NAGLAZYME (galsulfase) is a normal variant form of the polymorphic human enzyme, N-acetylgalactosamine 4-sulfatase that is produced by recombinant DNA technology in a Chinese hamster ovary cell line. N-acetylgalactosamine 4-sulfatas (glycosaminoglycan N-acetylgalactosamine 4-sulfatase EC 3.1.6.12) is a lysosomal hydrolase that catalyzes the cleavage of the sulfate ester from terminal N-acetylgalactosamine 4-sulfate residues of glycosaminoglycans (GAG) chondroitin 4-sulfate and dermatan sulfate.

Galsulfase is a glycoprotein with a molecular weight of approximately 56 kD. The recombinant protein is comprised of 495 amino acids and contains six asparagine-linked glycosylation sites, four of which carry a bis mannose-6-phosphate manose7 oligosaccharide for specific cellular recognition. Post-translational modification of Cys53 produces the catalytic amino acid residue Ca-formylglycine, which is required for enzyme activity and is conserved in all members of the sulfatase enzyme family.

NAGLAZYME has a specific activity of approximately 70 U/mg protein content. One activity unit (U) is defined as the amount of enzyme required to convert 1m mole of 4-methylumbelliferyl sulfate to 4-methylumbellferone and free sulfate per minute at 37° C.

NAGLAZYME, for intravenous infusion, is supplied as a sterile nonpyrogenic, colorless to pale yellow, clear to slightly opalescent solution that must be diluted in 0.9% Sodium Chloride Injection USP, prior to administration. The solution in each vial contains a nominal galsulfase concentration of 1 mg/mL (expressed as protein concentration) at a pH of approximately 5.8. The extractable volume of 5 mL from each vial provides 5 mg galsulfase, 43. 8 mg sodium chloride, 6.20 mg sodium phosphate monobasic monohydrate, 1.34 mg sodium phosphate dibasic heptahydrate, and 0.25 mg polysorbate 80. NAGLAZYME does not contain preservatives; vials are for single use only.

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