| Systematic (IUPAC) name | |
|---|---|
| Human Beta-glucocerebrosidase | |
| Clinical data | |
| AHFS/Drugs.com | monograph |
| MedlinePlus | a601149 |
| Licence data | EMA:Link, US FDA:link |
| Pregnancy cat. | ? |
| Legal status | ? |
| Routes | Intravenous |
| Pharmacokinetic data | |
| Half-life | 3.6-10.4 min |
| Identifiers | |
| CAS number | 143003-46-7 |
| ATC code | A16AB02 |
| DrugBank | BTD00026 |
| UNII | Q6U6J48BWY |
| KEGG | D02810 |
| ChEMBL | CHEMBL1201632 |
| Chemical data | |
| Formula | C2532H3854N672O711S16 |
| Mol. mass | 55597.4 g/mol (unglycosylated) |
| (what is this?) (verify) |
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Imiglucerase is a medication used in the treatment of Gaucher's disease.[1][2]
It is a recombinant DNA-produced analogue of human β-glucocerebrosidase. Cerezyme is a freeze-dried medicine containing imiglucerase, manufactured by Genzyme Corporation. It is given intravenously after reconstitution as a treatment for Type 1 Gaucher's disease. It is available in formulations containing 200 or 400 units per vial. The specific activity of highly purified human enzyme is 890,000 units/mg.[3] A typical dose is 2.5U/kg every two weeks, up to a maximum of 60 U/kg once every two weeks, and safety has been established from ages 2 and up.[4] It is one of the most expensive drugs sold, with an annual cost to U.S. patients of $200,000.[5] Due to the low profitability (and high expense) of developing medications for rare conditions, imiglucerase has been granted orphan drug status in the USA, Australia, and Japan.[6]
Cerezyme was one of the drugs manufactured at Genzyme's Allston, Massachusetts plant, for which production was disrupted in 2009 after contamination with Vesivirus 2117.[7]
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