Agalsidase Alpha
- Atc Codes:A16AB03
- CAS Codes:104138-64-9
- PHARMGKB ID:104138-64-9
Table of contents
- Brand Names
- Chemistry
- Pharmacologic Category
- Mechanism of Action
- Therapeutic Use
- Pregnancy and Lactation Implications
- Contraindications
- Warnings and Precautions
- Adverse Reactions
- Genes that may be involved
- Drug Interactions
- Dosage
- Pharmacokinetics and Pharmacodynamics
- Special Considerations
Brand Names
Europe
Austria: Replagal; Belgium: Replagal; Bulgaria: Replagal; Cyprus: Replagal; Czech Republic: Replagal; Denmark: Replagal; Estonia: Replagal; Finland: Replagal; France: Replagal; Germany: Replagal; Greece: Replagal; Hungary: Replagal; Ireland: Replagal; Italy: Replagal; Latvia: Replagal; Lithuania: Replagal; Luxembourg: Replagal; Malta: Replagal; Netherlands: Replagal; Poland: Replagal; Portugal: Replagal; Romania: Replagal; Slovakia: Replagal; Slovenia: Replagal; Spain: Replagal; Sweden: Replagal; UK: Replagal.
North America
Canada: Replagal.
Asia
Japan: Replagal.
Drug combinations
Chemistry
Agalsidase Alpha: C~2029~H~3080~N~544~O~587~S~27~ (subunit protein moiety reduced). Mw: approx. 45351.01 daltons. (1) Galactosidase, α – (human clone λAG^18^ isoenzyme A subunit protein moiety reduced); (2) λ-Galactosidase (human-clone λAG^18^ isoenzyme A subunit protein moiety reduced). CAS-104138-64-9 (2000).
Pharmacologic Category
Enzymes. (ATC-Code: A16AB03).
Mechanism of action
Catalyzes the hydrolysis of globotriaosylceramide (Gb-3) and other glycosphingolipids.
Therapeutic use
Replacement therapy for Fabry disease.
Pregnancy and lactiation implications
The benefits versus risks should be considered before initiating therapy in pregnant women. Use with caution during lactation.
Unlabeled use
Contraindications
Hypersensitivity to agalsidase alpha or any component of the formulation. Concomitant use with chloroquine, amiodarone, monobenzone, or gentamicin (might inhibit intracellular agalsidase alpha activity).
Warnings and precautions
Patients may develop IgG antibody formation. Mild acute reactions (chills, facial flushing) are common. Severe reactions (nausea, pyrexia, rigor, tachycardia, urticaria, vomiting) are rare.