Cerezyme Description

Cerezyme is an analogue of the human enzyme β-glucocerebrosidase, produced by recombinant deoxyribonucleic acid (DNA) technology. β-glucocerebrosidase (β-D-glucosyl-N-acylsphingosine glucohydrolase, E.C.3.2.1.45) is a lysosomal 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.
The quantitative composition of the lyophilized drug is provided in Table 1. (See Table 1.)

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An enzyme unit (U) is defined as the amount of enzyme that catalyzes the hydrolysis of 1 micromole of the synthetic substrate para-nitrophenyl-β-D-glucopyranoside (pNP-Glc)/min at 37°C. After reconstitution with sterile water for injection, the imiglucerase concentration is 40 U/mL (see Dosage & Administration). Reconstituted solutions have a pH of approximately 6.1.