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Each 400-U vial contains imiglucerase (total amount)* 424 units, mannitol 340 mg, sodium citrates 140 mg (trisodium citrate 104 mg and disodium hydrogen citrate 36 mg), polysorbate 80 NF 1.06 mg.
Citric acid and/or sodium hydroxide may have been added at the time of manufacture to adjust pH.
*This provides a respective withdrawal dose of imiglucerase 400 U.
Cerezyme is an analogue of the human enzyme β-glucocerebrosides, produced by recombinant DNA technology. β-glucocerebrosides (β-D-glucosyl-N-acylsphingosine glucohydrolase, E.C. 3.2.1.45) is a lysomal glycoprotein enzyme which catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide.
Cerezyme is produced by recombinant DNA technology using mammalian cell culture (Chinese hamster ovary). Purified imiglucerase is a monomeric glycoprotein of 497 amino acids, containing 4 N-linked glycosylation sites (Mr=60,430). Imiglucerase differs from placental glucocerebrosidase by 1 amino acid at position 495, where histidine is substituted for arginine. The oligosaccharide chains at the glycosylation sites have been modified to terminate in mannose sugars. The modified carbohydrate structures on imiglucerase are somewhat different from those on placental glucocerebrosidase. These mannose-terminated oligosaccharide chains of imiglucerase are specifically recognized by endocytic carbohydrate receptors on macrophages, the cells that accumulate lipid in Gaucher disease.
An enzyme unit (U) is defined as the amount of enzyme that catalyzes the hydrolysis of 1 micromole of the synthetic substrate ρ-nitrophenyl-β-D-glucopyranoside (pNP-Glc)/min at 37°C.
After reconstitution with sterile water for injection, USP, the imiglucerase concentration is 40 U/mL. Reconstituted solutions have a pH of approximately 6.1.
