NP_000161.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
112,730 Da
NCBI Official Full Name
glycine dehydrogenase (decarboxylating), mitochondrial
NCBI Official Synonym Full Names
glycine decarboxylase
NCBI Protein Information
glycine dehydrogenase (decarboxylating), mitochondrial
UniProt Protein Name
Glycine dehydrogenase (decarboxylating), mitochondrial
UniProt Synonym Protein Names
Glycine cleavage system P protein; Glycine decarboxylase
NCBI Summary for GLDC
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). The protein encoded by this gene is the P protein, which binds to glycine and enables the methylamine group from glycine to be transferred to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH).[provided by RefSeq, Jan 2010]
UniProt Comments for GLDC
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.
Protein type: Amino Acid Metabolism - glycine, serine and threonine; EC 1.4.4.2; Oxidoreductase
Chromosomal Location of Human Ortholog: 9p24.1
Cellular Component: glycine cleavage complex; mitochondrial matrix; mitochondrion; nucleus; plasma membrane
Molecular Function: electron transfer activity; enzyme binding; glycine binding; glycine dehydrogenase (decarboxylating) activity; lyase activity; protein homodimerization activity; pyridoxal phosphate binding
Biological Process: glycine catabolic process; glycine decarboxylation via glycine cleavage system
Disease: Glycine Encephalopathy
Research Articles on GLDC
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Products associated with anti-GLDC antibody
Pathways associated with anti-GLDC antibody
Diseases associated with anti-GLDC antibody
Organs/Tissues associated with anti-GLDC antibody
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