1-Deoxygalactonojirimycin (hydrochloride)
mRNA synthesis
In vitro transcription of capped mRNA with modified nucleotides and Poly(A) tail
Tyramide Signal Amplification (TSA)
TSA (Tyramide Signal Amplification), used for signal amplification of ISH, IHC and IC etc.
Phos Binding Reagent Acrylamide
Separation of phosphorylated and non-phosphorylated proteins without phospho-specific antibody
Cell Counting Kit-8 (CCK-8)
A convenient and sensitive way for cell proliferation assay and cytotoxicity assay
SYBR Safe DNA Gel Stain
Safe and sensitive stain for visualization of DNA or RNA in agarose or acrylamide gels.
Inhibitor Cocktails
Protect the integrity of proteins from multiple proteases and phosphatases for different applications.
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].
References:
[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.
Physical Appearance | A crystalline solid |
Storage | Store at -20°C |
M.Wt | 199.6 |
Cas No. | 75172-81-5 |
Formula | C6H13NO4·HCl |
Solubility | ≤10mg/ml in DMSO |
Chemical Name | (2R,3S,4R,5S)-2-(hydroxymethyl)-3,4,5-piperidinetriol, monohydrochloride |
SDF | Download SDF |
Canonical SMILES | O[C@@H]1[C@@H](O)[C@@H](O)[C@@H](CO)NC1.Cl |
运输条件 | 蓝冰运输或根据您的需求运输。 |
一般建议 | 不同厂家不同批次产品溶解度各有差异,仅做参考。若实验所需浓度过大至产品溶解极限,请添加助溶剂助溶或自行调整浓度。溶液形式一般不宜长期储存,请尽快用完。 |