GCSH Products

Description

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.[supplied by OMIM]

Bioinformatics

Entrez	2653 Human
Uniprot	AAH00790 Human
Product By Gene ID	2653
Alternate Names	GCE glycine cleavage system H protein, mitochondrial glycine cleavage system protein H (aminomethyl carrier) lipoic acid-containing protein mitochondrial glycine cleavage system H-protein NKH

Research Areas for GCSH

Find related products by research area and learn more about each of the different research areas below.

Amino Acids, Drugs and other small molecules
Cancer
Signal Transduction