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

Stable Identifier
Reaction [transition]
Homo sapiens
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GARS (mitochondrial 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 (Antonellis et al. 2007).

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

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

Am J Hum Genet 2003
Catalyst Activity

glycine-tRNA ligase activity of GARS dimer [mitochondrial matrix]

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