FIGURE: 1 / 1
The enzyme system for cleavage of glycine (glycine cleavage system; EC 22.214.171.124), 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.
Protein Aliases: Aminomethyltransferase, mitochondrial; GCVT; Glycine cleavage system T protein
Gene Aliases: AMT; EG434437; GCE; GCST; GCVT; NKH
Molecular Function: methyltransferase