Agalsidase beta is a recombinant human alpha-galactosidase indicated to treat Fabry disease, a genetic deficiency in the enzyme leading to buildup of globotriaosylceramide. While patients generally do not experience a clinically significant difference in outcomes between the two drugs, some patients may experience greater benefit with agalsidase beta.
- Mechanism
- Curator reviewed · 3 references
α-galactosidase A is uptaken by cells via the mannose 6 phosphate receptor. Agalsidase beta hydrolyzes globotriaosylceramide and other glycosphingolipids that would normally be hydrolyzed by endogenous α-galactosidase A. Preventing the accumulation of glycosphingolipids prevents or reduces the severity of manifestations of Fabry disease such as renal failure, cardiomyopathy, or cerebrovascular events.
- Primary indication
- Agalsidase beta is indicated in the treatment of Fabry disease.Curator reviewed · 1 structured indication
- Formula / weight
- C2029H3080N544O587S27 · 45351.6 Da
- First approval
- Canada, 2024 · United States, 2008 · European Union, 2016
- Also known as
- Agalsidase beta (genetical recombination)
- Code names
- GZ419828
- Brand names
Resolves to
What you can answer from here — as of July 17, 2026
Which other approved drugs treat the same conditions as Agalsidase beta, and which companies have late-stage candidates in those indications?
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