Lumizyme

DRUG DESCRIPTION

LUMIZYME (alglucosidase alfa) consists of the human enzyme acid α-glucosidase
(GAA), encoded by the most predominant of nine observed haplotypes of this gene.
LUMIZYME (alglucosidase alfa) is produced by recombinant DNA technology in a Chinese hamster ovary
cell line. The LUMIZYME (alglucosidase alfa) manufacturing process differs from that for MYOZYME,
resulting in differences in some product attributes. Alglucosidase alfa degrades
glycogen by catalyzing the hydrolysis of α-1,4- and α-1,6 glycosidic
linkages of lysosomal glycogen.

Alglucosidase alfa is a glycoprotein with a calculated mass of 99,377 daltons
for the polypeptide chain, and a total mass of approximately 109,000 daltons,
including carbohydrates. Alglucosidase alfa has a specific activity of 3 to
5 Units/mg (one unit is defined as that amount of activity that results in the
hydrolysis of 1 micromole of synthetic substrate per minute under specified
assay conditions). LUMIZYME (alglucosidase alfa) is intended for intravenous infusion. It is supplied
as a sterile, nonpyrogenic, white to off-white, lyophilized cake or powder for
reconstitution with 10.3 mL Sterile Water for Injection, USP. Each 50 mg vial
contains 52.5 mg alglucosidase alfa, 210 mg mannitol, 0.5 mg polysorbate 80,
9.9 mg sodium phosphate dibasic heptahydrate, 31.2 mg sodium phosphate monobasic
monohydrate. Following reconstitution as directed, each vial contains 10.5 mL
reconstituted solution and a total extractable volume of 10 mL at 5 mg/mL alglucosidase
alfa. LUMIZYME (alglucosidase alfa) does not contain preservatives; each vial is for single use only.