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Active ingredient: Alglucerase - Brands, Medical Use, Clinical Data

Brands, Medical Use, Clinical Data

Drug Category

  • Enzyme replacement

Dosage Forms

  • Solution (IV Infusion)

Brands / Synonyms

Acid beta-glucosidase; Beta-glucocerebrosidase; Ceredase; Ceredase (Genzyme Corp) ; D-glucosyl-N-acylsphingosine glucohydrolase; Glucosylceramidase precursor; Imiglucerase

Description

Human Beta-glucocerebrosidase or Beta-D-glucosyl-N-acylsphingosine glucohydrolase E.C. 3.2.1.45. 497 residue protein with N-linked carbohydrates, MW=59.3 kD. Alglucerase 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

Indications

For the treatment of Gaucher's disease (deficiency in glucocerebrosidase)

Pharmacology

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 alglucerase 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.

Absorption

Not Available

Toxicity

Not Available

Biotrnasformation / Drug Metabolism

Not Available

Contraindications

Not Available

Drug Interactions

Not Available

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