The glycine cleavage system is comprised of AMT (known as Protein T), GCSH (known as Protein H), DLD (known as Protein L) and GLDC (known as Protein P), all of which work together to catalyze the cleavage and degradation of glycine. GLDC (glycine dehydrogenase ), also known as GCE, GCSP (glycine cleavage system P protein) or HYGN1, is a 1,020 amino acid protein that localizes to the mitochondria and belongs to the gcvP family. GLDC binds to glycine and enables the methylamine group from glycine to be transferred to the Protein T. GLDC exists as a homodimer and utilizes pyridoxal phosphate as a cofactor. Mutations in the gene encoding GLDC leads to nonketotic hyperglycinemia (NKH), also known as glycine encephalopathy (GCE), an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
Synonyms: GCE, GCSP, GCSP_HUMAN, GLDC, Glycine cleavage system P protein, glycine cleavage system protein P, Glycine decarboxylase, glycine decarboxylase P protein, Glycine dehydrogenase decarboxylating mitochondrial, Glycine dehydrogenase [decarboxylating], mitochondrial, Gycine dehydrogenase decarboxylating, HYGN1, MGC138198, MGC138200, NKH.