Molecular structure of Deoxygalactonojirimycin Hydrochloride, CAS Number: 75172-81-5
Deoxygalactonojirimycin Hydrochloride (CAS 75172-81-5)
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Alternate Names:
1-Deoxygalactonojirimycin Hydrochloride; Migalastat Hydrochloride; 1-Deoxygalactostatin Hydrochloride
Application:
Deoxygalactonojirimycin Hydrochloride is a potent and selective a-galactosidase inhibitor
CAS Number:
75172-81-5
Purity:
≥98%
Molecular Weight:
199.6
Molecular Formula:
C6H13NO4•HCl
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
* Refer to Certificate of Analysis for lot specific data.
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SDS & Certificate of Analysis
Deoxygalactonojirimycin Hydrochloride is a potent and selective alpha-gal A (alpha-galactosidase) inhibitor. By effectively inhibiting α-Gal A, migalastat hydrochloride significantly reduces the accumulation of glycosphingolipids in animal models with Fabry disease. The mechanism of migalastat hydrochloride involves binding to the active site of α-Gal A, thereby preventing the enzyme from catalyzing the hydrolysis of glycosphingolipids. Consequently, this inhibition leads to a decrease in the buildup of these lipids in cells and tissues.
Deoxygalactonojirimycin Hydrochloride (CAS 75172-81-5) References
- A novel enzymatic technique for limiting drug mobility in a hydrogel matrix. | Burke, MD., et al. 2005. J Control Release. 104: 141-53. PMID: 15866341
- Alpha-lactosylceramide as a novel 'sugar-capped' CD1d ligand for natural killer T cells: biased cytokine profile and therapeutic activities. | Zhang, W., et al. 2008. Chembiochem. 9: 1423-30. PMID: 18478523
- Enhanced endothelial delivery and biochemical effects of α-galactosidase by ICAM-1-targeted nanocarriers for Fabry disease. | Hsu, J., et al. 2011. J Control Release. 149: 323-31. PMID: 21047542
- Co-administration with the pharmacological chaperone AT1001 increases recombinant human α-galactosidase A tissue uptake and improves substrate reduction in Fabry mice. | Benjamin, ER., et al. 2012. Mol Ther. 20: 717-26. PMID: 22215019
- Novel alpha-galactosidase A mutation in a female with recurrent strokes. | Tuttolomondo, A., et al. 2012. Clin Biochem. 45: 1525-30. PMID: 22820434
- Novel imino sugar α-glucosidase inhibitors as antiviral compounds. | Howe, JD., et al. 2013. Bioorg Med Chem. 21: 4831-8. PMID: 23582447
- A family with various symptomatology suggestive of Anderson-Fabry disease and a genetic polymorphism of alpha galactosidase A gene. | Tuttolomondo, A., et al. 2015. Clin Biochem. 48: 55-62. PMID: 25281798
- Evidence that small molecule enhancement of β-hexosaminidase activity corrects the behavioral phenotype in Dutch APP(E693Q) mice through reduction of ganglioside-bound Aβ. | Knight, EM., et al. 2015. Mol Psychiatry. 20: 109-17. PMID: 25349165
- Coformulation of a Novel Human α-Galactosidase A With the Pharmacological Chaperone AT1001 Leads to Improved Substrate Reduction in Fabry Mice. | Xu, S., et al. 2015. Mol Ther. 23: 1169-1181. PMID: 25915924
- Increased glycolipid storage produced by the inheritance of a complex intronic haplotype in the α-galactosidase A (GLA) gene. | Gervas-Arruga, J., et al. 2015. BMC Genet. 16: 109. PMID: 26334996
- Inter-familial and intra-familial phenotypic variability in three Sicilian families with Anderson-Fabry disease. | Tuttolomondo, A., et al. 2017. Oncotarget. 8: 61415-61424. PMID: 28977874
- Salivary α-Iduronidase Activity as a Potential New Biomarker for the Diagnosis and Monitoring the Effect of Therapy in Mucopolysaccharidosis Type I. | van Doorn, J., et al. 2018. Biol Blood Marrow Transplant. 24: 1808-1813. PMID: 29906569
- Longitudinal Analysis of Ocular Disease in Children with Mucopolysaccharidosis I after Hematopoietic Cell Transplantation. | van den Broek, BTA., et al. 2020. Biol Blood Marrow Transplant. 26: 928-935. PMID: 31786241
- Assessment of Gene Variant Amenability for Pharmacological Chaperone Therapy with 1-Deoxygalactonojirimycin in Fabry Disease. | Lukas, J., et al. 2020. Int J Mol Sci. 21: PMID: 32023956
- Misfolding of Lysosomal α-Galactosidase a in a Fly Model and Its Alleviation by the Pharmacological Chaperone Migalastat. | Braunstein, H., et al. 2020. Int J Mol Sci. 21: PMID: 33036426
Inhibitor of:
alpha-gal A.Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
Deoxygalactonojirimycin Hydrochloride, 10 mg | sc-202130 | 10 mg | $388.00 |