Products
Imiglucerase is commercially available as a powder for the preparation of an infusion solution (Cerezyme). It has been approved in many countries since 1999.
Structure and properties
Imiglucerase is an enzyme produced by biotechnological methods. The glycoprotein consists of 497 amino acids. The sequence differs in one amino acid from the natural acid beta-glucocerebrosidase. Thanks to an altered glycosylation with mannose, the enzyme is incorporated into macrophages.
Effects
Imiglucerase (ATC A16AB02) is an analog of the enzyme beta-glucocerebrosidase. This enzyme hydrolyzes glucocerebroside into glucose and ceramide. Gaucher disease is characterized by a deficiency of this lysosomal enzyme. This leads to accumulation of glucocerebroside in cells, mainly in macrophages.
Indications
For long-term enzyme replacement therapy in patients with Gaucher disease (type 1 or type 3).
Dosage
According to the SmPC. The drug is administered as an intravenous infusion. The dosing interval is usually two weeks.
Contraindications
Imiglucerase is contraindicated in cases of hypersensitivity. Refer to the drug label for complete precautions.
Interactions
There are no known drug-drug interactions.
Adverse effects
The most common possible adverse effects include dyspnea (shortness of breath), cough, and hypersensitivity reactions.