Chemistry:Imiglucerase
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| Trade names | Cerezyme |
| AHFS/Drugs.com | Monograph |
| MedlinePlus | a601149 |
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| Routes of administration | Intravenous infusion |
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| Pharmacokinetic data | |
| Metabolism | probably proteolysis |
| Elimination half-life | 3.6–10.4 min |
| Identifiers | |
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| Chemical and physical data | |
| Formula | C2532H3854N672O711S16 |
| Molar mass | 55597.4 g·mol−1 (unglycosylated) |
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Imiglucerase is a medication used in the treatment of Gaucher's disease.[2][3]
It is a recombinant DNA-produced analogue of the human enzyme 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 and Type 3[4] 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,[5] meanwhile the enzyme activity produced by recombinant DNA technology is approximately 40 units/mg.[6] 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.[7] It is one of more expensive medications, with an annual cost of $200,000 per person in the United States.[8] Imiglucerase has been granted orphan drug status in the United States, Australia, and Japan.[9]
Cerezyme was one of the drugs manufactured at Genzyme's Allston, Massachusetts plant, for which production was disrupted in 2009 after contamination with Vesivirus 2017.[10]
Side effects
The most common side effect is hypersensitivity, which occurs in about 3% of patients. It is associated with symptoms such as cough, shortness of breath, rashes, itching, and angiooedema. Less common side effects include dizziness, headache, nausea, diarrhea, and reactions at the injection site; they are found in less than 1% of patients.[4]
Interactions
No clinical interaction studies have been conducted.[4] Miglustat appears to increase the clearance of imiglucerase by 70%, resulting in decreased enzyme activity.[11]
See also
- Other drugs for the treatment of Gaucher's disease
- Afegostat (development terminated)
- Eliglustat
- Miglustat
- Velaglucerase alfa
- taliglucerase alfa
References
- ↑ "Cerezyme EPAR". 17 September 2018. https://www.ema.europa.eu/en/medicines/human/EPAR/cerezyme.
- ↑ "Imiglucerase and its use for the treatment of Gaucher's disease". Expert Opinion on Pharmacotherapy 9 (11): 1987–2000. August 2008. doi:10.1517/14656566.9.11.1987. PMID 18627336.
- ↑ "The long-term international safety experience of imiglucerase therapy for Gaucher disease". Molecular Genetics and Metabolism 90 (2): 157–163. February 2007. doi:10.1016/j.ymgme.2006.09.003. PMID 17079176.
- ↑ 4.0 4.1 4.2 (in de) Austria-Codex. Vienna: Österreichischer Apothekerverlag. 2018. Cerezyme 400 U-Pulver für ein Konzentrat zur Herstellung einer Infusionslösung.
- ↑ "Gaucher disease: isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue". Proceedings of the National Academy of Sciences of the United States of America 75 (8): 3970–3973. August 1978. doi:10.1073/pnas.75.8.3970. PMID 29293. Bibcode: 1978PNAS...75.3970P.
- ↑ "Cerezyme Powder for concentrate for solution for infusion, Annex I: Summary of Product Characteristics". Sanofi-Aventis Latvia SIA. https://ec.europa.eu/health/documents/community-register/2017/20170602138065/anx_138065_en.pdf.
- ↑ "Cerezyme (imiglucerase for injection)". Genzyme product data sheet. http://www.cerezyme.com/global/pi.pdf.
- ↑ "Balancing innovation, access, and profits--market exclusivity for biologics". The New England Journal of Medicine 361 (20): 1917–1919. November 2009. doi:10.1056/NEJMp0908496. PMID 19828525.
- ↑ "Imiglucerase". Orphanet. https://www.orpha.net/consor/cgi-bin/Drugs_Search.php?lng=EN&data_id=23&Tradename=Imiglucerase&Typ=Sub&search=Drugs_Search_SubstanceTradename&data_type=Product&title=Imiglucerase&diseaseType=Drug&Drugs_Drugs_Search_ClinicalTrialPhase=&Drugs_Drugs_Search_GeographicType=&Drugs_Drugs_Search_country=&diseaseGroup=.
- ↑ "Virus shuts Genzyme plant, holds up drugs for 8,000". The Boston Globe. June 17, 2009. http://www.boston.com/business/healthcare/articles/2009/06/17/genzyme_temporarily_halts_production_on_2_key_drugs/.
- ↑ "Drug interactions between imiglucerase and miglustat". Drugs.com. https://www.drugs.com/drug-interactions/imiglucerase-with-miglustat-1322-0-1632-0.html?professional=1.
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