What is Alglucerase used for?

Alglucerase is used to treat Gaucher’s disease caused by the lack of a certain enzyme, glucocerebrosidase, in the body. This enzyme is necessary for your body to use fats.

What is Cerezyme used for?

Imiglucerase is used to treat a certain rare genetic problem (Gaucher disease). Imiglucerase replaces a certain natural substance (an enzyme called glucocerebrosidase) that is missing in people with Gaucher disease. This medication improves blood, bone, liver, and spleen problems caused by Gaucher disease.

How do you administer Cerezyme?

Cerezyme is administered as an intravenous infusion 1

1. The recommended dosage of Cerezyme based upon disease severity ranges from 2.5 units/kg 3 times a week to 60 units/kg once every 2 weeks.
2. For patients weighing 18 kg and greater, infuse the diluted Cerezyme solution over 1 to 2 hours.

How much does Cerezyme cost?

The cost for Cerezyme intravenous powder for injection 400 units is around $1,817 for a supply of 1 powder for injection, depending on the pharmacy you visit. Prices are for cash paying customers only and are not valid with insurance plans.

What is the substrate for glucocerebrosidase?

4MU-β-glc is the substrate most commonly used for the glucocerebrosidase enzyme assay, as well as for assaying many other lysosomal hydrolases.

How does Gaucher disease affect metabolism?

Gaucher disease is a rare, inherited metabolic disorder in which deficiency of the enzyme glucocerebrosidase results in the accumulation of harmful quantities of certain fats (lipids), specifically the glycolipid glucocerebroside, throughout the body especially within the bone marrow, spleen and liver.

How is Cerezyme made?

Cerezyme® is produced by recombinant DNA technology using mammalian cell culture (Chinese hamster ovary). Purified imiglucerase is a monomeric glycoprotein of 497 amino acids, containing 4 N-linked glycosylation sites (Mr = 60,430).

What is the substrate for beta-glucosidase?

β-Glucosidase is a predominantly liver enzyme which efficiently hydrolyzes β-D-glucoside and β-D-galactoside. Biochemicals that act as substrates for beta-glucosidase have many applications in biochemical and physiological research.

What is the relationship between lysosomes and Gaucher disease?

Enzymes within lysosomes break down or “digest” nutrients, including certain complex carbohydrates and fats. In Gaucher disease certain sugar (glucose) containing fat, known as glycolipids, abnormally accumulate in the body because of the lack of the enzyme, glucocerebrosidase.

How do replacement enzyme therapy biologics work?

Replacement enzymes for ERT are derived from human, animal, and plant cells that are then genetically modified and processed before being given to the patient. By receiving these enzyme replacements, the body is able to successfully perform the functions inhibited by the deficiency.