.Pharmacolibrary.Drugs.A_AlimentaryTractAndMetabolism.A16A_OtherAlimentaryTractAndMetabolismProducts.A16AB04_AgalsidaseBeta.AgalsidaseBeta .Pharmacolibrary.Drugs.A_AlimentaryTractAndMetabolism.A16A_OtherAlimentaryTractAndMetabolismProducts.A16AB04_AgalsidaseBeta.AgalsidaseBeta| name: | AgalsidaseBeta |
| ATC code: | A16AB04 | route: | intravenous |
| n-compartments | 2 |
Agalsidase beta is a recombinant form of human alpha-galactosidase A enzyme used as enzyme replacement therapy for patients with Fabry disease, a rare X-linked lysosomal storage disorder. It helps clear globotriaosylceramide (GL-3) accumulation in various tissues and is approved for long-term treatment of Fabry disease in adults and children.
Population PK study in adult male and female patients with Fabry disease receiving 1 mg/kg agalsidase beta as a 2-hour intravenous infusion every 2 weeks.
Keating, GM, & Simpson, D (2007). Agalsidase Beta: a review of its use in the management of Fabry disease. Drugs 67(3) 435–455. DOI:10.2165/00003495-200767030-00007 PUBMED:https://pubmed.ncbi.nlm.nih.gov/17335299