Reaction: glycine + tRNA(Gly) + ATP => Gly-tRNA(Gly) + AMP + pyrophosphate
- in pathway: Mitochondrial tRNA aminoacylation
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).
Reaction - small molecule participants:
AMP [mitochondrial matrix]
PPi [mitochondrial matrix]
Gly [mitochondrial matrix]
ATP [mitochondrial matrix]
Reactome.org reaction link: R-HSA-380240
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Reaction input - small molecules:
glycine zwitterion
ATP(4-)
Reaction output - small molecules:
adenosine 5'-monophosphate(2-)
diphosphate(3-)
Reactome.org link: R-HSA-380240