104138-64-9
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Basic information
- Product Name: Agalsidase Beta
- Synonyms: Agalsidase Beta
- CAS NO:104138-64-9
- Molecular Formula:
- Molecular Weight:
- EINECS: N/A
- Product Categories: N/A
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Chemical Properties
- Melting Point: N/A
- Boiling Point: N/A
- Flash Point: N/A
- Appearance: /
- Density: N/A
- Refractive Index: N/A
- Storage Temp.: N/A
- Solubility: N/A
- CAS DataBase Reference: Agalsidase Beta(CAS DataBase Reference)
- NIST Chemistry Reference: Agalsidase Beta(104138-64-9)
- EPA Substance Registry System: Agalsidase Beta(104138-64-9)
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Safety Data
- Hazard Codes: N/A
- Statements: N/A
- Safety Statements: N/A
- WGK Germany:
- RTECS:
- HazardClass: N/A
- PackingGroup: N/A
- Hazardous Substances Data: 104138-64-9(Hazardous Substances Data)
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104138-64-9 Usage
Uses
Used in Pharmaceutical Industry:Agalsidase Beta is used as an enzyme replacement therapy for the treatment of Fabry disease. It helps in the breakdown of the glycosphingolipid Gb3, which accumulates in the lysosomes of various organs due to the deficiency of a-galactosidase A enzyme. This enzyme replacement therapy mitigates the chronic symptoms and complications associated with Fabry disease, such as neuropathic pain, angiokeratomas, and organ damage.
Used in Clinical Trials:
In clinical trials, Agalsidase Beta is used as an intravenous infusion administered twice weekly for durations ranging from six months to one year. It has been found to be safe and effective in reversing the accumulation of microvascular endothelial deposits of globotriaosylceramide in the kidneys, heart, and skin, thereby improving the quality of life and life expectancy of patients with Fabry disease.
Brand Name:
Replagal (Transkaryotic Therapies) is the brand name under which Agalsidase Beta is marketed for the treatment of Fabry disease.
Originator
Transkaryotic Therapies (US)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
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
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
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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