Agalsidase Beta

Agalsidase Beta Uses, Dosage, Side Effects, Food Interaction and all others data.

Agalsidase Beta is a recombinant human α-galactosidase A similar to agalsidase alfa. 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.

Agalsidase Beta was granted FDA approval on 24 April 2003.

Agalsidase Beta is a recombinant human α-galactosidase A used as enzyme replacement therapy in the treatment of Fabry disease.[L16398] 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 Agalsidase Beta
Generic Agalsidase beta
Agalsidase beta Other Names Agalsidasa beta, Agalsidase beta, Agalsidase beta (genetical recombination)
Weight 35mg, 5mg,
Type Intravenous Powder For Injection, Intravenous
Formula C2029H3080N544O587S27
Weight 45351.6 Da
Protein binding

Data regarding the protein binding of agalsidase beta is not readily available.

Groups Approved, Investigational
Therapeutic Class
Manufacturer
Available Country United States
Last Updated: September 19, 2023 at 7:00 am
Agalsidase Beta
Agalsidase Beta

Uses

Agalsidase Beta is a recombinant human alpha-galactosidase indicated to treat Fabry disease, a genetic deficiency in the enzyme leading to buildup of globotriaosylceramide.

Agalsidase Beta is indicated in the treatment of Fabry disease.

Agalsidase Beta is also used to associated treatment for these conditions: Fabry's Disease

How Agalsidase Beta works

α-galactosidase A is uptaken by cells via the mannose 6 phosphate receptor. Agalsidase Beta 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 beta are not readily available. Patients experiencing an overdose of agalsidase beta 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

A 1 mg/kg dose of agalsidase beta with a mean infusion length of 115 minutes has a VSS of 112 ± 13 mL/kg.

Elimination Route

A 1 mg/kg dose of agalsidase beta with a mean infusion length of 115 minutes reaches a Cmax 5.0 ± 1.1 µg/mL with an AUC of 496 ± 137 µg*min/mL.

Half Life

agalsidase beta has a half like of 67 ± 12 min for a 1 mg/kg dose with a mean infusion length of 115 minutes.

Clearance

A 1 mg/kg dose of agalsidase beta with a mean infusion length of 115 minutes has a clearance of 2.1 ± 0.7 mL/min/kg.

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.

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*** Taking medicines without doctor's advice can cause long-term problems.
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