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Lysosomal Storage Disease

Lysosomal storage diseases (LSDs) are a group of rare, inherited metabolic disorders characterized by the deficiency of specific lysosomal enzymes. This deficiency leads to the abnormal accumulation of undigested or partially digested macromolecules within lysosomes, causing cellular dysfunction and progressive damage to various organs and tissues. LSDs present with a wide spectrum of clinical manifestations affecting neurological, skeletal, visceral, and hematological systems, often leading to severe and life-limiting conditions.
  • 1-Deoxynojirimycin
    1-Deoxynojirimycin
    CAS NO.：19130-96-2
    Purity：99% Brand：Phytopurify
    Catalog No Package Original Price Price Inventory Quantity Operating
    BP0030-20mg 20mg $20.00
    BP0030-100mg 100mg $40.00
    BP0030-1000mg 1000mg $96.00
  • Miglustat
    Miglustat
    CAS NO.：72599-27-0
    Formula：C10H21NO4
    Purity：98% Brand：Phytopurify
    Catalog No Package Original Price Price Inventory Quantity Operating
    BP5229-20mg 20mg $50.00
    BP5229-100mg 100mg $150.00
    BP5229-1000mg 1000mg $550.00
  • Diphyllin
    Diphyllin
    CAS NO.：22055-22-7
    Formula：C21H16O7
    Purity：98% Brand：Phytopurify
    Catalog No Package Original Price Price Inventory Quantity Operating
    BP4546-5mg 5mg $96.00
    BP4546-10mg 10mg $160.00
    BP4546-20mg 20mg $260.00
    BP4546-100mg 100mg $850.00
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