Article
Regulation of glycine metabolism by the glycine cleavage system and conjugation pathway in mouse models of non-ketotic hyperglycinemia.
Journal of inherited metabolic disease - 1 Nov 2020
Leung Kit-Yi, De Castro Sandra C P, Santos Chloe, Savery Dawn, Prunty Helen, Gold-Diaz Diana, Bennett Stuart, Heales Simon, Copp Andrew J, Greene Nicholas D E
Abstract excerpt
Glycine abundance is modulated in a tissue-specific manner by use in biosynthetic reactions, catabolism by the glycine cleavage system (GCS), and excretion via glycine conjugation. Dysregulation of glycine metabolism is associated with multiple disorders including epilepsy, developmental delay, and birth defects. Mutation of the GCS component glycine decarboxylase (GLDC) in non-ketotic hyperglycinemia (NKH)...
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