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| Primary information |
|---|
| ID | 10434 |
| Therapeutic ID | Th1074 |
| Protein Name | Alglucerase |
| Sequence | >Th1074_Alglucerase
ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
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| Molecular Weight | 55597.4 |
| Chemical Formula | C2532H3854N672O711S16 |
| Isoelectric Point | 7.41 |
| Hydrophobicity | -0.168 |
| Melting point | NA |
| Half-life | 3.6-10.4 min |
| Description | Human Beta-glucocerebrosidase or Beta-D-glucosyl-N-acylsphingosine glucohydrolase E.C. 3.2.1.45. 497 residue protein with N-linked carbohydrates. 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 |
| 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 alglucerase into Gaucher disease patients leads to elevated serum levels of the enzyme and reduction in the accumulation of glucocerebroside |
| Mechanism of Action | Alglucerase 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 |
| 49.4 to 282.1 mL/kg |
| Clearance | NA |
| Categories | Alimentary Tract and Metabolism,Enzyme Replacement Therapy,Enzymes,Enzymes and Coenzymes,Gaucher Disease,Glucosidases,Glycoside Hydrolases,Hydrolases,Hydrolytic Lysosomal Glucocerebroside-specific Enzyme,Recombinant Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Glucocerebroside |
| Brand Name | Ceredase |
| Company | Genzyme Corporation |
| Brand Description | Genzyme Corporation |
| Prescribed For | Ceredase (alglucerase injection) is indicated for use as long-term enzyme replacement therapy for children, adolescents and adult patients with a confirmed diagnosis of Type I Gaucher disease who exhibit signs and symptoms that are severe enough to result in moderate-to-severe anemia, thrombocytopenia with bleeding tendency, bone disease, significant hepatomegaly or splenomegaly. |
| Chemical Name | NA |
| Formulation | Ceredase citrate is supplied as buffered solution (53 mM citrate, 143 mM sodium) containing 1% albumin human USP. The enzyme is supplied in one concentration, 400 units per bottle (80 units/mL) with a fill volume of 5 mL per bottle. An enzyme unit (U) is defined as the amount of enzyme required to hydrolyze in one minute one micromole of the synthetic substrate, p-nitrophenyl-β-D-glucopyranoside. |
| Physical Appearance | Ceredase (alglucerase injection) is supplied as a clear sterile non-pyrogenic solution of alglucerase in a citrate buffered solution |
| Route of Administration | Intravenous infusion |
| Recommended Dosage | Ceredase (alglucerase injection) is administered by Intravenous infusion over 1-2 hours. Dosage should be individualized for each patient. Initial dosage may be as little as 2.5 units/kg of body weight 3 times a week up to as much as 60 units/kg administered as frequently as once a week or as infrequently as every 4 weeks. 60 units/kg every 2 weeks is the dose for which the most data are available. Disease severity may dictate that drug be initiated with relatively high doses or relatively frequent administration. After patient response is well-established, a reduction in dosage may be attempted for maintenance therapy. Progressive reductions can be made at intervals of 3-6 months while carefully monitoring response parameters. |
| Contraindication | There are no known contraindications to the use of Ceredase (alglucerase injection). |
| Side Effects | Events were related to the route of administration including discomfort,pruritus, burning and swelling or sterile abscess at the site of venipuncture. The remaining experiences consisted of slight fever, chills, abdominaldiscomfort, nausea or vomiting. Additional adverse symptoms which have been reported include: fatigue, vasomotor irritability or hot flash, weakness, headache, light headedness, dysosmia, oral ulcerations, backache and transient peripheral edema, and diarrhea. Menstrual abnormalities and false positive pregnancy tests |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |