추천 제품
product name
Deoxygalactonojirimycin hydrochloride,
생물학적 소스
synthetic (organic)
Quality Level
분석
≥98% (HPLC)
형태
powder or solid
solubility
water: soluble 1 mg/mL
저장 온도
−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
애플리케이션
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.
생화학적/생리학적 작용
Deoxygalactonojirimycin hydrochloride is an inhibitor of α-galactosidase A. Deoxygalactonojirimycin exhibits therapeutic effects against Fabry disease.
Storage Class Code
11 - Combustible Solids
WGK
WGK 3
개인 보호 장비
dust mask type N95 (US), Eyeshields, Gloves
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
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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