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| Primary information |
|---|
| ID | 10324 |
| Therapeutic ID | Th1045 |
| Protein Name | Imiglucerase |
| Sequence | >Th1045_Imiglucerase
ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
|
| Molecular Weight | 55597.4 |
| Chemical Formula | C2532H3854N672O711S16 |
| Isoelectric Point | 7.41 |
| Hydrophobicity | -0.168 |
| Melting point | NA |
| 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 | NA |
| Metabolism | NA |
| Absorption | NA |
| 0.09 to 0.15 L/kg |
| Clearance | 14.5 ± 4.0 mL/min/kg |
| Categories | Alimentary Tract and Metabolism, Enzyme Replacement Therapy, Enzymes, Enzymes and Coenzymes, Gaucher Disease, Glucosidases, Glycoside Hydrolases, Hydrolases, Hydrolytic Lysosomal Glucocerebroside-specific Enzyme |
| Patents Number | US5549892 |
| Date of Issue | 27-Aug-1996 |
| Date of Expiry | 27-Aug-2013 |
| Drug Interaction | Zavesca (miglustat) |
| Target | Glucocerebroside |
| Brand Name | Cerezyme |
| Company | Genzyme |
| Brand Description | Genzyme |
| Prescribed For | It is used as an enzyme replacement in people with Type I Gaucher disease. |
| Chemical Name | NA |
| 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. |
| Physical Appearance | 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 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |