Glycine dehydrogenase (decarboxylating)

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AliasesGLDC, GCE, GCSP, HYGN1, Glycine dehydrogenase, glycine decarboxylase
External IDsOMIM: 238300 MGI: 1341155 HomoloGene: 141 GeneCards: GLDC
Gene location (Human)
Chromosome 9 (human)
Chr.Chromosome 9 (human)[1]
Chromosome 9 (human)
Genomic location for GLDC
Genomic location for GLDC
Band9p24.1Start6,532,464 bp[1]
End6,645,783 bp[1]
RNA expression pattern
PBB GE GLDC 204836 at fs.png
More reference expression data
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC)Chr 9: 6.53 – 6.65 MbChr 19: 30.1 – 30.18 Mb
PubMed search[3][4]
View/Edit HumanView/Edit Mouse
glycine decarboxylase
EC number1.4.4.2
CAS number37259-67-9
IntEnzIntEnz view
ExPASyNiceZyme view
MetaCycmetabolic pathway
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO

Glycine decarboxylase also known as glycine cleavage system P protein or glycine dehydrogenase is an enzyme that in humans is encoded by the GLDC gene.[5][6][7]


Glycine decarboxylase (EC is an enzyme that catalyzes the following chemical reaction:

glycine + H-protein-lipoyllysine H-protein-S-aminomethyldihydrolipoyllysine + CO2

Thus, the two substrates of this enzyme are glycine and H-protein-lipoyllysine, whereas its two products are H-protein-S-aminomethyldihydrolipoyllysine and CO2.

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-NH2 group of donors with a disulfide as acceptor. This enzyme participates in glycine, serine and threonine metabolism. It employs one cofactor, pyridoxal phosphate.


Glycine decarboxylase is the P-protein of the glycine cleavage system in eukaryotes. The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor. Carbon dioxide is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein.

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).[7]

Clinical significance[edit]

Glycine encephalopathy is due to defects in GLDC or AMT of the glycine cleavage system.[7]


  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000178445 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000024827 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Kume A, Koyata H, Sakakibara T, Ishiguro Y, Kure S, Hiraga K (Mar 1991). "The glycine cleavage system. Molecular cloning of the chicken and human glycine decarboxylase cDNAs and some characteristics involved in the deduced protein structures". J Biol Chem. 266 (5): 3323–9. PMID 1993704.
  6. ^ Kure S, Narisawa K, Tada K (Mar 1991). "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 (3): 1176–82. doi:10.1016/0006-291X(91)91545-N. PMID 1996985.
  7. ^ a b c "Entrez Gene: GLDC glycine dehydrogenase (decarboxylating)".

Further reading[edit]