Reaction: arginine + tRNA(Arg) + ATP => Arg-tRNA(Arg) + AMP + pyrophosphate
- in pathway: Mitochondrial tRNA aminoacylation
RARS2 (mitochondrial arginyl tRNA synthetase) catalyzes the reaction of arginine, mitochondrial tRNA(Arg), and ATP to form Arg tRNA(Arg), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase. Homozygosity for RARS2 mutations has been associated with pontocerebellar hypoplasia – a disease characterized by a severe reduction in cerebellum and brainstem size (Edvardson et al. 2007).
Reaction - small molecule participants:
AMP [mitochondrial matrix]
PPi [mitochondrial matrix]
ATP [mitochondrial matrix]
L-Arg [mitochondrial matrix]
Reactome.org reaction link: R-HSA-380224
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Reaction input - small molecules:
ATP(4-)
L-argininium(1+)
Reaction output - small molecules:
adenosine 5'-monophosphate(2-)
diphosphate(3-)
Reactome.org link: R-HSA-380224