Glycine Dehydrogenase (GLDC)

GCSP; NKH; Decarboxylating; Glycine Decarboxylase; Glycine Cleavage System Protein P; Glycine dehydrogenase (aminomethyl-transferring)

SPN	Q334
HGNC	4313
MGI	1341155
PubMed	21107318
RGD	1308660
UniProt	P23378
Wiki	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).

GLDC 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). The GLDC gene spans at least 135 kb and contains 25 exons. All donor and acceptor sites adhered to the canonical GT-AG rule, except for the donor site of intron 21, where a variant form GC is used instead of GT.

Reference

"Structural and expression analyses of normal and mutant mRNA encoding glycine decarboxylase: three-base deletion in mRNA causes nonketotic hyperglycinemia."
Biochem. Biophys. Res. Commun. 174:1176-1182(1991) [PubMed] [Europe PMC] [Abstract]

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"Identification of a common mutation in Finnish patients with nonketotic hyperglycinemia."
J. Clin. Invest. 90:160-164(1992) [PubMed] [Europe PMC] [Abstract]

"Recurrent mutations in P- and T-proteins of the glycine cleavage complex and a novel T-protein mutation (N145I): a strategy for the molecular investigation of patients with nonketotic hyperglycinemia (NKH)."
Mol. Genet. Metab. 72:322-325(2001) [PubMed] [Europe PMC] [Abstract]

"Nonketotic hyperglycinemia (glycine encephalopathy): laboratory diagnosis."
Mol. Genet. Metab. 74:139-146(2001) [PubMed] [Europe PMC] [Abstract]