Arginyl-tRNA synthetase 1 (RARS1) (RARS1)

Arginyl-tRNA synthetase 1 (RARS1) is a vital cytosolic enzyme responsible for the aminoacylation of tRNA with arginine, a prerequisite for protein synthesis (UniProt: P54136). It functions as a component of the multi-tRNA synthetase complex (MSC), which is involved in various non-canonical cellular processes such as signaling and RNA transport (PubMed: 25512440). Mutations in the RARS1 gene are linked to Hypomyelinating leukodystrophy 9 (HLD9), a severe neurodevelopmental disorder characterized by deficient myelin formation (PubMed: 24569165). Furthermore, RARS1 is frequently overexpressed in several types of cancer, where it promotes tumor growth and is associated with poor prognosis (PubMed: 28651077). Although no clinical drugs currently target RARS1, research into small-molecule inhibitors like arginyl-sulfamoyl adenosine is ongoing for potential anti-proliferative and antimicrobial applications (PubMed: 11752152). Targeting this enzyme requires high selectivity to avoid disrupting mitochondrial protein synthesis or causing systemic toxicity due to its essential role in healthy cells.

Other names

RARSArgRSArginine--tRNA ligase, cytoplasmicArginyl-tRNA synthetase 1

Competitive inhibition of the aminoacyl-tRNA synthetase activity, preventing the charging of tRNA with arginine and subsequently inhibiting protein translation.