For treatment of Fabry's disease (alpha-galactosidase A deficiency)
Alpha-galactosidase A catalyzes the hydrolysis of globotriaosylceramide (GL-3) and other a-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose.
45-102 min
* 4.1 +/- 1.2 mL/min/kg [adult patients with Fabry disease,0.3 mg/kg, 1 infusion] * 4.6 +/- 2.2 mL/min/kg [adult patients with Fabry disease, 0.3 mg/kg, 5 infusions] * 2.1 +/- 0.7 mL/min/kg [adult patients with Fabry disease, 1 mg/kg, 1 infusion] * 3.2 +/- 2.6 mL/min/kg [adult patients with Fabry disease, 1 mg/kg, 5 infusions] * 0.8 +/- 0.3 mL/min/kg [adult patients with Fabry disease, 3 mg/kg, 1 infusion] * 0.8 +/- 0.4 mL/min/kg [adult patients with Fabry disease, 3 mg/kg, 5 infusions] * 1.8 +/- 0.8 mL/min/kg [Pediatric Patients with Fabry Disease, 1 mg/kg, 1-3 infusions] * 4.9 +/- 5.6 mL/min/kg [Pediatric Patients with Fabry Disease, 1 mg/kg, 7 infusions] * 2.3 +/- 2.2 mL/min/kg [Pediatric Patients with Fabry Disease, 1 mg/kg, 11 infusions]