Agalsidase beta drug data and news

Agalsidase beta drug data, resources, and news articles (when available). Onconews.org provides news on cancer research. This section, which includes profiles on medicines that may or not be cancer-related is in beta form. If things run smoothly we will be releasing a new format late in the summer of 2006.

Generic name Agalsidase beta
Brand Names/Synonyms Alpha-galactosidase A precursor;Melibiase;Alpha-D- galactoside galactohydrolase;Alpha-D-galactosidase A;Agalsidase alfa; Fabrazyme
Indication For treatment of Fabry's disease (alpha-galactosidase A deficiency)
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Description Recombinant human alpha-galactosidase A. The mature protein is composed of 2 subunits of 398 residues. Protein is glycosylated and produced by CHO cells
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.
Agalsidase beta News
(When available)
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Dosage Forms POWDER FOR SOLUTION
Drug_Category Enzyme replacement; enzymes (44:00.00)
Absorption Not Available
Interactions Not Available
Toxicity Not Available
Organisms Affected Humans and other mammals
Chemical IUPAC Name Human alpha-galactosidase A.
Chemical Formula C2029H3080N544O587S27
Molecular Weight 45351.6
Smiles String Not Available
Melting Point Not Available
Water Solubility Not Available
State Transparent, slightly viscous, aqueous liquid
LogP/Hphobicity -0.307
Isoelectric Point 5.17
Biotransformation Not Available
Half Life 45-102 min
Protein Binding [%] Not Available
RxList Link RXlist
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Drug Reference http://www.drugs.com/cons/Agalsidase_beta.html
http://www.rxlist.com/cgi/generic3/fabrazyme.htm
Drug Type Approved Drug
Accession No BIOD00075
CAS Registry Number 104138-64-9
KEGG Compound ID Not Available
PubChem ID Not Available
PharmGKB ID PA10006
SwissProt ID AGAL_HUMAN (P06280)
GenBank ID X14448
Drug ID Number [DIN] 2248966

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