GCSH (Glycine Cleavage System Protein H (aminomethyl carrier), Lipoic Acid-containing Protein, Mitochondrial glycine cleavage system H-Protein, Part of Mitochondrial Matrix Glycine Cleavage Enzyme Complex of 4 Proteins: H-, L-, P-, and T-Proteins, GCE, NKH) (AP)

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; MIM 238300), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme; MIM 238310), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899), also called nonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes. Applications: Suitable for use in ELISA and Western Blot. Other...