Cat# : THP-0045
|Product Name:||Alglucerase, Human GBA|
|Description:||Human Beta-glucocerebrosidase or Beta-D-glucosyl-N-acylsphingosine glucohydrolase E.C. 188.8.131.52. 497 residue protein with N-linked carbohydrates, MW=59.3 kD. The product is prepared by modification of the oligosaccharide chains of human Beta-glucocerebrosidase. The modification alters the sugar residues at the non-reducing ends of the oligosaccharide chains of the glycoprotein so that they are predominantly terminated with mannose residues.|
|Molecular Weight:||55597.4 Da|
|Purity:||>99% by SDS-Page and HPLC analysis|
|Endotoxin Level:||<0.001 EU per 1 μg of the peptide by the LAL method|
|Biological Activity:||80 U/1mL|
|Storage:||Lyophilized protein should be stored at < -20°C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 2-8 °C for 1 week. Aliquots of reconstituted samples are stable at < -20°C for 3 months.|
|Applications:||For the treatment of Gaucher's disease (deficiency in glucocerebrosidase).|
|Examples of Clinical Use:||Gaucher's disease (deficiency in glucocerebrosidase)|
|Pharmacodynamics:||Gaucher disease is characterized by a functional deficiency in Beta-glucocerebrosidase enzymatic activity and the resultant accumulation of lipid glucocerebroside in tissue macrophages which become engorged and are termed Gaucher cells. Gaucher cells are typically found in liver, spleen and bone marrow. This can lead to an enlarged spleen and liver (hepatosplenomegaly). Secondary hematologic sequelae include severe anemia and thrombocytopenia. Injections of the product into Gaucher disease patients leads to elevated serum levels of the enzyme and reduction in the accumulation of glucocerebroside.|
|Mechanism of action:||Alglucerase catalyzes the hydrolysis of the glycolipid, glucocerebroside, to glucose and ceramide as part of the normal degradation pathway for membrane lipids.|
|Affected organisms:||Humans and other mammals|
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