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D9641

Sigma-Aldrich

Deoxygalactonojirimycin hydrochloride

powder or solid, ≥98% (HPLC)

Sinónimos:

1,5-Dideoxy-1,5-imino-D-galactitol, Migalastat

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About This Item

Fórmula empírica (notación de Hill):
C6H13NO4 · HCl
Número de CAS:
75172-81-5
Peso molecular:
199.63
MDL number:
MFCD00269962
UNSPSC Code:
12352200
PubChem Substance ID:
329798908
NACRES:
NA.32

product name

Deoxygalactonojirimycin hydrochloride,

biological source

synthetic (organic)

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_germany

WGK 3

ppe

dust mask type N95 (US), Eyeshields, Gloves

Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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Visite la Librería de documentos

Paulsen, H., et al.
Chemische Berichte, 113, 2601-2601 (1980)
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
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
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
Antonino Tuttolomondo et al.
Clinical biochemistry, 48(1-2), 55-62 (2014-10-05)
Anderson/Fabry disease expresses a wide range of clinical variability in patients that it is possible to explain referring to a genetic variability with numerous mutations described in the literature (more than 600). We report some clinical cases of some members

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