An α-galactosidase inhibitor and chaperone
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1-Deoxygalactonojirimycin (hydrochloride)

Item No. 17179

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
(2R,3S,4R,5S)-2-(hydroxymethyl)-3,4,5-piperidinetriol, monohydrochloride
CAS Number
75172-81-5
Synonyms
  • DGJ
  • Migalastat
Molecular Formula
C6H13NO4 • HCl
Formula Weight
199.6
Purity
≥98%
Formulation
A crystalline solid
Solubility(Learn about Variance in Solubility)
DMSO: 10 mg/mlPBS (pH 7.2): 1 mg/ml
λmax
314 nm
SMILES
O[C@@H]1[C@@H](O)[C@@H](O)[C@@H](CO)NC1.Cl
InChi Code
InChI=1S/C6H13NO4.ClH/c8-2-3-5(10)6(11)4(9)1-7-3;/h3-11H,1-2H2;1H/t3-,4+,5+,6-;/m1./s1
InChi Key
ZJIHMALTJRDNQI-OLALXQGDSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    1 mg

    $42

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    5 mg

    $84

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    10 mg

    $126

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    Product Description

    1-Deoxygalactonojirimycin is a competititve inhibitor of α-galactosidase (IC50 = 40 nM).1 At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to α-galactosidase and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes.2,3 Pharmacological chaperones, including 1-Deoxygalactonojirimycin, are used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease.3

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Asano, N., Ishii, S., Kizu, H., et al. In vitro inhibition and intracellular enhancement of lysosomal α-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives. Eur. J. Biochem. 267(13), 4179-4186 (2000).

    2. Ishii, S., Chang, H.H., Yoshioka, H., et al. Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease. J. Pharmacol. Exp. Ther. 328(3), 723-731 (2009).

    3. Siekierska, A., De Baets, G., Ruemers, J., et al. α-Galactosidase aggregation is a determinant of pharmacological chaperone efficacy on Fabry disease mutants. The Journal of Biological Chemisty 287(34), 28386-28397 (2012).