Imiglucerase resources


DRUG INFO
Imiglucerase

Drug Name: Imiglucerase

Indication: For 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 imiglucerase into Gaucher disease patients leads to elevated serum levels of the enzyme and reduction in the accumulation of glucocerebroside leading to reduced anemia and thrombocytopenia, reduced spleen and liver size, and decreased cachexia

Mechanism Of Action: Imiglucerase catalyzes the hydrolysis of the glycolipid, glucocerebroside, to glucose and ceramide as part of the normal degradation pathway for membrane lipids.

Drug Category: Enzyme replacement; enzymes (44:00.00)

Brand Names/Synonyms: Glucosylceramidase precursor;Beta-glucocerebrosidase;Acid beta-glucosidase;D-glucosyl-N-acylsphingosine glucohydrolase;Alglucerase;Imiglucerase; Cerezyme (Genzyme Corp); Ceredase (Genzyme)

Dosage Forms: POWDER FOR SOLUTION

Absorption: Not Available

Interactions: Not Available



Chemical IUPAC Name: Human Beta-glucocerebrosidase

Chemical Formula: C2532H3854N672O711S16

Half Life: 3.6-10.4 min

Drug Type: Approved Drug

# Accession No: BIOD00026

CAS Registry Number: 143003-46-7



Imiglucerase News
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