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GLDC The glycine cleavage system catalyzes the degradation of glycine. The P protein 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. Defects in GLDC are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvP family. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - glycine, serine and threonine; EC; Oxidoreductase
Chromosomal Location of Human Ortholog: 9p24.1
Cellular Component: mitochondrial matrix; mitochondrion; nucleus; plasma membrane
Molecular Function: electron carrier activity; glycine dehydrogenase (decarboxylating) activity; protein homodimerization activity
Biological Process: glycine catabolic process
Disease: Glycine Encephalopathy
Reference #:  P23378 (UniProtKB)
Alt. Names/Synonyms: GCE; GCSP; GLDC; Glycine cleavage system P protein; glycine cleavage system protein P; Glycine decarboxylase; glycine decarboxylase P-protein; glycine dehydrogenase (decarboxylating); Glycine dehydrogenase [decarboxylating], mitochondrial; HYGN1; MGC138198; MGC138200
Gene Symbols: GLDC
Molecular weight: 112,730 Da
Basal Isoelectric point: 6.68  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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