Replagal
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