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Results for "

GM1-gangliosidosis

" in MedChemExpress (MCE) Product Catalog:

3

Inhibitors & Agonists

1

Natural
Products

Cat. No. 상품명 Target 연구분야 Chemical Structure
  • HY-N7390
    Cholesterol glucuronide

    		Endogenous Metabolite Metabolic Disease
    Cholesterol glucuronide is an endogenous metabolite of lipid generated in the liver by UDP glucuonyltransferase [1] .
    Cholesterol glucuronide
  • HY-14829A
    Afegostat hydrochloride

    D-Isofagomine hydrochloride; Isofagomine hydrochloride

    		 Glycosidase Metabolic Disease
    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 [1].
    Afegostat hydrochloride
  • HY-153922
    N-Octyl-α-4-epivalienamine

    		Glycosidase Neurological Disease
    N-Octyl-α-4-epivalienamine is an orally active and CNS-penetrant molecular chaperone that induces high expression of the deficient β-galactosidase activity. N-Octyl-α-4-epivalienamine ameliorates symptoms and increase survival rate in a mouse model of GM1-gangliosidosis. N-Octyl-α-4-epivalienamine can be used for neurogenetic disease research [1].
    N-Octyl-α-4-epivalienamine

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