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1-Deoxygalactonojirimycin(hydrochloride)
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
1-Deoxygalactonojirimycin(hydrochloride)图片
CAS NO:75172-81-5
规格:98%
分子量:199.6
包装与价格:
包装价格(元)
1mg电议
5mg电议
10mg电议

产品介绍
α-galactosidase inhibitor
CAS:75172-81-5
分子式:C6H13NO4 ? HCl
分子量:199.6
纯度:98%
存储:Store at -20°C

Background:

Target: α-galactosidase


IC50: 40 nM


1-Deoxygalactonojirimycin (hydrochloride) is a competitive inhibitor of α-galactosidase, with the IC50 value of 40 nM [1]. 1-Deoxygalactonojirimycin (hydrochloride) is used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease. Fabry disease is a rare genetic lysosomal storage disease, inherited in an X-linked manner which is caused by mutations in the α-galactosidase gene that commonly lead to enzyme instability, misfolding, and degradation.


In Vivo: In transgenic mice expressed human mutant α-galactosidase A, treatment with 1-Deoxygalactonojirimycin at a dosage of approximately 3 mg/kg body weight/day could significantly reduce globotriaosylceramide storage in the kidney. Besides, no abnormality of blood chemistry and pathological tissue damage was found in mice treated with 1-Deoxygalactonojirimycin at about 30 mg/kg body weight/day for 9 weeks [2].


参考文献:
[1] Asano N, Ishii S, Kizu H, et al.  In vitro inhibition and intracellular enhancement of lysosomal α‐galactosidase A activity in Fabry lymphoblasts by 1‐deoxygalactonojirimycin and its derivatives[J]. FEBS Journal, 2000, 267(13): 4179-4186.
[2] Ishii S, Chang H, Yoshioka H, et al.  Preclinical Efficacy and Safety of 1-Deoxygalactonojirimycin in Mice for Fabry Disease[J]. Journal of Pharmacology and Experimental Therapeutics, 2009, 328(3): 723-731.