1. Imiglucerase Protein chemical formula : C2532H3854N672O711S16
Drugbank ID: DB00053
Protein chemical formula : C2532H3854N672O711S16
Protein average weight :
Half-life : min

2. Description Indication Pharmacodynamics
Human Beta-glucocerebrosidase or Beta-D-glucosyl-N-acylsphingosine glucohydrolase E.C 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.
Indication
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

3. Volume of Distribution
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.
Volume of Distribution
0.09 to 0.15 L/kg
Clearance
14.5 +/- 4.0 mL/min/kg
Categories
Enzyme Replacement Agents
Affected Organism
Humans and other mammals

4. Patents Sequence Targets Country Patent Number Approved Expires
United States
Sequence
ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
Targets
Glucocerebroside

5. Brands : Cerezyme Company : Genzyme Description : Cerezyme® (imiglucerase for injection) is an analogue of the human enzyme β-glucocerebrosidase, produced by recombinant DNA technology. β-Glucocerebrosidase (β-D-glucosyl-N-acylsphingosine glucohydrolase, E.C ) is a lysosomal glycoprotein enzyme which catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide. Cerezyme® (imiglucerase) is produced by recombinant DNA technology using mammalian cell culture (Chinese hamster ovary). Purified imiglucerase is a monomeric glycoprotein of 497 amino acids, containing 4 N-linked glycosylation sites (Mr = 60,430). Imiglucerase differs from placental glucocerebrosidase by one amino acid at position 495, where histidine is substituted for arginine. The oligosaccharide chains at the glycosylation sites have been modified to terminate in mannose sugars. The modified carbohydrate structures on imiglucerase are somewhat different from those on placental gluco-cerebrosidase. These mannose-terminated oligosaccharide chains of imiglucerase are specifically recognized by endocytic carbohydrate receptors on macrophages, the cells that accumulate lipid in Gaucher disease. Used For/Prescribed for : It is used as an enzyme replacement in people with Type I Gaucher disease.

6. Formulation : Certezyme is supplied as 200 unit vial and 400 unit vial
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. Form : sterile, non-pyrogenic, white to off-white lyophilized product Route of administration : intravenous infusion 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 : Common Cerezyme side effects may include: 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. Drug Interaction : A total of 1 drugs (2 brand and generic names) are known to interact moderately with Cerezyme (imiglucerase). 1) miglustat 2) Zavesca (miglustat)

7. Genral References
# Pastores GM, Hughes DA: Gaucher Disease "Pubmed":

8. Refrence : http://www.cerezyme.com/ http://www.drugs.com/cerezyme.html