Afegostat hydrochloride (D-Isofagomine hydrochloride) is a potent beta-galactosidase inhibitor with activity ameliorating GM1-gangliosidosis and Morquio B disease-associated mutations. Afegostat hydrochloride is able to induce the maturation of mutant beta-galactosidase in fibroblasts from patients with GM1-gangliosidosis. Afegostat hydrochloride also promotes the reduction of keratin sulfate and oligosaccharide load in patient cells[1].
Molekulargewicht:
183.63
CAS Nummer:
[161302-93-8]
Formel:
C6H14ClNO3
Target-Kategorie:
Glycosidase
Anwendungsbeschreibung:
MCE Product type: Reference compound
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