Alglucosidase Alpha
- Atc Codes:A16AB07
- CAS Codes:420794-05-0
- PHARMGKB ID:420794-05-0
Table of contents
- Brand Names
- Chemistry
- Pharmacologic Category
- Mechanism of Action
- Therapeutic Use
- Contraindications
- Warnings and Precautions
- Adverse Reactions
- Genes that may be involved
- Dosage
- Pharmacokinetics and Pharmacodynamics
- Special Considerations
Brand Names
Europe
Austria: Myozyme; Belgium: Myozyme; Bulgaria: Myozyme; Cyprus: Myozyme; Czech Republic: Myozyme; Denmark: Myozyme; Estonia: Myozyme; Finland: Myozyme; France: Myozyme; Germany: Myozyme; Greece: Myozyme; Hungary: Myozyme; Ireland: Myozyme; Italy: Myozyme; Latvia: Myozyme; Lithuania: Myozyme; Luxembourg: Myozyme; Malta: Myozyme; Netherlands: Myozyme; Poland: Myozyme; Portugal: Myozyme; Romania: Myozyme; Slovakia: Myozyme; Slovenia: Myozyme; Spain: Myozyme; Sweden: Myozyme; UK: Myozyme.
North America
USA: Lumizyme, Myozyme.
Asia
Japan: Myozyme.
Drug combinations
Chemistry
Alglucosidase Alpha: C~4758~H~7262~N~1274~O~1369~S~35~. Mw: 105338. (1) Glucosidase, prepro-α-[199-arginine,223-histidine] (human); (2) [199-Arginine,223-histidine]prepro-α-glucosidase (human). CAS-420794-05-0 (2004).
Pharmacologic Category
Enzymes. (ATC-Code: A16AB07).
Mechanism of action
Recombinant form of the enzyme acid α-glucosidase which binds to mannose-6-phosphate receptors on the cell surface, becomes internalized and transported to lysosomes, resulting in increased enzymatic activity and glycogen cleavage.
Therapeutic use
Replacement therapy for Pompe disease (infantile onset).
Pregnancy and lactiation implications
Unlabeled use
Contraindications
Hypersensitivity to alglucosidase alpha or any component of the formulation.
Warnings and precautions
Severe hypersensitivity reactions (anaphylactic reactions and anaphylactic shock) might occur. Patients with cardiovascular and respiratory diseases are at increased risk of infusion-related reactions (arrhythmias, cardiorespiratory failure). Risk of infusion-related cardiorespiratory failure in Pompe disease (possibly due to fluid overload).