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Agalsidase beta
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DRUG INFO
Agalsidase beta
Drug Name:
Agalsidase beta
Indication: For treatment of Fabry's disease (alpha-galactosidase A deficiency)
Pharmacology: Used in the treatment of Fabry disease, an X-linked genetic disorder of glycosphingolipid metabolism. The disease is characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A, which leads to progressive accumulation of glycosphingolipids, predominantly GL-3, in many body tissues. Clinical manifestations of Fabry disease include renal failure, cardiomyopathy, and cerebrovascular accidents. Fabrazyme is intended to provide an exogenous source of alpha-galactosidase A and to limit the accumulation of these glycolipids in the tissues.
Mechanism Of Action: alpha-galactosidase A catalyzes the hydrolysis of globotriaosy-lceramide (GL-3) and other a-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose.
Drug Category: Enzyme replacement; enzymes (44:00.00)
Brand Names/Synonyms: Alpha-galactosidase A precursor;Melibiase;Alpha-D- galactoside galactohydrolase;Alpha-D-galactosidase A;Agalsidase alfa; Fabrazyme
Dosage Forms: POWDER FOR SOLUTION
Absorption: Not Available
Interactions: Not Available
Chemical IUPAC Name: Human alpha-galactosidase A.
Chemical Formula: C2029H3080N544O587S27
Half Life: 45-102 min
Drug Type: Approved Drug
# Accession No: BIOD00075
CAS Registry Number: 104138-64-9
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