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
Db00053
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)

Pharmacology
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Associated Conditions
Contraindications & Blackbox Warnings
Contraindications
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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.

TargetActionsOrganism
AGlucocerebroside
other/unknown
Humans
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
Adverseeffects
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Toxicity

Not Available

Pathways
Not Available
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
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

Products2
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Brand Name Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing EndRegionImage
CerezymePowder, for solution400 unit / vialIntravenousSanofi Genzyme, a Division of Sanofi Aventis Canada Inc2000-06-14Not applicableCanada flag
CerezymeInjection, powder, for solution400 UIntravenousGenzyme Europe Bv2020-12-16Not applicableEU flag
CerezymeInjection, powder, lyophilized, for solution40 U/1mLIntravenousGenzyme Corporation1994-05-23Not applicableUS flag
CerezymeInjection, powder, for solution400 UIntravenousGenzyme Europe Bv2020-12-16Not applicableEU flag
CerezymePowder, for solution200 unit / vialIntravenousSanofi Genzyme, a Division of Sanofi Aventis Canada Inc1997-05-012016-08-04Canada flag
CerezymeInjection, powder, lyophilized, for solution40 U/1mLIntravenousGenzyme Corporation1994-05-232012-05-31US flag
CerezymeInjection, powder, for solution400 UIntravenousGenzyme Europe Bv2020-12-16Not applicableEU flag

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
  1. Pastores GM, Hughes DA: Gaucher Disease . [Article]
UniProt
P04062
Genbank
M16328
KEGG Drug
D02810
PubChem Substance
46508744
RxNav
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
PhaseStatusPurposeConditionsCount
4CompletedTreatmentCerebroside Lipidosis Syndrome / Clucocerebrosidase Deficiency Disease / Gaucher Disease, Non-Neuronopathic Form / Gaucher Disease, Type 1 / Glucosylceramide Beta-Glucosidase Deficiency Disease1
4CompletedTreatmentCerebroside Lipidosis Syndrome / Gaucher Disease, Non-Neuronopathic Form / Gaucher Disease, Type 1 / Glucocerebrosidase Deficiency Disease / Glucosylceramide Beta-Glucosidase Deficiency Disease1
4RecruitingTreatmentGaucher's Disease1
3CompletedTreatmentGaucher Disease, Type 13
3RecruitingTreatmentGaucher Disease, Type 1 / Gaucher Disease, Type III1
3WithdrawnNot AvailableGaucher's Disease1
2Active Not RecruitingTreatmentGaucher Disease, Type 1 / Gaucher Disease, Type 31
2Unknown StatusTreatmentGaucher's Disease1
1CompletedNot AvailableGaucher's Disease1
1CompletedTreatmentGaucher's Disease1

Pharmacoeconomics

Manufacturers
  • Genzyme corp
Packagers
  • Genzyme Inc.
Dosage Forms
FormRouteStrength
Injection, powder, for solutionIntravenous
Injection, powder, for solutionIntravenous400 U
Injection, powder, lyophilized, for solutionIntravenous200 IU
Injection, powder, lyophilized, for solutionIntravenous40 U/1mL
PowderIntravenous200 U
Powder, for solutionIntravenous200 unit / vial
Powder, for solutionIntravenous400 unit / vial
Powder, for solutionIntravenous; Parenteral400 U
InjectionIntravenous
Injection, powder, for solutionIntravenous424 U
PowderIntravenous400 unit/1vial
Prices
Unit descriptionCostUnit
Cerezyme 400 unit vial1903.2USD vial
Cerezyme 200 unit vial951.6USD vial
Ceredase 80 unit/ml vial380.64USD ml
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
Patents
Patent NumberPediatric ExtensionApprovedExpires (estimated)Region
US5549892No1996-08-272013-08-27US flag

Properties

State
Liquid
Experimental Properties
PropertyValueSource
hydrophobicity-0.168Not Available
isoelectric point7.41Not Available

Drug created on June 13, 2005 13:24 / Updated on October 24, 2021 16:00