GLDC

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 P protein, which binds to glycine and enables the methylamine group from glycine to be transferred to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH).[provided by RefSeq, Jan 2010]

Full Name

Glycine Decarboxylase

Function

The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO2 is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH).

Biological Process

Glycine catabolic process Source: UniProtKB
Glycine decarboxylation via glycine cleavage system Source: GO_Central
Protein-containing complex assembly Source: Ensembl
Response to lipoic acid Source: UniProtKB
Response to methylamine Source: UniProtKB

Cellular Location

Mitochondrion

Involvement in disease

Non-ketotic hyperglycinemia (NKH):
Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.