Agalsidase Beta 35 mg Suppliers & Bulk Manufacturers

Available Forms: Injection

Available Strengths: 35 mg

Reference Brands: Fabrazyme (USA/EU)

Category: Orphan Drugs

Agalsidase beta 35 mg powder for IV infusion, recombinant alpha-galactosidase A enzyme replacement for Fabry disease. For rare disease centres, hospital pharmacies and importers with a 2-8°C cold chain.

Product Description:

Agalsidase beta is a recombinant form of human alpha-galactosidase A, the lysosomal enzyme that is missing or deficient in Fabry disease. Made in Chinese hamster ovary cells, it enters cells through mannose-6-phosphate receptors and breaks down globotriaosylceramide (Gb3), the lipid that otherwise builds up in kidney, heart, blood vessels and nerves. It is a freeze-dried powder in 35 mg vials, with a 5 mg vial also made, reconstituted with water for injections and diluted in sodium chloride 0.9% for intravenous infusion.

It is enzyme replacement therapy for confirmed Fabry disease, given at 1 mg/kg every two weeks. Infusions start slowly and may be sped up once tolerated. Infusion-associated reactions such as fever, chills and rigors are common, especially early on and in patients who develop IgG antibodies; slower rates and premedication help. Anaphylaxis is possible, so resuscitation equipment should be at hand. Agalsidase alfa uses a different dose, and oral migalastat suits only amenable mutations, so any switch is a specialist decision.

Sourcing notes. This biological medicine is kept at 2-8°C and never frozen, so ask for temperature records with every delivery. Monthly vial need depends on each patient's weight, which makes forecasting with the treating centre essential. Registration exists in many markets but not all; where it is missing, named-patient supply is the usual route. Request the batch CoA, batch release certificate, GMP certificate and CoPP, and confirm remaining shelf life on arrival. It is prescription-only. Specify vial size, as 35 mg and 5 mg vials are combined to fit the dose.