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

Stable Identifier
R-HSA-380224
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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arginine + tRNA(Arg) + ATP => Arg-tRNA(Arg) + AMP + pyrophosphate
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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

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

Am J Hum Genet 2007
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Catalyst Activity

arginine-tRNA ligase activity of RARS2 [mitochondrial matrix]

Physical Entity
RARS2 [mitochondrial matrix] (Homo sapiens)
Activity
arginine-tRNA ligase activity (GO:0004814)
Authored
D'Eustachio, P  (2008-11-29)
Reviewed
Antonellis, A  (2008-12-02)
Created
D'Eustachio, P  (2008-11-08)
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