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D9641

Sigma-Aldrich

Deoxygalactonojirimycin hydrochloride

powder or solid, ≥98% (HPLC)

Synonym(e):

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

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About This Item

Empirische Formel (Hill-System):
C6H13NO4 · HCl
CAS-Nummer:
75172-81-5
Molekulargewicht:
199.63
MDL-Nummer:
MFCD00269962
UNSPSC-Code:
12352200
PubChem Substanz-ID:
329798908
NACRES:
NA.32

product name

Deoxygalactonojirimycin hydrochloride,

Biologische Quelle

synthetic (organic)

Qualitätsniveau

200

Assay

≥98% (HPLC)

Form

powder or solid

Löslichkeit

water: soluble 1 mg/mL

Lagertemp.

−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

InChIKey

ZJIHMALTJRDNQI-OLALXQGDSA-N

Anwendung

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. Wirkung

Deoxygalactonojirimycin hydrochloride is an inhibitor of α-galactosidase A. Deoxygalactonojirimycin exhibits therapeutic effects against Fabry disease.

Lagerklassenschlüssel

11 - Combustible Solids

WGK

WGK 3

Persönliche Schutzausrüstung

dust mask type N95 (US), Eyeshields, Gloves

Analysenzertifikate (COA)

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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)
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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