Imiglucerase

Imiglucerase
Clinical data
Trade names	Cerezyme
AHFS/Drugs.com	Monograph
MedlinePlus	a601149
License data	EU EMA: by INN; US DailyMed: Imiglucerase;
Routes of; administration	Intravenous infusion
ATC code	A16AB02 (WHO) ;
Legal status
Legal status	US: ℞-only; EU: Rx-only;
Pharmacokinetic data
Metabolism	probably proteolysis
Elimination half-life	3.6–10.4 min
Identifiers
	IUPAC name Human beta-glucocerebrosidase;
CAS Number	154248-97-2 ;
DrugBank	DB00053 ;
ChemSpider	none;
UNII	Q6U6J48BWY;
KEGG	D02810 ;
ChEMBL	ChEMBL1201632 ;
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]

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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]

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]

No clinical interaction studies have been conducted.^[4] Miglustat appears to increase the clearance of imiglucerase by 70%, resulting in decreased enzyme activity.^[11]

Other drugs for the treatment of Gaucher's disease
- Afegostat (development terminated)
- Eliglustat
- Miglustat
- Velaglucerase alfa
- taliglucerase alfa

[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]
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.

[1] [1]
"Cerezyme EPAR". European Medicines Agency (EMA). 17 September 2018. Retrieved 18 January 2021.

[pmid18627336-2] [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.

[pmid17079176-3] [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.

[AC-4] [4]
Austria-Codex (in German). Vienna: Österreichischer Apothekerverlag. 2018. Cerezyme 400 U-Pulver für ein Konzentrat zur Herstellung einer Infusionslösung.

[5] [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] [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] [7]
"Cerezyme (imiglucerase for injection)" (PDF). Genzyme product data sheet. Archived from the original (PDF) on 2003-06-05.

[Engelberg-8] [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] [9]
"Imiglucerase". Orphanet. Retrieved 26 April 2022.

[10] [10]
Ailworth E, Weisman R (June 17, 2009). "Virus shuts Genzyme plant, holds up drugs for 8,000". The Boston Globe.

[11] [11]
"Drug interactions between imiglucerase and miglustat". Drugs.com. Retrieved 11 April 2019.

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Imiglucerase

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Side effects

Interactions

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References