D9641
Deoxygalactonojirimycin hydrochloride
powder or solid, ≥98% (HPLC)
Synonyme(s) :
1,5-Dideoxy-1,5-imino-D-galactitol, Migalastat
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A propos de cet article
Formule empirique (notation de Hill) :
C6H13NO4 · HCl
Numéro CAS:
Poids moléculaire :
199.63
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.32
MDL number:
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Laissez-nous vous aiderNom du produit
Deoxygalactonojirimycin hydrochloride,
biological source
synthetic (organic)
Quality Level
assay
≥98% (HPLC)
form
powder or solid
solubility
water: soluble 1 mg/mL
storage temp.
−20°C
SMILES string
Cl.OC[C@H]1NC[C@H](O)[C@@H](O)[C@H]1O
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
Application
Deoxygalactonojirimycin hydrochloride has been used as an α-galactosidase A inhibitor to assess the enzymatic activity of α-galactosidase A. It has also been used as an α-galactosidase A inhibitor to study its effects on the mRNA levels in human embryonic kidney (HEK) cells and hippocampal neurons.
Biochem/physiol Actions
Deoxygalactonojirimycin hydrochloride is an inhibitor of α-galactosidase A. Deoxygalactonojirimycin exhibits therapeutic effects against Fabry disease.
Classe de stockage
11 - Combustible Solids
wgk
WGK 3
ppe
dust mask type N95 (US), Eyeshields, Gloves
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Libia Catalina Salinas Castellanos et al.
Neurochemistry international, 140, 104824-104824 (2020-08-26)
Neuropathic pain is one of the key features of the classical phenotype of Fabry disease (FD). Acid sensing ion channels (ASICs) are H+-gated cation channels, which belong to the epithelial sodium channel/DeGenerin superfamily, sensitive to the diuretic drug Amiloride. Molecular
Anthony Markham
Drugs, 76(11), 1147-1152 (2016-06-29)
Migalastat (Galafold™)-a small molecule drug developed by Amicus Therapeutics that restores the activity of specific mutant forms of α-galactosidase-has been approved for the treatment of Fabry disease in the EU in patients with amenable mutations. Fabry disease is a rare
Paulsen, H., et al.
Chemische Berichte, 113, 2601-2601 (1980)