FABRAZYME 35mg - Agalsidase Beta by Genzyme (Sanofi)

    FABRAZYME

    35mg
    Specialty Medications

    FABRAZYME35mg

    FABRAZYME (Agalsidase Beta) 35mg is a recombinant enzyme replacement therapy for Fabry disease, providing human alpha-galactosidase A to clear accumulated GL-3 from tissues.

    Generic Name:Agalsidase Beta
    Manufacturer:Genzyme (Sanofi)

    Key Benefits

    • Only FDA-approved agalsidase beta for Fabry disease
    • Demonstrated GL-3 clearance from kidney capillary endothelium (69% vs 0% placebo)
    • Long-term stabilization of renal function (eGFR) over 10+ years
    • Reduction in left ventricular hypertrophy
    • Improvement in neuropathic pain symptoms
    • Every-2-week dosing schedule

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    How FABRAZYME Works

    1

    Fabrazyme provides exogenous recombinant human alpha-galactosidase A enzyme. In Fabry disease, patients lack this enzyme causing GL-3 accumulation. The infused enzyme is taken up by cells via mannose-6-phosphate receptors, localizes to lysosomes, and catalyzes hydrolysis of GL-3, reducing its pathological accumulation in kidneys, heart, skin, and other organs.

    About FABRAZYME 35mg

    FABRAZYME (Agalsidase Beta) 35mg by Genzyme (Sanofi) is the gold-standard enzyme replacement therapy (ERT) for Fabry disease, a rare X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A enzyme. Fabry disease affects approximately 1 in 40,000-60,000 males and leads to progressive accumulation of globotriaosylceramide (GL-3/Gb3) in lysosomes throughout the body. Without treatment, patients develop progressive renal failure, hypertrophic cardiomyopathy, stroke, and severe neuropathic pain. Fabrazyme provides recombinant human alpha-galactosidase A produced in Chinese Hamster Ovary (CHO) cells. The enzyme is taken up by cells via mannose-6-phosphate receptors, transported to lysosomes, and catalyzes hydrolysis of accumulated GL-3. FDA-approved since 2003, Fabrazyme has demonstrated sustained stabilization of renal function and reduction of cardiac mass over 10+ years of treatment. As a licensed pharmaceutical supplier, we provide authentic Genzyme/Sanofi Fabrazyme with validated cold chain handling, certificate of authenticity, and competitive wholesale pricing for hospitals and metabolic disease centers worldwide.

    Common Uses

    • Fabry disease (alpha-galactosidase A deficiency)
    • GL-3 accumulation in kidney, heart, and other organs
    • Prevention of progressive renal failure in Fabry disease
    • Management of Fabry-related cardiomyopathy
    • Lysosomal storage disorder treatment

    Dosage Information

    1 mg/kg body weight IV infusion every 2 weeks. Initial rate ≤0.25 mg/min (15 mg/hr), may increase gradually if tolerated. Each 35mg vial reconstituted with 7.2 mL Sterile Water for Injection, then diluted in 0.9% NaCl.

    Always consult with a qualified healthcare professional for proper dosing. This information is for reference only.

    Storage & Handling

    Store lyophilized powder at 2-8°C (36-46°F). Do not freeze. Protect from light. After reconstitution, diluted solution should be used within 24 hours. Ship in validated cold chain packaging with temperature data loggers.

    We ensure proper cold chain handling during shipping to maintain product integrity.

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