Primary information |
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ThPP ID | Th1045 |
Therapeutic Peptide/Protein Name | Imiglucerase |
Sequence | ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRME view full sequnce in fasta |
Functional Classification | Ia |
Molecular Weight | 55597.4 |
Chemical Formula | C2532H3854N672O711S16 |
Isoelectric Point | 7.41 |
Hydrophobicity | -0.168 |
Melting Point (℃) | N.A. |
Half Life | 0.06-0.173 hours |
Description | 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-glucocerebros. |
Indication/Disease | For the treatment of Gaucher's disease (deficiency in glucocerebrosidase). |
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. |
Toxicity | N.A. |
Metabolism | N.A. |
Absorption | N.A. |
Volume of Distribution | 0.09 to 0.15 L/kg |
Clearance | 14.5 ± 4.0 mL/min/kg |
Categories | Enzyme Replacement Agents |
Patents Number | US5549892 |
Date of Issue | 27/08/93 |
Date of Expiry | 27/08/13 |
Drug Interaction | Zavesca (miglustat) |
Target | Glucocerebroside |
Information of corresponding available drug in the market |
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Brand Name | Cerezyme |
Company | Genzyme |
Brand Discription | Cerezyme(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 for | It is used as an enzyme replacement in people with Type I Gaucher disease. |
Chemical Name | N.A. |
Formulation | Certezyme 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 Appearnce | Sterile, non-pyrogenic, white to off-white lyophilized product |
Route of Administration | Intravenous infusion |
Recommended Dosage | Dosage 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. |
Contraindication | There are no known contraindications to the use of Cerezyme |
Side Effects | Stomach 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 Link | http://www.cerezyme.com/ |
PubMed ID | 19265748, 25501675, 24612151, 24411065, 23430813, 20336596, 24950666, 24630271, 19277123 |
3-D Structure | Th1045 (View) or (Download) |