Imiglucerase

Imiglucerase is a medication used in the treatment of Gaucher's disease.[2][3]

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
IUPAC name
  • Human beta-glucocerebrosidase
CAS Number
DrugBank
ChemSpider
  • none
UNII
KEGG
ChEMBL
Chemical and physical data
FormulaC2532H3854N672O711S16
Molar mass55597.4 g·mol−1 (unglycosylated)
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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, diarrhoea, 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). Retrieved 18 January 2021.
  2. Weinreb NJ (August 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. 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". Mol. Genet. Metab. 90 (2): 157–63. doi:10.1016/j.ymgme.2006.09.003. PMID 17079176.
  4. Austria-Codex (in German). Vienna: Österreichischer Apothekerverlag. 2018. Cerezyme 400 U-Pulver für ein Konzentrat zur Herstellung einer Infusionslösung.
  5. 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. Bibcode:1978PNAS...75.3970P. doi:10.1073/pnas.75.8.3970. PMC 392911. PMID 29293.
  6. "ANNEX I : SUMMARY OF PRODUCT CHARACTERISTICS" (PDF). Ec.europa.eu. Retrieved 26 April 2022.
  7. "Cerezyme (imiglucerase for injection) Genzyme product data sheet" (PDF). Archived from the original (PDF) on 2003-06-05.
  8. Alfred B. Engelberg, Aaron S. Kesselheim, and Jerry Avorn (November 12, 2009). "Perspective: Balancing Innovation, Access, and Profits — Market Exclusivity for Biologics". N Engl J Med. 361 (20): 1917–1919. doi:10.1056/NEJMp0908496. PMID 19828525.{{cite journal}}: CS1 maint: uses authors parameter (link)
  9. "Orphanet: Search for an orphan drug". Orpha.net. Retrieved 26 April 2022.
  10. Erin Ailworth; Robert Weisman (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, accessed 2019-04-11.
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