Detailed description page of THPdb

This page displays user query in tabular form.

1324 details

Primary information
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 Â± 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
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)