Product Description:

The glycine cleavage system (GCS), aka the glycine decarboxylase complex, is a series of enzymes (the T-protein, P-protein, L-protein, and H-protein) that are loosely associated and involved in the metabolism of the amino acid glycine. The reactions are reversible and can generate glycine in the reverse direction. Defects in GCS are a cause of nonketotic hyperglycinemia (NKH), which is a rare autosomal recessive disorder of glycine metabolism. Glycine encephalopathy is the second most common disorder of amino acid metabolism after phenylketonuria (PKU). The symptoms are exclusively neurological in nature. Mutations in the GLDC gene account for ~80% of all cases of glycine encephalopathy. The GCS assay is based on the reduction of the tetrazolium salt INT in a NADH-coupled reaction to INT-formazan, which exhibits an absorption maximum at 492 nm (molar extinction coefficient = 18 mM-1cm-1) and allows for sensitive detection of GCS in tissue/cell samples. Assay solution is stable for at least one year if handled properly.

#Glycine Cleavage System​

Kit Components:

GCS Assay Solution: 5 ml, store at -80ºC

10x GCS Substrate: 0.5 ml, store at 4ºC

10x Cell Lysis Solution: 25 ml, store at 4ºC

SDS:

TX-100, DMSO, INT, Hepes

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