Imiglucerase

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 Y
ATC code	A16AB02 (WHO)
DrugBank	DB00053 Y
UNII	Q6U6J48BWY Y
KEGG	D02810 Y
ChEMBL	CHEMBL1201632 N
Chemical data
Formula	C2532H3854N672O711S16
Molecular mass	55597.4 g/mol (unglycosylated)
NY (what is this?)  (verify)

Imiglucerase is a medication used in the treatment of Gaucher's disease.

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. 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. It is one of the most expensive drugs sold, with an annual cost to U.S. patients of $200,000. 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.

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.

See also

• Other drugs for the treatment of Gaucher's disease
  • Afegostat (development terminated)
  • Eliglustat
  • Miglustat