1-Deoxygalactonojirimycin is a competititve inhibitor of α-galactosidase (IC50 = 40 nM). At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to α-galactosidase and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes. Pharmacological chaperones, including 1-Deoxygalactonojirimycin, are used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease.
1-Deoxygalactonojirimycin is a competititve inhibitor of α-galactosidase (IC50 = 40 nM). At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to α-galactosidase and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes. Pharmacological chaperones, including 1-Deoxygalactonojirimycin, are used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease.
Shipping & Handling
Shipped In
Room Temperature in continental US; may vary elsewhere
Safety & Storage
Storage Temperature
-20°C
Safety Statement
WARNING This product is not for human or veterinary use. Information provided in the product description is from published literature. Due to the nature of scientific experimentation, your results (e.g., selectivity and effective concentrations) or specific application for this product may differ. If you have questions about how this product fits your application, please contact our technical support staff.
Room Temperature in continental US; may vary elsewhere
Safety & Storage
Storage Temperature
-20°C
Safety Statement
WARNING This product is not for human or veterinary use. Information provided in the product description is from published literature. Due to the nature of scientific experimentation, your results (e.g., selectivity and effective concentrations) or specific application for this product may differ. If you have questions about how this product fits your application, please contact our technical support staff.
Regulatory & Compliance
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We’re currently in BETA - Interested in joining? Let us know and we’ll get you set up with early access.