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Replagal: Full Drug Profile

Medically reviewed by Min Clinic Staff | Updated: January 2026

Replagal - General Information

Recombinant human alpha-galactosidase A. The mature protein is composed of 2 subunits of 398 residues. Protein is glycosylated and produced by CHO cells

 

Pharmacology of Replagal

Used in the treatment of Fabry disease, an X-linked genetic disorder of glycosphingolipid metabolism. The disease is characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A, which leads to progressive accumulation of glycosphingolipids, predominantly GL-3, in many body tissues. Clinical manifestations of Fabry disease include renal failure, cardiomyopathy, and cerebrovascular accidents. Fabrazyme is intended to provide an exogenous source of alpha-galactosidase A and to limit the accumulation of these glycolipids in the tissues.

 

Replagal for patients

Patients should be informed that a Registry has been established in order to better understand the variability and progression of Fabry disease in the population as a whole and in women, and to monitor and evaluate long-term treatment effects of Fabrazyme. The Registry will also monitor the effect of Fabrazyme on pregnant women and their offspring, and determine if FabrazymeÒ is excreted in breast milk. Patients should be encouraged to participate and advised that their participation is voluntary and may involve long-term follow-up. For more information visit www.fabryregistry.com or call (800) 745-4447.

Please also refer to the WARNINGS and PRECAUTIONS sections.

 

Replagal Interactions

No drug interaction studies were performed.

No in vitro metabolism studies were performed.

 

Replagal Contraindications

No known contraindications.

 

Additional information about Replagal

Replagal Indication: For treatment of Fabry's disease (alpha-galactosidase A deficiency) Mechanism Of Action: Alpha-galactosidase A catalyzes the hydrolysis of globotriaosylceramide (GL-3) and other a-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose. Drug Interactions: Not Available Food Interactions: Not Available Generic Name: Agalsidase beta Synonyms: Agalsidase alfa; Alpha-D- galactoside galactohydrolase; Alpha-D-galactosidase A; Alpha-galactosidase A precursor; Melibiase Drug Category: Enzyme Replacement Agents Drug Type: Biotech; Approved; Investigational Other Brand Names containing Agalsidase beta: Fabrazyme (Genzyme Corp); Replagal; Absorption: Not Available Toxicity (Overdose): Not Available Protein Binding: Not Available Biotransformation: Not Available Half Life: 45-102 min Dosage Forms of Replagal: Powder, for solution Intravenous Chemical IUPAC Name: Human alpha-galactosidase A. Chemical Formula: C2029H3080N544O587S27 Agalsidase beta on Wikipedia: https://en.wikipedia.org/wiki/Agalsidase_beta Organisms Affected: Humans and other mammals