A competitive inhibitor of α-galactosidase (IC50 = 40 nM); binds to α-galactosidase in cells and chaperones unstable enzyme variants through the endoplasmic reticulum

1-Deoxygalactonojirimycin (hydrochloride) – 10 mg

Brand:
Cayman
CAS:
75172-81-5
Storage:
-20
UN-No:
Non-Hazardous - /

Fabry disease is an X-linked hereditary lysosomal storage disorder caused by mutations in the α-galactosidase gene that commonly lead to enzyme instability, misfolding, and degradation.{28086,28087} 1-Deoxygalactonojirimycin is a competititve inhibitor of α-galactosidase (IC50 = 40 nM).{28089} At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to α-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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Description

A competitive inhibitor of α-galactosidase (IC50 = 40 nM); binds to α-galactosidase in cells and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes

Formal name: (2R,3S,4R,5S)-2-(hydroxymethyl)-3,4,5-piperidinetriol, monohydrochloride

Synonyms:  DGJ|Migalastat

Molecular weight: 199.6

CAS: 75172-81-5

Purity: ≥98%

Formulation: A crystalline solid

Product Type|Biochemicals|Small Molecule Inhibitors||Research Area|Endocrinology & Metabolism|Carbohydrate Metabolism||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism

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