Methionyl-tRNA synthetase 2, mitochondrial (MARS2)

Methionyl-tRNA synthetase 2, mitochondrial (MARS2), is a nuclear-encoded enzyme imported into the mitochondrial matrix, where it catalyzes the charging of mitochondrial tRNA^Met with methionine, a critical step for mitochondrial protein translation essential for forming components of the oxidative phosphorylation system[1][2][7]. Deficiency or mutation in MARS2 impairs mitochondrial translation, leading to compromised respiratory chain activity, increased oxidative stress, and pathologies such as autosomal recessive spastic ataxia with leukoencephalopathy (ARSAL) and developmental delay[1][2][4]. MARS2 is also implicated in cancer biology, notably contributing to cell proliferation and survival in tumors such as gastric cancer[2]. As an essential aminoacyl-tRNA synthetase, MARS2 is thus fundamental for normal mitochondrial and cellular function, with pathogenic variants causing multisystem disease and possible oncogenic transformation.

Other names

Methionine--tRNA ligase, mitochondrialMethionine tRNA ligase 2, mitochondrialMethionyl-tRNA synthetase 2, mitochondrialMtMetRSmtMetRSSPAX3COXPD25MetRS

Future hypothetical modulators would alter mitochondrial translation by affecting methionine-tRNA ligase activity