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The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 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). Glycine encephalopathy (GCE) may be due to a defect in any one of these enzymes.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: Aminomethyltransferase, mitochondrial; GCVT; Glycine cleavage system T protein
基因别名: AMT; EG434437; GCE; GCST; GCVT; NKH
UniProt ID: (Human) P48728, (Mouse) Q8CFA2
Entrez Gene ID: (Human) 275, (Mouse) 434437
分子生物学功能: methyltransferase