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About This Item
Empirical Formula (Hill Notation):
C6H13NO4 · HCl
CAS Number:
Molecular Weight:
199.63
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.32
MDL number:
Product Name
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.
Storage Class
11 - Combustible Solids
wgk
WGK 3
ppe
dust mask type N95 (US), Eyeshields, Gloves
Regulatory Information
涉药品监管产品
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Paulsen, H., et al.
Chemische Berichte, 113, 2601-2601 (1980)
Antonino Tuttolomondo et al.
Oncotarget, 8(37), 61415-61424 (2017-10-06)
Anderson-Fabry disease (AFD) is an inborn lysosomal enzymopathy resulting from the deficient or absent activity of the lysosomal exogalactohydrolase, α-galactosidase A. This deficiency, results in the altered metabolism of glycosphingolipids which leads to their accumulation in lysosomes, thus to cellular
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
Global Trade Item Number
| SKU | GTIN |
|---|---|
| D9641-2MG | 04061833270639 |