| Drug Name |
Alglucosidase alfa |
| Drug ID |
BADD_D00071 |
| 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. |
| Indications and Usage |
For the treatment of Pompe disease (GAA deficiency) in infants and pediatric patients. |
| Marketing Status |
approved |
| ATC Code |
A16AB07 |
| DrugBank ID |
DB01272
|
| KEGG ID |
D03207
|
| MeSH ID |
C509951
|
| PubChem ID |
Not Available
|
| TTD Drug ID |
D07DUU
|
| NDC Product Code |
58468-0160 |
| UNII |
DTI67O9503
|
| Synonyms |
GAA protein, human | LYAG protein, human | acid alpha-glucosidase, human | acid maltase, human | glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II), human | lysosomal alpha-glucosidase, human | alglucosidase alfa | rhGAA | recombinant human acid alpha-glucosidase | Nexviazyme | avlglucosidase alfa | Myozyme |
