Imiglucerase

{{Short description|Pharmaceutical drug}}

{{Drugbox

| Verifiedfields = changed

| verifiedrevid = 458273717

| image =

| alt =

| tradename = Cerezyme

| Drugs.com = {{drugs.com|monograph|imiglucerase}}

| MedlinePlus = a601149

| licence_EU = yes

| DailyMedID = Imiglucerase

| pregnancy_category =

| ATC_prefix = A16

| ATC_suffix = AB02

| ATC_supplemental =

| legal_US = Rx-only

| legal_EU = Rx-only

| legal_EU_comment = {{cite web | title=Cerezyme EPAR | website=European Medicines Agency (EMA) | date=17 September 2018 | url=https://www.ema.europa.eu/en/medicines/human/EPAR/cerezyme | access-date=18 January 2021}}

| routes_of_administration = Intravenous infusion

| bioavailability =

| protein_bound =

| metabolism = probably proteolysis

| elimination_half-life = 3.6–10.4 min

| ChemSpiderID_Ref = {{chemspidercite|changed|chemspider}}

| ChemSpiderID = none

| CAS_number_Ref = {{cascite|changed|CAS}}

| CAS_number = 154248-97-2

| PubChem =

| DrugBank_Ref = {{drugbankcite|correct|drugbank}}

| DrugBank = DB00053

| UNII_Ref = {{fdacite|correct|FDA}}

| UNII = Q6U6J48BWY

| KEGG_Ref = {{keggcite|correct|kegg}}

| KEGG = D02810

| ChEMBL_Ref = {{ebicite|changed|EBI}}

| ChEMBL = 1201632

| IUPAC_name = Human beta-glucocerebrosidase

| C=2532 | H=3854 | N=672 | O=711 | S=16

| molecular_weight = 55597.4

| molecular_weight_comment = (unglycosylated)

}}

Imiglucerase is a medication used in the treatment of Gaucher's disease.{{cite journal | vauthors = Weinreb NJ | title = Imiglucerase and its use for the treatment of Gaucher's disease | journal = Expert Opinion on Pharmacotherapy | volume = 9 | issue = 11 | pages = 1987–2000 | date = August 2008 | pmid = 18627336 | doi = 10.1517/14656566.9.11.1987 | s2cid = 72183308 }}{{cite journal | vauthors = Starzyk K, Richards S, Yee J, Smith SE, Kingma W | title = The long-term international safety experience of imiglucerase therapy for Gaucher disease | journal = Molecular Genetics and Metabolism | volume = 90 | issue = 2 | pages = 157–163 | date = February 2007 | pmid = 17079176 | doi = 10.1016/j.ymgme.2006.09.003 }}

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{{cite book|title=Austria-Codex|at=Cerezyme 400 U-Pulver für ein Konzentrat zur Herstellung einer Infusionslösung|publisher=Österreichischer Apothekerverlag|location=Vienna|year=2018|language=de}} 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,{{cite journal | vauthors = Pentchev PG, Brady RO, Blair HE, Britton DE, Sorrell SH | title = Gaucher disease: isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue | journal = Proceedings of the National Academy of Sciences of the United States of America | volume = 75 | issue = 8 | pages = 3970–3973 | date = August 1978 | pmid = 29293 | pmc = 392911 | doi = 10.1073/pnas.75.8.3970 | doi-access = free | bibcode = 1978PNAS...75.3970P }} meanwhile the enzyme activity produced by recombinant DNA technology is approximately 40 units/mg.{{cite web|url=https://ec.europa.eu/health/documents/community-register/2017/20170602138065/anx_138065_en.pdf|title= Cerezyme Powder for concentrate for solution for infusion, Annex I: Summary of Product Characteristics | work = Sanofi-Aventis Latvia SIA | via = Ec.europa.eu|access-date=26 April 2022}} 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.{{cite web|url=http://www.cerezyme.com/global/pi.pdf|title=Cerezyme (imiglucerase for injection) | work = Genzyme product data sheet|url-status=dead|archive-url=https://web.archive.org/web/20030605012113/http://www.cerezyme.com/global/pi.pdf|archive-date=2003-06-05}} It is one of more expensive medications, with an annual cost of $200,000 per person in the United States.{{cite journal | vauthors = Engelberg AB, Kesselheim AS, Avorn J | title = Balancing innovation, access, and profits--market exclusivity for biologics | journal = The New England Journal of Medicine | volume = 361 | issue = 20 | pages = 1917–1919 | date = November 2009 | pmid = 19828525 | doi = 10.1056/NEJMp0908496 }} Imiglucerase has been granted orphan drug status in the United States, Australia, and Japan.{{Cite web|url=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= | title = Imiglucerase | work = Orphanet |access-date=26 April 2022}}

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.{{cite journal |url= http://www.boston.com/business/healthcare/articles/2009/06/17/genzyme_temporarily_halts_production_on_2_key_drugs/ |title=Virus shuts Genzyme plant, holds up drugs for 8,000|journal=The Boston Globe| vauthors = Ailworth E, Weisman R |date=June 17, 2009}}