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RX BIN: 015558RX PCN: HTGroup ID: DDN6600Card Holder ID: DDN6600
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2019 Price of Alglucosidase alfa

Myozyme 50 mg vial

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Alglucosidase alfa volume of distribution

* 96 16 mL/kg (20 mg/kg dose) * 119 28 mL/kg (40 mg/kg dose)

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What is Alglucosidase alfa

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.

Alglucosidase alfa 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.

Dosage forms of Alglucosidase alfa

Injection, powder, for solutionintravenous5 mg/mL
Injection, powder, lyophilized, for solutionintravenous5 mg/mL
Powder for concentrate for solution for infusionIntravenous use50 mg

Prescription Generics


International Brands



Acid maltase Acid-alpha glucosidase


Genzyme Corporation

CAS number






Affected organisms

Humans and other mammals

Indication of Alglucosidase alfa

For the treatment of Pompe disease (GAA deficiency) in infants and pediatric patients.

Toxicity of Alglucosidase alfa

There have been no reports of overdose with alglucosidase alfa.

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