推荐产品
product name
Deoxygalactonojirimycin hydrochloride,
生物源
synthetic (organic)
化驗
≥98% (HPLC)
形狀
powder or solid
溶解度
water: soluble 1 mg/mL
儲存溫度
−20°C
SMILES 字串
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 密鑰
ZJIHMALTJRDNQI-OLALXQGDSA-N
應用
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.
生化／生理作用
Deoxygalactonojirimycin hydrochloride is an inhibitor of α-galactosidase A. Deoxygalactonojirimycin exhibits therapeutic effects against Fabry disease.
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
個人防護裝備
dust mask type N95 (US), Eyeshields, Gloves
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)
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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