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Revision as of 19:15, 30 October 2011 by The chemistds (talk | contribs) (No ChemSpider ID for this record)(diff) ← Previous revision | Latest revision (diff) | Newer revision → (diff) Pharmaceutical compound| Clinical data | |
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| AHFS/Drugs.com | Monograph |
| MedlinePlus | a601149 |
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| Routes of administration | Intravenous |
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| Elimination half-life | 3.6-10.4 min |
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IUPAC name
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| Chemical and physical data | |
| Formula | C2532H3854N672O711S16 |
| Molar mass | 55597.4 g/mol (unglycosylated) g·mol |
Imiglucerase is a medication used in the treatment of Gaucher's disease.
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. 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. It is one of the most expensive drugs sold, with an annual cost to U.S. patients of $200,000. 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.
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.
See also
- Other drugs for the treatment of Gaucher's disease
References
- Weinreb NJ (2008). "Imiglucerase and its use for the treatment of Gaucher's disease". Expert Opin Pharmacother. 9 (11): 1987–2000. doi:10.1517/14656566.9.11.1987. PMID 18627336.
{{cite journal}}: Unknown parameter|month=ignored (help) - Starzyk K, Richards S, Yee J, Smith SE, Kingma W (2007). "The long-term international safety experience of imiglucerase therapy for Gaucher disease". Mol. Genet. Metab. 90 (2): 157–63. doi:10.1016/j.ymgme.2006.09.003. PMID 17079176.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - Pentchev; Brady, RO; Blair, HE; Britton, DE; Sorrell, SH; et al. (August 1978). "Gaucher disease: Isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue". Proc. Natl. Acad. Sci. USA. 75 (8): 3970–3973. doi:10.1073/pnas.75.8.3970. PMC 392911. PMID 29293.
{{cite journal}}: Explicit use of et al. in:|author=(help) - "Cerezyme® (imiglucerase for injection) Genzyme product data sheet" (PDF).
- Balancing innovation, access, and profits -- marketing exclusivity for biologics, Alfred B. Engelberg et al., N Engl J Med 361:1917
- "Imiglucerase on Orpha.net: The portal for rare diseases and orphan drugs".
- Erin Ailworth and Robert Weisman (June 17, 2009). "Virus shuts Genzyme plant, holds up drugs for 8,000". The Boston Globe.
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