Imiglucerase

Imiglucerase Uses, Dosage, Side Effects, Food Interaction and all others data.

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.

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

Trade Name Imiglucerase
Availability Prescription only
Generic Imiglucerase
Imiglucerase Other Names Imiglucerasa, Imiglucerase
Related Drugs prednisone, dexamethasone, Decadron, Deltasone, Promacta, miglustat, VPRIV, eliglustat, Cerezyme, Elelyso
Weight 200units, 400units,
Type Intravenous Powder For Injection, Intravenous
Formula C2532H3854N672O711S16
Weight 55597.4 Da
Groups Approved
Therapeutic Class
Manufacturer
Available Country United States,
Last Updated: September 19, 2023 at 7:00 am
Imiglucerase
Imiglucerase

Uses

Imiglucerase is a form of recombinant human beta-glucocerebrosidase enzyme used to replace the deficient endogenous enzyme in the treatment of Gaucher disease.

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

Imiglucerase is also used to associated treatment for these conditions: Gaucher Disease, Type 1, Type 3 Gaucher disease

How Imiglucerase works

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

Food Interaction

No interactions found.

Volume of Distribution

  • 0.09 to 0.15 L/kg

Half Life

3.6-10.4 min

Clearance

  • 14.5 +/- 4.0 mL/min/kg

Innovators Monograph

You find simplified version here Imiglucerase

*** Taking medicines without doctor's advice can cause long-term problems.
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