause of pontocerebellar hypoplasia type 6 (PCH6). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016] Expression Ubiquitous expression in adrenal (RPKM 9.8), testis (RPKM 8.4) and 25 other tissues See more Orthologs mouse all