Afegostat hydrochloride (D-Isofagomine hydrochloride) is a potent β-galactosidase inhibitor with activity ameliorating GM1-gangliosidosis and Morquio B disease-associated mutations. Afegostat hydrochloride is able to induce the maturation of mutant β-galactosidase in fibroblasts from patients with GM1-gangliosidosis. Afegostat hydrochloride also promotes the reduction of keratin sulfate and oligosaccharide load in patient cells.
Products | Products > Disease Areas > Metabolic or Endocrine Disease | Products > Signaling Pathways > Metabolic Enzyme/Protease | Products > Research Areas > Metabolic Disease | Products > Signaling Pathways > Glycosidase
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