.Pharmacolibrary.Drugs.ATC.A.A16AB04 .Pharmacolibrary.Drugs.ATC.A.A16AB04| name: | AgalsidaseBeta | |
| ATC code: | A16AB04 | |
| route: | intravenous | |
| compartments: | 2 | |
| dosage: | 1 | mg |
| volume of distribution: | 3.0 | L |
| clearance: | 2.6 | L/h |
| other parameters in model implementation | ||
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