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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1-Deoxygalactonojirimycin (hydrochloride)
17179-1
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1-Deoxygalactonojirimycin (hydrochloride)
17179-1
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