Product Information
Registration Status: ActiveMYOZYME (ALGLUCOSIDASE ALFA) 50MG POWDER FOR SOLUTION FOR INFUSION is approved to be sold in Singapore with effective from 2008-08-28. It is marketed by SANOFI-AVENTIS SINGAPORE PTE LTD, with the registration number of SIN13543P.
This product contains Alglucosidase Alfa 52.5mg in the form of INJECTION, POWDER, LYOPHILIZED, FOR SOLUTION. It is approved for INTRAVENOUS use.
This product is manufactured by Genzyme Ireland LTD in IRELAND.
It is a Prescription Only Medicine that can only be obtained from a doctor or a dentist, or a pharmacist with a prescription from a Singapore-registered doctor or dentist.
Description
Aglucosidase alfa consists of the human enzyme acid alpha-glucosidase (GAA) which is essential for the degradation of glygogen to glucose in lysosomes. It is encoded by the most predominant of nine observed haplotypes of this gene. Aglucosidase alfa is produced by recombinant DNA technology in a Chinese hamster ovary cell line. Alglucosidase alfa degrades glycogen by catalyzing the hydrolysis of a-1,4- and a-1,6- glycosidic linkages of lysosomal glycogen. Structurally, Alglucosidase alfa is a glycoprotein with a calculated mass of 98,008 daltons for the 883 residue mature polypeptide chain, and a total mass of approximately 109,000 daltons, including carbohydrates. It is used for the treatment of Pompe disease (GAA deficiency) in infants and pediatric patients.
Indication
For the treatment of Pompe disease (GAA deficiency) in infants and pediatric patients.
Mechanism of Action
Alglucosidase alfa is designed to act as an exogenous source of GAA, acting to correct GAA deficiency that is the hallmark of Pompe disease. Alglucosidase alfa binds to mannose-6-phosphate receptors on the cell surface via carbohydrate groups on the GAA molecule, after which it is internalized and transported into lysosomes, where it undergoes proteolytic cleavage that results in increased enzymatic activity. It then exerts enzymatic activity in cleaving glycogen. Specifically, it hydrolyses alpha-1,4-glucose bonds.
Clearance
* 25+/- 4 mL/hr/kg [4-hour IV infusion of 20 mg/kg]
Toxicity
There have been no reports of overdose with alglucosidase alfa.
Active Ingredient/Synonyms
Acid maltase | Acid-alpha glucosidase | Aglucosidase alfa | Aglucosidase alpha | alpha-1,4-glucosidase | Alpha-glucosidase | Lysosomal Alpha-Glucosidase | Alglucosidase alfa |
Source of information: Drugbank (External Link). Last updated on: 3rd July 18. *Trade Name used in the content below may not be the same as the HSA-registered product.