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1-Deoxygalactonojirimycin (hydrochloride)

α-半乳糖苷酶抑制剂，Migalastat hydrochloride (1-Deoxygalactonojirimycin hydrochloride)是一种有效和竞争性的α-galactosidase A抑制剂，对人α-Gal A的 IC50值为0.04 μM。

目录号: PC12696 纯度: ≥98%

CAS No. :75172-81-5

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中文名称	1-Deoxygalactonojirimycin (hydrochloride)
中文别名	1-脱氧半乳糖野尻霉素盐酸盐;脱氧半乳糖野尻霉素(DGI);脱氧半乳糖野生霉素盐酸盐
英文名称	1-Deoxygalactonojirimycin (hydrochloride)
英文别名	3,4,5-Piperidinetriol,2-(hydroxymethyl)-, hydrochloride (1:1), (2R,3S,4R,5S)-;1-Deoxygalactonojirimycin Hydrochloride;Deoxygalactonojirimycin Hydrochloride;(+)-(2R,3R,4R,5S)-2-hydroxymethyl-3,4,5-trihydroxypiperidine hydrochloride;(+)-1-deoxynojirimycin hydrochloride;(1R,3R,4R,5S)-3,4,5-trihydroxy-2-(hydroxymethyl)piperidinium chloride;(2R,3S,4R,5S)-2-(Hydroxymethyl)piperidine-3,4,5-triol hydrochloride;1,5-dideoxy-1,5-imino-D-glucitol hydrochloride;1-DEOXYMANNOJIRIMYCIN HCL;1-DeoxymannojirimycineHCl;1-Deoxymannojirimycinehydrochloride;DEOXYMANNOJIRIMYCIN HCL;DEOXYMANNOJIRIMYCIN,HYDROCHLORIDE;DEOXYMANNONOJIRIMYCIN HCL;DMJ,HYDROCHLORIDE;DMM
Cas No.	75172-81-5
分子式	C₆H₁₃NO₄^.HCl
分子量	199.63
包装储存	4°C, sealed storage, away from moisture *In solvent : -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture)

生物活性

Migalastat hydrochloride (GR181413A) is a potent and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC₅₀ of 0.04 μM for human α-Gal A.

性状

Solid

IC50 & Target[1][2]

IC50: 0.04 μM (human α-Gal A); Ki: 0.04 μM (human α-Gal A)

体外研究(In Vitro)

Both IC₅₀ and K_i values of Migalastat hydrochloride (GR181413A) toward human lysosomal a-Gal A are 0.04 μM.

Medlife has not independently confirmed the accuracy of these methods. They are for reference only.

Cell Viability Assay

Cell Line:	EHK cells mutated α-Gal A
Concentration:	10 μM
Incubation Time:	9 days
Result:	Reduced Gb3 accumulation and lysosome volume.

体内研究(In Vivo)

Fabry disease is an X-linked recessive disorder caused by the deficient activity of α-galactosidase A.
Migalastat (oral gavage, 3 mg/kg daily for 4 weeks) increases α-Gal A activity in heart, kidney, spleen, and liver in a dose- and time-dependently in transgenic mice that express human mutant alpha-Gal A (TgM).
Migalastat shows the half-life of less than 1 day in all major issues in TgM for 2 weeks pretreatment.
Migalastat (oral gavage, 100 mg/kg daily for 28 days) to transgenic mice reduces lyso-Gb3 levels up to 64%, 59%, and 81% in kidney, heart, and skin, respectively.

Medlife has not independently confirmed the accuracy of these methods. They are for reference only.

Animal Model:	Male nontransgenic (Non-Tg) C57BL/6 mice; transgenic mice expressing human mutant R301Q α-Gal A (TgM), α-Gal A knockout mice (KO), mice express human R301Q α-Gal A in a null background (TgM/KO)
Dosage:	3 mg/kg
Administration:	Oral gavage; every day for 4 weeks
Result:	Reduced Globotriaosylceramide (Gb3) storage remarkably in kidney of mice.

运输条件

Room temperature or refrigerated transportation.

储存方式

4°C, sealed storage, away from moisture

*In solvent : -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture)

ClinicalTrial

参考文献

[1]. Asano N, et al. 体外研究 inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives. Eur J Biochem. 2000 Jul;267(13):4179-86. [Information]

[2]. Ishii S, et al. Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease. J Pharmacol Exp Ther. 2009 Mar;328(3):723-31. [Information]

[3]. Young-Gqamana B, et al. Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients. PLoS One. 2013;8(3):e57631. [Information]

[4]. Welford RWD, et al. Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types. Hum Mol Genet. 2018 Oct. 27(19):3392-3403. [Information]

溶解度数据

体外研究:

H₂O : ≥ 200 mg/mL (1001.85 mM)

* "≥" means soluble, but saturation unknown.

配制储备溶液

浓度溶剂体积质量	1 mg	5 mg	10 mg

1 mM	5.0093 mL	25.0463 mL	50.0927 mL
5 mM	1.0019 mL	5.0093 mL	10.0185 mL
10 mM	0.5009 mL	2.5046 mL	5.0093 mL

产品不同，其溶解度不同。建议根据产品选择合适的溶剂配制储备溶液；配成溶液后，建议分装保存，避免反复冻融造成的产品失效。
储备液的保存方式和期限：-80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture)。-80°C 储存时，建议在 6 个月内使用，-20°C 储存时，建议在 1 个月内使用。

体内研究:

建议根据您的实验动物和给药方式选择适当的溶解方案。以下溶解方案都建议先按照 体外研究 方式配制澄清的储备液，再依次添加助溶剂：

——为保证实验结果的可靠性，澄清的储备液可以根据储存条件，适当保存；体内实验的工作液，建议您现用现配，当天使用；以下溶剂前显示的百
分比是指该溶剂在您配制终溶液中的体积占比；如在配制过程中出现沉淀、析出现象，可以通过加热和/或超声的方式助溶

1.

建议依照次序添加每种溶剂： PBS
Solubility: 100 mg/mL (500.93 mM); Clear solution; Need ultrasonic

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产品使用指南

Data Sheet

1：一般建议：溶解度为Medlife测试数据，可能与文献描述存在差异。这是由于生产工艺和批次不同产生的正常现象。为了使其更好的溶解，请用37℃加热试管并在超声波水浴中震动片刻。不同批次产品溶解度各有差异，仅做参考，具体以实验方案为准。

2：储存条件：粉末-20°C一般情况可以保存3年，溶于溶剂-80°C一般情况可以保存1年。不同产品及不同批次产品可能存在差异，请细致阅读产品信息，并辅助参考相关文献描述。

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