1-Deoxygalacto<wbr/>nojirimycin (hydro<wbr>chloride)

1-Deoxygalactonojirimycin (hydrochloride)

CAT N°: 17179
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From 66.00 56.10

1-Deoxygalactonojirimycin is a competititve inhibitor of ?-galactosidase (IC50 = 40 nM).{28089} At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to ?-galactosidase and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes.{28088,28086} Pharmacological chaperones, including 1-Deoxygalactonojirimycin, are used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease.{28086}

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