Asparaginyl-tRNA synthetase 2, mitochondrial (NARS2)

Asparaginyl-tRNA synthetase 2, mitochondrial (NARS2) is a nuclear-encoded enzyme imported into mitochondria, classified as a class II aminoacyl-tRNA synthetase[2][3]. It catalyzes the ATP-dependent attachment of asparagine to its cognate mitochondrial tRNA (tRNA^Asn), which is essential for mitochondrial protein synthesis and cellular energy metabolism[2]. Structurally, NARS2 functions as a homodimer, comprising an anticodon-binding domain and a catalytic domain—unlike its paralog NARS1, it lacks an N-terminal editing domain[1][3]. Pathogenic mutations in NARS2 disrupt mitochondrial translation, impair electron transport chain function, and are implicated in autosomal recessive deafness (DFNB94), combined oxidative phosphorylation deficiency 24 (COXPD24), and Leigh syndrome, with neurological and multisystem phenotypes depending on mutation severity[3]. Currently, no drugs directly target NARS2; its clinical impact is primarily through genetic and metabolic investigations[2][3].

Other names

Asparaginyl-tRNA synthetase, mitochondrialAsnRSFLJ23441SLM5Asparagine--tRNA ligase, mitochondrialasparagine tRNA ligase 2, mitochondrial (putative)DFNB94deafness, autosomal recessive 94probable asparaginyl-tRNA synthetase, mitochondrial

null