| 1-Deoxygalactonojirimycin (hydrochloride)α-galactosidase inhibitor |
Sample solution is provided at 25 µL, 10mM.
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- Purity = 98.00%
- COA (Certificate Of Analysis)
- MSDS (Material Safety Data Sheet)
Chemical structure
1-Deoxygalactonojirimycin (hydrochloride) Dilution Calculator
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| Cas No. | 75172-81-5 | SDF | Download SDF |
| Chemical Name | (2R,3S,4R,5S)-2-(hydroxymethyl)-3,4,5-piperidinetriol, monohydrochloride | ||
| Canonical SMILES | O[C@@H]1[C@@H](O)[C@@H](O)[C@@H](CO)NC1.Cl | ||
| Formula | C6H13NO4 • HCl | M.Wt | 199.6 |
| Solubility | ≤10mg/ml in DMSO | Storage | Store at -20°C |
| Physical Appearance | A crystalline solid | Shipping Condition | Evaluation sample solution : ship with blue ice.All other available size:ship with RT , or blue ice upon request |
| General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.Stock solution can be stored below -20℃ for several months. | ||
Target: α-galactosidase
IC50: 40 nM
1-Deoxygalactonojirimycin (hydrochloride) is a competitive inhibitor of α-galactosidase, with the IC50 value of 40 nM [1]. 1-Deoxygalactonojirimycin (hydrochloride) is used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease. Fabry disease is a rare genetic lysosomal storage disease, inherited in an X-linked manner which is caused by mutations in the α-galactosidase gene that commonly lead to enzyme instability, misfolding, and degradation.
In Vivo: In transgenic mice expressed human mutant α-galactosidase A, treatment with 1-Deoxygalactonojirimycin at a dosage of approximately 3 mg/kg body weight/day could significantly reduce globotriaosylceramide storage in the kidney. Besides, no abnormality of blood chemistry and pathological tissue damage was found in mice treated with 1-Deoxygalactonojirimycin at about 30 mg/kg body weight/day for 9 weeks [2].
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[J]. FEBS Journal, 2000, 267(13): 4179-4186.[2] Ishii S, Chang H, Yoshioka H, et al. Preclinical Efficacy and Safety of 1-Deoxygalactonojirimycin in Mice for Fabry Disease[J]. Journal of Pharmacology and Experimental Therapeutics, 2009, 328(3): 723-731.
