Glycine—tRNA ligase

Glycine—tRNA ligase also known as glycyl-tRNA synthetase is an is_associated_with::enzyme that in humans is encoded by the GARS is_associated_with::gene.

Function
This gene encodes glycyl-tRNA synthetase, one of the aminoacyl-tRNA synthetases that charge tRNAs with their cognate amino acids. The encoded enzyme is an (alpha)2 dimer which belongs to the class II family of tRNA synthetases.

Reaction
In is_associated_with::enzymology, a glycine-tRNA ligase is an is_associated_with::enzyme that catalyzes the is_associated_with::chemical reaction


 * ATP + glycine + tRNAGly $$\rightleftharpoons$$ AMP + diphosphate + glycyl-tRNAGly

The 3 substrates of this enzyme are ATP, is_associated_with::glycine, and is_associated_with::tRNA(Gly), whereas its 3 products are AMP, is_associated_with::diphosphate, and is_associated_with::glycyl-tRNA(Gly).

This enzyme belongs to the family of is_associated_with::ligases, to be specific those forming carbon-oxygen bonds in aminoacyl-tRNA and related compounds. The systematic name of this enzyme class is glycine:tRNAGly ligase (AMP-forming). Other names in common use include glycyl-tRNA synthetase, glycyl-transfer ribonucleate synthetase, glycyl-transfer RNA synthetase, glycyl-transfer ribonucleic acid synthetase, and glycyl translase. This enzyme participates in is_associated_with::glycine, serine and threonine metabolism and is_associated_with::aminoacyl-trna biosynthesis.

Interactions
Glycyl-tRNA synthetase has been shown to interact with is_associated_with::EEF1D.

Clinical relevance
Glycyl-tRNA synthetase has been shown to be a target of autoantibodies in the human autoimmune diseases, is_associated_with::polymyositis or is_associated_with::dermatomyositis.

The is_associated_with::peripheral neuropathy is_associated_with::Charcot-Marie-Tooth disease type 2D (CMT2D) has been liked to dominant mutations in GARS. CMT2D usually manifests during the teenage years, and results in muscle weakness predominantly in the hands and feet. Two mouse models of CMT2D have been used to better understand the disease, identifying that the disorder is caused by a toxic gain of function of the mutant glycine-tRNA ligase protein. The CMT2D mice display peripheral nerve is_associated_with::axon degeneration and defective development of the is_associated_with::neuromuscular junction.