Imiglucerase

Imiglucerase

Systematic (IUPAC) name
Human Beta-glucocerebrosidase
Clinical data
AHFS/Drugs.com	monograph
MedlinePlus	a601149
Licence data	EMA:Link, US FDA:link
Pregnancy cat.	?
Legal status	?
Routes	Intravenous
Pharmacokinetic data
Half-life	3.6-10.4 min
Identifiers
CAS number	143003-46-7 Y
ATC code	A16AB02
DrugBank	BTD00026
UNII	Q6U6J48BWY Y
KEGG	D02810 Y
ChEMBL	CHEMBL1201632 N
Chemical data
Formula	C2532H3854N672O711S16
Mol. mass	55597.4 g/mol (unglycosylated)
N(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

• Other drugs for the treatment of Gaucher's disease
  • Isofagomine tartrate
  • Miglustat

References

1. ^ 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. http://www.expertopin.com/doi/abs/10.1517/14656566.9.11.1987. 
2. ^ 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. http://linkinghub.elsevier.com/retrieve/pii/S1096-7192(06)00297-6. 
3. ^ Pentchev et al.; Brady, RO; Blair, HE; Britton, DE; Sorrell, SH (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. doi:10.1073/pnas.75.8.3970. PMC 392911. PMID 29293. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=392911. 
4. ^ "Cerezyme® (imiglucerase for injection) Genzyme product data sheet". http://www.cerezyme.com/global/pi.pdf. 
5. ^ Balancing innovation, access, and profits -- marketing exclusivity for biologics, Alfred B. Engelberg et al., N Engl J Med 361:1917
6. ^ "Imiglucerase on Orpha.net: The portal for rare diseases and orphan drugs.". http://www.orpha.net/consor/cgi-bin/Drugs_Search.php?lng=EN&data_id=23&Substance=Imiglucerase&search=Drugs_Search_SubstanceTradename&data_type=Product&diseaseType=Drug&Typ=Sub&title=&diseaseGroup=. 
7. ^ Erin Ailworth and Robert Weisman (June 17, 2009). "Virus shuts Genzyme plant, holds up drugs for 8,000". The Boston Globe. http://www.boston.com/business/healthcare/articles/2009/06/17/genzyme_temporarily_halts_production_on_2_key_drugs/. 

This drug article relating to the gastrointestinal system is a stub. You can help Wikipedia by expanding it.v · Categories: Drugs acting on the gastrointestinal system and metabolism Gastrointestinal system drug stubs Wikimedia Foundation. 2010. Игры ⚽ Поможем написать реферат Ring of the Dolphin Blaine Bishop Look at other dictionaries: imiglucerase — noun A medication used in the treatment of Gauchers disease, a recombinant DNA produced analogue of human β glucocerebrosidase …   Wiktionary imiglucerase — im·i·glu·cer·ase (im″ĭ glooґsər ās) an analogue of glucosylceramidase produced by recombinant DNA technology, used as an enzyme replenisher to replace glucosylceramidase (glucocerebrosidase) in type 1 Gaucher disease;… …   Medical dictionary Gaucher disease — ▪ disease       rare inherited metabolic disorder characterized by anemia, mental and neurologic impairment, yellowish pigmentation of the skin, enlargement of the spleen, and bone deterioration resulting in pathological fractures. Gaucher… …   Universalium Имиглуцераза — Химическое соединение ИЮПАК Человеская бета глюкоцереброзидаза Брутто формула C2532 …   Википедия Alglucerase — Systematic (IUPAC) name Human beta glucocerebrosidase Clinical data AHFS/Drugs.com monograph MedlinePlus …   Wikipedia Carnitine — Systematic (IUPAC) name 3 hydroxy 4 (trimethylazaniumyl)butanoate Clinical data AHFS/Drugs.com …   Wikipedia Gaucher's disease — Classification and external resources Acid beta glucosidase ICD 10 E75 …   Wikipedia ATC code A16 — is a division of the Anatomical Therapeutic Chemical Classification System and part of the A Alimentary tract and metabolism section.A16A Other alimentary tract and metabolism products*A16AA Amino acids and derivatives:A16AA01… …   Wikipedia Tetrahydrobiopterin — Systematic (IUPAC) name (6R) 2 Amino 6 [(1R,2S) 1,2 dihydroxypropyl] 5,6,7,8 tetrahydropteridin 4(1H) one Clinical data …   Wikipedia Glucocerebrosidase — NOTOC Glucocerebrosidase (also called glucosylceramidase, β glucosidase, or D glucosyl N acylsphingosine glucohydrolase) is an enzyme (EC number|3.2.1.45) that is needed to cleave, by hydrolysis, the beta glucosidic linkage of the chemical… …   Wikipedia 18+ © Academic, 2000-2024 Contact us: Technical Support, Advertising Dictionaries export, created on PHP, Joomla, Drupal, WordPress, MODx. Mark and share Search through all dictionaries Translate… Search Internet Share the article and excerpts Direct link … Do a right-click on the link above and select “Copy Link”