velaglucerase alfa - Medications

Velaglucerase alfa is a hydrolytic lysosomal glucocerebroside-specific enzyme produced by gene activation technology in a human fibroblast cell line. Velaglucerase alfa is a glycoprotein of 497 amino acids and a molecular weight of approximately 63 kDa. Velaglucerase alfa has the same amino acid sequence as the naturally occurring human enzyme, glucocerebrosidase. Velaglucerase alfa contains five potential N-linked glycosylation sites; four of these sites are occupied by glycan chains. Velaglucerase alfa contains predominantly high mannose-type N-linked glycan chains. The high mannose type N-linked glycan chains are specifically recognized and internalized via the mannose receptor present on the surface on macrophages, the cells that accumulate glucocerebroside in Gaucher disease. VPRIV is dosed by units/kg, where one unit of enzyme activity is defined as the quantity of enzyme required to convert one micromole of p-nitrophenyl ß-D-glucopyranoside to p-nitrophenol per minute at 37ºC. VPRIV (velaglucerase alfa) for injection is supplied as a sterile, preservative free, white to off-white lyophilized powder in single-dose vials for intravenous infusion after reconstitution and dilution. Each single-dose vial contains 400 units of velaglucerase alfa, and citric acid, monohydrate (5.04 mg), polysorbate 20 (0.44 mg), sodium citrate, dihydrate (51.76 mg), and sucrose (200 mg). After reconstitution with 4.3 mL Sterile Water for Injection, USP, the final concentration is 100 units/mL with a pH of approximately 6.0.