GCSH

Glycine cleavage system H protein, mitochondrial is a is_associated_with::protein that in humans is encoded by the GCSH is_associated_with::gene.

Function
The enzyme system for cleavage of glycine (is_associated_with::glycine cleavage system), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a is_associated_with::lipoic acid-containing protein; this protein), T protein (a tetrahydrofolate-requiring is_associated_with::aminomethyltransferase enzyme), and L protein (a lipoamide dehydrogenase). The H protein shuttles the methylamine group of glycine from the P protein to the T protein.

Clinical significance
Glycine encephalopathy, also called nonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes.