MGP Database

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MGP001224

UniProt Annotations

Entry Information
Gene Nameglycine dehydrogenase (decarboxylating)
Protein EntryGCSP_HUMAN
UniProt IDP23378
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityGlycine + [glycine-cleavage complex H protein]-N(6)-lipoyl-L-lysine = [glycine-cleavage complex H protein]-S-aminomethyl-N(6)-dihydrolipoyl-L-lysine + CO(2).
CofactorName=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
DiseaseNon-ketotic hyperglycinemia (NKH) [MIM:605899]: Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. {ECO:0000269|PubMed:11286506, ECO:0000269|PubMed:11592811, ECO:0000269|PubMed:1634607, ECO:0000269|PubMed:1996985}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationStimulated by lipoic acid. Inhibited in presence of methylamine (By similarity). {ECO:0000250}.
FunctionThe glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH).
SimilarityBelongs to the GcvP family. {ECO:0000305}.
Subcellular LocationMitochondrion.
SubunitInteracts with GCSH (By similarity). Homodimer. The glycine cleavage system is composed of four proteins: P (GLDC), T (GCST), L (DLD) and H (GCSH). {ECO:0000250}.
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