Glycine dehydrogenase (decarboxylating)

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Glycine dehydrogenase (decarboxylating)
External IDs OMIM238300 MGI1341155 HomoloGene141 GeneCards: GLDC Gene
EC number
RNA expression pattern
PBB GE GLDC 204836 at tn.png
More reference expression data
Species Human Mouse
Entrez 2731 104174
Ensembl ENSG00000178445 ENSMUSG00000024827
UniProt P23378 Q91W43
RefSeq (mRNA) NM_000170 NM_138595
RefSeq (protein) NP_000161 NP_613061
Location (UCSC) Chr 9:
6.53 – 6.65 Mb
Chr 19:
30.1 – 30.18 Mb
PubMed search [1] [2]
glycine dehydrogenase (decarboxylating)
EC number
CAS number 37259-67-9
IntEnz IntEnz view
ExPASy NiceZyme view
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO

Glycine dehydrogenase [decarboxylating], mitochondrial also known as glycine cleavage system P protein or glycine decarboxylase is an enzyme that in humans is encoded by the GLDC gene.[1][2][3]


Glycine dehydrogenase (decarboxylating) (EC is an enzyme that catalyzes the following chemical reaction:

glycine + H-protein-lipoyllysine \rightleftharpoons 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 a 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).[3]

Clinical significance[edit]

Glycine encephalopathy may be due to a defect in any one of these enzymes.[3]


  1. ^ 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. 
  2. ^ 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. 
  3. ^ a b c "Entrez Gene: GLDC glycine dehydrogenase (decarboxylating)". 

Further reading[edit]