Imiglucerase 200 U/vial, 400 U/vial Suppliers & Bulk Manufacturers

Available Forms: Injection

Available Strengths: 200 U/vial, 400 U/vial

Reference Brands: Cerezyme (USA/EU/ India), VPRIV (EU)

Category: Immune Disorder

Imiglucerase is a recombinant DNA–derived form of the human enzyme β-glucocerebrosidase, used in enzyme replacement therapy for patients with Type 1 Gaucher disease. It helps break down fatty substances (glucocerebrosides) that accumulate in organs and tissues, thereby reducing symptoms such as anemia, enlarged liver/spleen, and bone pain.

Product Description:

Imiglucerase is a recombinant DNA-produced analogue of the human enzyme β-glucocerebrosidase, formulated for intravenous administration to treat Gaucher disease types 1 and 3. Gaucher disease is a rare lysosomal storage disorder caused by a deficiency of β-glucocerebrosidase, leading to the accumulation of glucocerebroside in macrophages. This accumulation results in enlarged liver and spleen, anemia, thrombocytopenia, and skeletal complications.

Cerezyme is a highly purified freeze-dried formulation of imiglucerase manufactured by Genzyme Corporation. The enzyme is reconstituted prior to intravenous infusion. Each vial contains either 200 or 400 units of imiglucerase, with recombinant enzyme activity of approximately 40 units/mg. The standard dosing regimen is 2.5 units/kg every two weeks, which may be adjusted up to 60 units/kg depending on clinical response. Imiglucerase has been shown to effectively reduce glucocerebroside accumulation, improve organ function, and alleviate clinical symptoms associated with Gaucher disease.

This therapy is well-tolerated and is approved for patients aged 2 years and above. Due to its targeted mechanism of action and clinical efficacy, imiglucerase has been granted orphan drug status in multiple regions, including the United States, Australia, and Japan. It represents a cornerstone in enzyme replacement therapy for managing Gaucher disease.