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Literature summary for 1.4.1.27 extracted from

  • Leung, K.Y.; De Castro, S.C.P.; Santos, C.; Savery, D.; Prunty, H.; Gold-Diaz, D.; Bennett, S.; Heales, S.; Copp, A.J.; Greene, N.D.E.
    Regulation of glycine metabolism by the glycine cleavage system and conjugation pathway in mouse models of non-ketotic hyperglycinemia (2020), J. Inherit. Metab. Dis., 43, 1186-1198 .
    View publication on PubMed

Organism

Organism UniProt Comment Textmining
Mus musculus Q91W43 i.e. glycine dehydrogenase component P-protein, cf. EC 1.4.4.2
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Synonyms

Synonyms Comment Organism
GLDC glycine dehydrogenase Mus musculus

General Information

General Information Comment Organism
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