Imiglucerasa
Imiglucerasa 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 | Imiglucerasa |
Availability | Prescription only |
Generic | Imiglucerase |
Imiglucerase Other Names | Imiglucerasa, Imiglucerase |
Related Drugs | prednisone, dexamethasone, Decadron, Deltasone, Promacta, miglustat, VPRIV, eliglustat, Cerezyme, Elelyso |
Type | |
Formula | C2532H3854N672O711S16 |
Weight | 55597.4 Da |
Groups | Approved |
Therapeutic Class | |
Manufacturer | |
Available Country | |
Last Updated: | September 19, 2023 at 7:00 am |
Uses
Imiglucerasa 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)
Imiglucerasa is also used to associated treatment for these conditions: Gaucher Disease, Type 1, Type 3 Gaucher disease
How Imiglucerasa works
Imiglucerasa 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
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