arginine + tRNA(Arg) + ATP => Arg-tRNA(Arg) + AMP + pyrophosphate

Stable Identifier
R-HSA-380224
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
17847012 Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia

Edvardson, S, Shaag, A, Kolesnikova, O, Gomori, JM, Tarassov, I, Einbinder, T, Saada, A, Elpeleg, O

Am J Hum Genet 2007
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
arginine-tRNA ligase activity of RARS2 [mitochondrial matrix]
Physical Entity
Activity
Authored
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Created