基因名:
NARS2
产品别名:
DFNB94; SLM5; asnRS; NARS2; asparaginyl-tRNA synthetase 2, mitochondrial; asparaginyl-tRNA synthetase 2, mitochondrial; probable asparagine--tRNA ligase, mitochondrial; asparagine tRNA ligase 2, mitochondrial (putative); asparaginyl-tRNA synthetase 2, mitochondrial (putative); deafness, autosomal recessive 94; probable asparaginyl-tRNA synthetase, mitochondrial; 天冬氨酰tRNA连接酶2; 天冬酰tRNA合成酶2(NARS2);
背景信息:
SLM5 (mitochondrial asparaginyl-tRNA synthetase, Asparagine-tRNA ligase) is a mitochondrial protein encoded by the human gene NARS2. AsnRS belongs to the class-II aminoacyl-tRNA synthetase family. Aminoacyl tRNA synthetases (aaRS) are enzymes that catalyze the esterification of a specific amino acid or its precursor to its compatible cognate tRNA to form an aminoacyl-tRNA. The synthetase first binds ATP and the corresponding amino acid or its precursor to form an aminoacyl-adenylate and release inorganic pyrophosphate (PPi). The adenylate-aaRS complex then binds the appropriate tRNA molecule, and the amino acid is transferred from the aa-AMP to either the 2'- or 3'-OH of the last tRNA base (A76) at the 3'-end. Some synthetases also mediate a proofreading reaction to ensure high fidelity of tRNA charging; if the tRNA is found to be improperly charged, the aminoacyl-tRNA bond is hydrolyzed. SLM5 acts to attach asparagine residues to its cognate tRNA.