Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | Q91W43 | i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2 | - |
Synonyms | Comment | Organism |
---|---|---|
GLDC | glycine dehydrogenase | Mus musculus |
General Information | Comment | Organism |
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physiological function | mutations in Gldc result in severe or mild elevations of plasma glycine and model non-ketotic hyperglycinemia. Liver of Gldc-deficient mice accumulates glycine and numerous glycine derivatives, including multiple acylglycines. Levels of dysregulated metabolites increase with age and are normalised by liver-specific rescue of Gldc expression. Brain tissue exhibits increased abundance of glycine, as well as derivatives including guanidinoacetate. Elevation of brain tissue glycine occurs even in the presence of only mildly elevated plasma glycine in mice carrying a missense allele of Gldc. Treatment with benzoate enhances hepatic glycine conjugation thereby lowering plasma and tissue glycine. Administration of glycine conjugation pathway intermediate, cinnamate, similarly achieves normalisation of liver glycine derivatives and circulating glycine | Mus musculus |