Agalsidase Beta

Base Information

• Chemical Name: Agalsidase Beta
• CAS No.: 104138-64-9
• Molecular Weight: 0.00000
• Mol file: 104138-64-9.mol

Synonyms:Agalsidase alfa;Galactosidase, alpha- (human Clone PGB73);Replagal;Agalsidase alfa [USAN];Fabrazyme;Galactosidase, alpha-(human clone lambdaAG18 isoenzyme A subunit protein moiety reduced);EC 3.2.1.22;Galactosidase, alpha- (human isoenzyme A reduced);Adefovir;Agalsidase beta;

Chemical Property of Agalsidase Beta

Chemical Property:

• PSA: 0.00000
• LogP: 0.00000

Purity/Quality:

99% ^*data from raw suppliers

AGALSIDASE BETA 95.00% ^*data from reagent suppliers

Safty Information:

• Pictogram(s):  
• Hazard Codes: 

MSDS Files:

Useful:

• Description: Fabry's disease is a rare genetic glycolipid-storage disorder characterized by the deficiency of the lysosomal enzyme a-galactosidase A. This enzyme participates in the catabolism of the glycosphingolipid globotriaosylceramide (Gb3) by specifically cleaving its terminal s-linked galactose residue. Progressive accumulation of Gb3 and related glycosphingolipids in vascular endothelial lysosomes of the kidneys, heart, skin and brain leads to several chronic symptoms such as debilitating neuropathic pain and characteristic cutaneous lesions called angiokeratomas. As vital organs are affected with age, premature death usually occurs in the fourth or fifth decade of life due to renal, cardiac or cerebral complications. Agalsidase alfa is delivered to its lysosomal site of action via the recognition of its mannose-6-phosphate (M6P) residues by specific M6P receptors on the cell surface followed by endocytosis. In clinical trials, twice weekly intravenous infusions of agalsidase alfa over six months to one year have been found to be safe and effective in reversing the accumulation of microvascular endothelial deposits of globotriaosylceramide in the kidneys, heart and skin.
• Uses: Treatment of Fabry Disease.
• Clinical Use: Treatment of Fabry disease. Only to be prescribed by specialist centres.
• Drug interactions: Potentially hazardous interactions with other drugs Fabrazyme should not be administered with chloroquine, amiodarone, benoquin or gentamicin due to a theoretical risk of inhibition of intra-cellular α-galactosidase activity.