GCSH: Proteins and Enzymes

The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to themitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase;MIM 238300), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme; MIM238310), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899), also callednonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes.(supplied by OMIM)