Imiglucerase
Clinical data
Trade namesCerezyme
AHFS/Drugs.comMonograph
MedlinePlusa601149
License data
Routes of
administration		Intravenous infusion
ATC code
Legal status
Legal status
Pharmacokinetic data
Metabolismprobably proteolysis
Elimination half-life3.6–10.4 min
Identifiers
CAS Number
DrugBank
ChemSpider
  • none
UNII
KEGG
ChEMBL
Chemical and physical data
FormulaC2532H3854N672O711S16
Molar mass55597.4 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

References

  1. ^ "Cerezyme EPAR". European Medicines Agency (EMA). 17 September 2018. Retrieved 18 January 2021.
  2. ^ Weinreb NJ (August 2008). "Imiglucerase and its use for the treatment of Gaucher's disease". Expert Opinion on Pharmacotherapy. 9 (11): 1987–2000. doi:10.1517/14656566.9.11.1987. PMID 18627336. S2CID 72183308.
  3. ^ Starzyk K, Richards S, Yee J, Smith SE, Kingma W (February 2007). "The long-term international safety experience of imiglucerase therapy for Gaucher disease". Molecular Genetics and Metabolism. 90 (2): 157–163. doi:10.1016/j.ymgme.2006.09.003. PMID 17079176.
  4. ^ a b c Austria-Codex (in German). Vienna: Österreichischer Apothekerverlag. 2018. Cerezyme 400 U-Pulver für ein Konzentrat zur Herstellung einer Infusionslösung.
  5. ^ Pentchev PG, Brady RO, Blair HE, Britton DE, Sorrell SH (August 1978). "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. Bibcode:1978PNAS...75.3970P. doi:10.1073/pnas.75.8.3970. PMC 392911. PMID 29293.
  6. ^ "Cerezyme Powder for concentrate for solution for infusion, Annex I: Summary of Product Characteristics" (PDF). Sanofi-Aventis Latvia SIA. Retrieved 26 April 2022 – via Ec.europa.eu.
  7. ^ "Cerezyme (imiglucerase for injection)" (PDF). Genzyme product data sheet. Archived from the original (PDF) on 2003-06-05.
  8. ^ Engelberg AB, Kesselheim AS, Avorn J (November 2009). "Balancing innovation, access, and profits--market exclusivity for biologics". The New England Journal of Medicine. 361 (20): 1917–1919. doi:10.1056/NEJMp0908496. PMID 19828525.
  9. ^ "Imiglucerase". Orphanet. Retrieved 26 April 2022.
  10. ^ Ailworth E, Weisman R (June 17, 2009). "Virus shuts Genzyme plant, holds up drugs for 8,000". The Boston Globe.
  11. ^ "Drug interactions between imiglucerase and miglustat". Drugs.com. Retrieved 11 April 2019.
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