|Other Names||Glycine dehydrogenase (decarboxylating), mitochondrial, Glycine cleavage system P protein, Glycine decarboxylase, Glycine dehydrogenase (aminomethyl-transferring), GLDC, GCSP|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||The 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).|
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Provided below are standard protocols that you may find useful for product applications.
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 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).
Chang, C.Y., et al. Acta Paediatr Taiwan 49(1):35-37(2008)Kanno, J., et al. J. Med. Genet. 44 (3), E69 (2007) Kure, S., et al. Hum. Mutat. 27(4):343-352(2006)
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