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

Stable Identifier
R-HSA-380240
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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

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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
glycine-tRNA ligase activity of GARS dimer [mitochondrial matrix]
Physical Entity
Activity
Authored
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Created