Imiglucerase
Identification
- Summary
Imiglucerase is a form of recombinant human beta-glucocerebrosidase enzyme used to replace the deficient endogenous enzyme in the treatment of Gaucher disease.
- Brand Names
- Cerezyme
- Generic Name
- Imiglucerase
- DrugBank Accession Number
- DB00053
- Background
Human Beta-glucocerebrosidase or Beta-D-glucosyl-N-acylsphingosine glucohydrolase E.C. 3.2.1.45. 497 residue protein with N-linked carbohydrates, MW=59.3 kD. Alglucerase is prepared by modification of the oligosaccharide chains of human Beta-glucocerebrosidase. The modification alters the sugar residues at the non-reducing ends of the oligosaccharide chains of the glycoprotein so that they are predominantly terminated with mannose residues.
- Type
- Biotech
- Groups
- Approved
- Biologic Classification
- Protein Based Therapies
Recombinant Enzymes - Protein Structure
- Protein Chemical Formula
- C2532H3854N672O711S16
- Protein Average Weight
- 55597.4 Da
- Sequences
>DB00053 sequence ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANH TGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIR VPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWT SPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGL LSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPE AAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRG MQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHL GHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFL ETISPGYSIHTYLWRRQ
Download FASTA Format- Synonyms
- Imiglucerasa
- Imiglucerase
- External IDs
- ISU-302
Pharmacology
- Indication
For the treatment of Gaucher's disease (deficiency in glucocerebrosidase)
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Indication Type Indication Combined Product Details Approval Level Age Group Patient Characteristics Dose Form Management of Type 1 gaucher disease •••••••••••• ••••••••••• •••••• ••••••••• ••••••••• Management of Type 3 gaucher disease •••••••••••• ••••••••••• •••••• ••••••••• •• •••••••••••• •••••••• ••••••••• - Contraindications & Blackbox Warnings
- Prevent Adverse Drug Events TodayTap into our Clinical API for life-saving information on contraindications & blackbox warnings, population restrictions, harmful risks, & more.Avoid life-threatening adverse drug events with our Clinical API
- Pharmacodynamics
Gaucher disease is characterized by a functional deficiency in Beta-glucocerebrosidase enzymatic activity and the resultant accumulation of lipid glucocerebroside in tissue macrophages which become engorged and are termed Gaucher cells. Gaucher cells are typically found in liver, spleen and bone marrow. This can lead to an enlarged spleen and liver (hepatosplenomegaly). Secondary hematologic sequelae include severe anemia and thrombocytopenia. Injections of imiglucerase into Gaucher disease patients leads to elevated serum levels of the enzyme and reduction in the accumulation of glucocerebroside leading to reduced anemia and thrombocytopenia, reduced spleen and liver size, and decreased cachexia
- Mechanism of action
Imiglucerase catalyzes the hydrolysis of the glycolipid, glucocerebroside, to glucose and ceramide as part of the normal degradation pathway for membrane lipids.
Target Actions Organism AGlucocerebroside other/unknownHumans - Absorption
Not Available
- Volume of distribution
- 0.09 to 0.15 L/kg
- Protein binding
Not Available
- Metabolism
- Not Available
- Route of elimination
Not Available
- Half-life
3.6-10.4 min
- Clearance
- 14.5 +/- 4.0 mL/min/kg
- Adverse Effects
- Improve decision support & research outcomesWith structured adverse effects data, including: blackbox warnings, adverse reactions, warning & precautions, & incidence rates. View sample adverse effects data in our new Data Library!Improve decision support & research outcomes with our structured adverse effects data.
- Toxicity
Not Available
- Pathways
- Not Available
- Pharmacogenomic Effects/ADRs Browse all" title="About SNP Mediated Effects/ADRs" id="snp-actions-info" class="drug-info-popup" href="javascript:void(0);">
- Not Available
Interactions
- Drug Interactions Learn More" title="About Drug Interactions" id="structured-interactions-info" class="drug-info-popup" href="javascript:void(0);">
- This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.No interactions found.
- Food Interactions
- No interactions found.
Products
- Drug product information from 10+ global regionsOur datasets provide approved product information including:dosage, form, labeller, route of administration, and marketing period.Access drug product information from over 10 global regions.
- Brand Name Prescription Products
Name Dosage Strength Route Labeller Marketing Start Marketing End Region Image Cerezyme Injection, powder, for solution 400 U Intravenous Sanofi B.V. 2020-12-16 Not applicable EU Cerezyme Injection, powder, lyophilized, for solution 40 U/1mL Intravenous Genzyme Corporation 1994-05-23 Not applicable US Cerezyme Injection, powder, for solution 400 U Intravenous Sanofi B.V. 2020-12-16 Not applicable EU Cerezyme Powder, for solution 200 unit / vial Intravenous Sanofi Genzyme, a Division of Sanofi Aventis Canada Inc 1997-05-01 2016-08-04 Canada Cerezyme Injection, powder, lyophilized, for solution 40 U/1mL Intravenous Genzyme Corporation 1994-05-23 2012-05-31 US
Categories
- ATC Codes
- A16AB02 — Imiglucerase
- Drug Categories
- Chemical TaxonomyProvided by Classyfire
- Description
- Not Available
- Kingdom
- Organic Compounds
- Super Class
- Organic Acids
- Class
- Carboxylic Acids and Derivatives
- Sub Class
- Amino Acids, Peptides, and Analogues
- Direct Parent
- Peptides
- Alternative Parents
- Not Available
- Substituents
- Not Available
- Molecular Framework
- Not Available
- External Descriptors
- Not Available
- Affected organisms
- Humans and other mammals
Chemical Identifiers
- UNII
- Q6U6J48BWY
- CAS number
- 154248-97-2
References
- General References
- Pastores GM, Hughes DA: Gaucher Disease . [Article]
- External Links
- UniProt
- P04062
- Genbank
- M16328
- KEGG Drug
- D02810
- PubChem Substance
- 46508744
- 84959
- ChEMBL
- CHEMBL1201632
- PharmGKB
- PA164742934
- RxList
- RxList Drug Page
- Drugs.com
- Drugs.com Drug Page
- Wikipedia
- Imiglucerase
- FDA label
- Download (111 KB)
Clinical Trials
- Clinical Trials Learn More" title="About Clinical Trials" id="clinical-trials-info" class="drug-info-popup" href="javascript:void(0);">
Phase Status Purpose Conditions Count 4 Completed Treatment Cerebroside Lipidosis Syndrome / Clucocerebrosidase Deficiency Disease / Gaucher Disease, Non-Neuronopathic Form / Gaucher Disease, Type 1 / Glucosylceramide Beta-Glucosidase Deficiency Disease 1 4 Completed Treatment Cerebroside Lipidosis Syndrome / Gaucher Disease / Gaucher Disease, Non-Neuronopathic Form / Gaucher Disease, Type 1 / Glucosylceramide Beta-Glucosidase Deficiency Disease 1 4 Completed Treatment Gaucher Disease 1 3 Active Not Recruiting Treatment Gaucher Disease, Type 1 / Gaucher Disease, Type III 1 3 Completed Treatment Gaucher Disease, Type 1 3
Pharmacoeconomics
- Manufacturers
- Genzyme corp
- Packagers
- Genzyme Inc.
- Dosage Forms
Form Route Strength Injection, powder, for solution Intravenous Injection, powder, for solution Intravenous 400 U Injection, powder, lyophilized, for solution Intravenous 200 IU Injection, powder, lyophilized, for solution Intravenous 40 U/1mL Powder Intravenous 200 U Powder, for solution Intravenous 200 unit / vial Powder, for solution Intravenous 400 unit / vial Powder, for solution Intravenous; Parenteral 400 U Injection Intravenous 200 U Injection Intravenous 400 U Injection Intravenous Injection, powder, for solution Intravenous 424 U Powder Intravenous 400 unit/1vial - Prices
Unit description Cost Unit Cerezyme 400 unit vial 1903.2USD vial Cerezyme 200 unit vial 951.6USD vial Ceredase 80 unit/ml vial 380.64USD ml DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.- Patents
Patent Number Pediatric Extension Approved Expires (estimated) Region US5549892 No 1996-08-27 2013-08-27 US
Properties
- State
- Liquid
- Experimental Properties
Property Value Source hydrophobicity -0.168 Not Available isoelectric point 7.41 Not Available
Targets
![](https://go.drugbank.com/assets/locked/DrugTargets2-682eb846d210ae4bf33ca03febccfb57bb1c61318e699b729e5df4dfab2c5b67.png)
References
- Pastores GM, Hughes DA: Gaucher Disease . [Article]
Drug created at June 13, 2005 13:24 / Updated at February 20, 2024 23:55