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104138-64-9

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  • Galactosidase, α- (human clone λAG18 isoenzyme A subunit protein moiety reduced) (9CI)

    Cas No: 104138-64-9

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104138-64-9 Usage

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.

Originator

Transkaryotic Therapies (US)

Uses

Treatment of Fabry Disease.

Brand name

Replagal (Transkaryotic Therapies).

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.

Metabolism

Metabolic degradation pathway similar to other proteins.

Check Digit Verification of cas no

The CAS Registry Mumber 104138-64-9 includes 9 digits separated into 3 groups by hyphens. The first part of the number,starting from the left, has 6 digits, 1,0,4,1,3 and 8 respectively; the second part has 2 digits, 6 and 4 respectively.
Calculate Digit Verification of CAS Registry Number 104138-64:
(8*1)+(7*0)+(6*4)+(5*1)+(4*3)+(3*8)+(2*6)+(1*4)=89
89 % 10 = 9
So 104138-64-9 is a valid CAS Registry Number.

104138-64-9Upstream product

104138-64-9Downstream Products

104138-64-9Related news

Clinical observations on enzyme replacement therapy in patients with Fabry disease and the switch from Agalsidase Beta (cas 104138-64-9) to agalsidase alfa08/03/2019

BackgroundFabry disease is an X-linked inherited lysosomal storage disease that can be treated with the enzymes of agalsidase beta (Fabrazyme) and agalsidase alfa (Replagal). Since June 2009, viral contamination of Genzyme's production facility has resulted in a worldwide shortage of agalsi...detailed

Non-clinical evaluation of JR-051 as a biosimilar to Agalsidase Beta (cas 104138-64-9) for the treatment of Fabry disease08/02/2019

Fabry disease (FD) is an X-linked lysosomal storage disease. It is caused by deficiency of the enzyme α-galactosidase A (α-Gal A), which leads to excessive deposition of neutral glycosphingolipids, especially globotriaosylceramide (GL-3), in cells throughout the body. Progressive accumulation ...detailed

Low-dose Agalsidase Beta (cas 104138-64-9) treatment in male pediatric patients with Fabry disease: A 5-year randomized controlled trial08/01/2019

BackgroundFabry disease is a rare, X-linked, lifelong progressive lysosomal storage disorder. Severely deficient α-galactosidase A activity in males is associated with the classic phenotype with early-onset, multisystem manifestations evolving to vital organ complications during adulthood. We a...detailed

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