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Alglucosidase alfa (TP-017CL)

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.      

Specifications
Classify
Protein therapeutics replacing a protein that is deficient or abnormal: Metabolic enzyme deficiencies
Therapeutic
Alglucosidase-α
Trade Name
Myozyme
Function
Degrades glycogen by catalyzing the hydrolysis of a-1,4 and a-1,6 glycosidic linkages of lysosomal glycogen
Examples of Clinical Use
Pompe disease (glycogen storage disease type ii)
References
Synonyms
Acid maltase, Acid-alpha glucosidase, Aglucosidase alfa, Aglucosidase alpha, alpha-1,4-glucosidase, Alpha-glucosidase, Lysosomal Alpha-Glucosidase

For lab research use only, not for diagnostic, therapeutic or any in vivo human use.

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