Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial 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). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.
GCSH; glycine cleavage system protein H (aminomethyl carrier); glycine cleavage system H protein, mitochondrial; lipoic acid containing protein; mitochondrial; GCE; GCSH_HUMAN; Glycine cleavage system H protein; Glycine cleavage system H protein mitochondrial; Glycine cleavage system protein H; Mitochondrial glycine cleavage system H protein; NKH; OTTHUMP00000174971; lipoic acid-containing protein; mitochondrial glycine cleavage system H-protein; GCE, NKH; H-PROTEIN; hydrogen carrier protein