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Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein is the P protein, which binds to glycine and enables the methylamine group from glycine to be transferred to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH).
D030049L12Rik; D19Wsu57e; GCE; GCSP; GLDC; Glycine cleavage system P protein; glycine cleavage system protein P; glycine decarboxylase; glycine decarboxylase P-protein; glycine decarboxylase, glycine cleavage system protein P); glycine dehydrogenase (aminomethyl-transferring); glycine dehydrogenase (decarboxylating); glycine dehydrogenase (decarboxylating), mitochondrial; glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P); glycine dehydrogenase [decarboxylating], mitochondrial; HYGN1; RP11-472F14.1
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