Isoleucine-tRNA synthetase 2, mitochondrial (IARS2)

Isoleucine-tRNA synthetase 2, mitochondrial (IARS2) is a nuclear-encoded, mitochondrially localized enzyme responsible for the specific attachment of isoleucine to its cognate mitochondrial tRNA (tRNA(Ile)). IARS2 is part of the class I aminoacyl-tRNA synthetase family and is essential for translation of mitochondrial DNA-encoded proteins, thereby maintaining mitochondrial function and ATP production[3][9][2][7][8]. Mutations in IARS2 disrupt mitochondrial protein synthesis and lead to a constellation of mitochondrial diseases, including CAGSSS, Leigh syndrome, isolated cataracts, and others[5][3][4]. There are no approved drugs targeting mitochondrial IARS2, but its central function renders it critically important for cell viability[3][5][2].

Other names

IARS2Isoleucyl-tRNA synthetase 2, mitochondrialIsoleucine--tRNA ligase, mitochondrialIleRSFLJ10326CAGSSSILERSProbable isoleucine--tRNA ligase, mitochondrialIsoleucyl-tRNA synthetase, mitochondrialMitochondrial isoleucine tRNA synthetase

Not applicable (no known drugs targeting IARS2)[3][5]. Mechanistically, for theoretical agents, inhibition would block mitochondrial protein synthesis by preventing aminoacylation of mitochondrial tRNA(Ile), leading to mitochondrial dysfunction[2][3].