==== Reference: Usmani SS, Bedi G, Samuel JS, Singh S, Kalra S, Kumar P, et al. (2017) THPdb: Database of FDA-approved peptide and protein therapeutics. PLoS ONE 12(7) e0181748.====

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1324 details
Primary information
ThPP IDTh1045
Therapeutic Peptide/Protein NameImiglucerase
SequenceARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRME view full sequnce in fasta
Functional ClassificationIa
Molecular Weight55597.4
Chemical FormulaC2532H3854N672O711S16
Isoelectric Point7.41
Hydrophobicity-0.168
Melting Point (℃)N.A.
Half Life0.06-0.173 hours
DescriptionHuman 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-glucocerebros.
Indication/DiseaseFor the treatment of Gaucher's disease (deficiency in glucocerebrosidase).
PharmacodynamicsGaucher 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 ActionImiglucerase catalyzes the hydrolysis of the glycolipid, glucocerebroside, to glucose and ceramide as part of the normal degradation pathway for membrane lipids.
ToxicityN.A.
MetabolismN.A.
AbsorptionN.A.
Volume of Distribution0.09 to 0.15 L/kg
Clearance14.5 ± 4.0 mL/min/kg
CategoriesEnzyme Replacement Agents
Patents NumberUS5549892
Date of Issue27/08/93
Date of Expiry27/08/13
Drug InteractionZavesca (miglustat)
TargetGlucocerebroside
Information of corresponding available drug in the market
Brand NameCerezyme
CompanyGenzyme
Brand DiscriptionCerezyme(imiglucerase for injection) is an analogue of the human enzyme _-glucocerebrosidase, produced by recombinant DNA technology. _-Glucocerebrosidase (_-D-glucosyl-N-acylsphingosine glucohydrolase, E.C. 3.2.1.45) is a lysosomal glycoprotein enzyme wh
Prescribed forIt is used as an enzyme replacement in people with Type I Gaucher disease.
Chemical NameN.A.
FormulationCertezyme is supplied as 200 unit vial and 400 unit vial. The constituent of 200 unit vial are 212 units of Imiglucerase, 170 mg mannitol , 70 mg sodium citrates, 52 mg trisodium citrate, 18 mg Disodium hydrogen citrate and 0.53 mg polysorbate.
Physcial AppearnceSterile, non-pyrogenic, white to off-white lyophilized product
Route of AdministrationIntravenous infusion
Recommended DosageDosage should be individualized to each patient. Initial dosages range from 2.5 U/kg of body weight 3 times a week to 60 U/kg once every 2 weeks.
ContraindicationThere are no known contraindications to the use of Cerezyme
Side EffectsStomach pain, nausea, vomiting, diarrhea; fast heartbeats; headache, dizziness; back pain; fever, chills, tired feeling; mild rash; or itching, burning, swelling, or other discomfort around the IV needle.
Useful Linkhttp://www.cerezyme.com/
PubMed ID19265748, 25501675, 24612151, 24411065, 23430813, 20336596, 24950666, 24630271, 19277123
3-D StructureTh1045 (View) or (Download)