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| http://www.biopax.org/relea... | |
| http://www.biopax.org/relea... |
Glycine dehydrogenase [decarboxylating], mitochondrial
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| http://www.biopax.org/relea... |
GCSP_HUMAN
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| http://www.biopax.org/relea... | |
| http://www.biopax.org/relea... |
1.4.4.2,
Glycine cleavage system P protein,
Glycine decarboxylase
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| http://www.biopax.org/relea... |
FUNCTION: 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. CATALYTIC ACTIVITY: Glycine + H-protein-lipoyllysine = H-protein- S-aminomethyldihydrolipoyllysine + CO(2). COFACTOR: Pyridoxal phosphate. SUBUNIT: Homodimer. The glycine cleavage system is composed of four proteins: P, T, L and H. SUBCELLULAR LOCATION: Mitochondrion. DISEASE: Defects in GLDC are a cause of non-ketotic hyperglycinemia (NKH) [MIM:605899]; 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. SIMILARITY: Belongs to the gcvP family. WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/GLDC"; GENE SYNONYMS: GCSP. COPYRIGHT: Protein annotation is derived from the UniProt Consortium (http://www.uniprot.org/). Distributed under the Creative Commons Attribution-NoDerivs License.
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