Imiglucerase

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Imiglucerase
Systematic (IUPAC) name
Human Beta-glucocerebrosidase
Clinical data
AHFS/Drugs.com monograph
MedlinePlus a601149
Licence data EMA:Link, US FDA:link
Routes of
administration		 Intravenous
Pharmacokinetic data
Biological half-life 3.6-10.4 min
Identifiers
CAS Number 143003-46-7 YesY
ATC code A16AB02 (WHO)
DrugBank DB00053 YesY
UNII Q6U6J48BWY YesY
KEGG D02810 YesY
ChEMBL CHEMBL1201632 N
Chemical data
Formula C2532H3854N672O711S16
Molecular mass 55597.4 g/mol (unglycosylated)
 NYesY (what is this?)  (verify)

Imiglucerase is a medication used in the treatment of Gaucher's disease.[1][2]

It is a recombinant DNA-produced analogue of human 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 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.[3] 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.[4] It is one of the most expensive drugs sold, with an annual cost to U.S. patients of $200,000.[5] Due to the low profitability (and high expense) of developing medications for rare conditions, imiglucerase has been granted orphan drug status in the USA, Australia, and Japan.[6]

Cerezyme was one of the drugs manufactured at Genzyme's Allston, Massachusetts plant, for which production was disrupted in 2009 after contamination with Vesivirus 2117.[7]

See also

References

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  5. ↑ Balancing innovation, access, and profits -- marketing exclusivity for biologics, Alfred B. Engelberg et al., N Engl J Med 361:1917
  6. ↑ Lua error in package.lua at line 80: module 'strict' not found.
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