Alpha-D-galactoside Galactohydrolase
Alpha-D-galactoside Galactohydrolase Uses, Dosage, Side Effects, Food Interaction and all others data.
Alpha-D-galactoside Galactohydrolase is a recombinant human α-galactosidase A similar to agalsidase beta. While patients generally do not experience a clinically significant difference in outcomes between the two drugs, some patients may experience greater benefit with agalsidase beta. Use of agalsidase beta has decreased in Europe, in favor of agalsidase alfa, after a contamination event in 2009.
Alpha-D-galactoside Galactohydrolase was granted EMA approval on 3 August 2001.
Alpha-D-galactoside Galactohydrolase is a recombinant human α-galactosidase A used as enzyme replacement therapy in the treatment of Fabry disease. It has a long duration of action and a wide therapeutic index. Patients should be counselled regarding the risk of infusion related reactions and hypersensitivity.
| Trade Name | Alpha-D-galactoside Galactohydrolase |
| Generic | Agalsidase alfa |
| Agalsidase alfa Other Names | Agalsidase alfa, Agalsidase alfa (genetical recombination), Agalsidase alpha, alpha-D-galactopyranosidase, alpha-D-galactosidase, alpha-D-galactosidase enzyme, alpha-D-galactoside galactohydrolase, alpha-galactisidase, alpha-galactosidase A, Recombinant alpha-galactosidase A |
| Type | |
| Formula | C2029H3080N544O587S27 |
| Weight | 45351.6 Da |
| Protein binding | Agalsidase alfa is not expected to be protein bound in circulation. |
| Groups | Approved |
| Therapeutic Class | |
| Manufacturer | |
| Available Country | |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
Alpha-D-galactoside Galactohydrolase is a recombinant human alpha-galactosidase indicated to treat Fabry disease, a genetic deficiency in the enzyme leading to buildup of globotriaosylceramide.
Alpha-D-galactoside Galactohydrolase is indicated in the treatment of Fabry disease.
Alpha-D-galactoside Galactohydrolase is also used to associated treatment for these conditions: Fabry's Disease, Enzyme replacement
How Alpha-D-galactoside Galactohydrolase works
α-galactosidase A is uptaken by cells via the mannose 6 phosphate receptor. Alpha-D-galactoside Galactohydrolase hydrolyzes globotriaosylceramide and other glycosphingolipids that would normally be hydrolyzed by endogenous α-galactosidase A. Preventing the accumulation of glycosphingolipids prevents or reduces the severity of manifestations of Fabry disease such as renal failure, cardiomyopathy, or cerebrovascular events.
Toxicity
Data regarding overdoses of agalsidase alfa are not readily available. Patients experiencing an overdose of agalsidase alfa may experience an increased incidence and severity of adverse effects. Overdose can be managed through the use of symptomatic and supportive measures.
Food Interaction
No interactions found.Volume of Distribution
The volume of distribution at steady state in non end stage renal disease patients was approximately 17% of body weight regardless of sex.
Elimination Route
A dose of agalsidase alfa in non end stage renal disease patients reaches a Cmax of 3710 ± 855 U/mL with an AUC of 256,958 ± 63,499 min*U/mL.
Half Life
The elimination half life was 108 ± 17 minutes for males and 89 ± 28 minutes for females.
Clearance
The clearance for doses of 0.007-0.2 mg/kg were 2.66 mL/min/kg for males and 2.10 mL/min/kg for females.
Elimination Route
After nonspecific proteolysis, the amino acids from protein drugs are reused for protein synthesis or further broken down and eliminated by the kidneys.
Innovators Monograph
You find simplified version here Alpha-D-galactoside Galactohydrolase
