glycine + tRNA(Gly) + ATP => Gly-tRNA(Gly) + AMP + pyrophosphate

Stable Identifier
R-HSA-380048
Type
Reaction [transition]
Species
Homo sapiens
Compartment
cytosol
ReviewStatus
5/5
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glycine + tRNA(Gly) + ATP => Gly-tRNA(Gly) + AMP + pyrophosphate
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GARS (cytosolic glycyl tRNA synthetase) catalyzes the reaction of glycine, tRNA(Gly), and ATP to form Gly tRNA(Gly), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a dimer. Cytosolic and mitochondrial glycyl tRNA synthetase enzymes are both encoded by the same gene; the cytosolic protein lacks an aminoterminal 54 residue sequence found in the mitochondrial protein. Mutations in GARS are associated with Charcot Marie Tooth disease and distal spinal muscular atrophy, two diseases characterized by axonal dysfunction (Antonellis et al. 2006)
Literature References
PubMed ID Title Journal Year
12690580 Glycyl tRNA synthetase mutations in Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V

Antonellis, A, Ellsworth, RE, Sambuughin, N, Puls, I, Abel, A, Lee-Lin, SQ, Jordanova, A, Kremensky, I, Christodoulou, K, Middleton, LT, Sivakumar, K, Ionasescu, V, Funalot, B, Vance, JM, Goldfarb, LG, Fischbeck, KH, Green, ED

Am J Hum Genet 2003
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Catalyst Activity

glycine-tRNA ligase activity of GARS dimer [cytosol]

Physical Entity
GARS dimer [cytosol] (Homo sapiens)
Activity
glycine-tRNA ligase activity (GO:0004820)
Authored
D'Eustachio, P  (2008-11-29)
Reviewed
Antonellis, A  (2008-12-02)
Created
D'Eustachio, P  (2008-11-07)
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