Shanghai Nianxing Industrial Co., Ltd
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ShangHai DC Chemicals Co.,Ltd
China
Product Name: Amigal
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DC Chemicals Limited
China
Product Name: Amigal
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Dayang Chem (Hangzhou) Co., Ltd.
China
Product Name: DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE
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108147-54-2

108147-54-2 structure

108147-54-2 structure

Name: Amigal
Chemical Name: deoxygalactonojirimycin, hydrochloride
CAS Number: 108147-54-2
Molecular Formula: C₆H₁₃NO₄
Molecular Weight: 163.17200
Catalog: Research Areas Others
Create Date: 2016-11-23 15:28:19
Modify Date: 2024-01-06 17:05:00
Migalastat (GR181413A (free base)) is an orally active and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC50 of 0.04 μM for human α-Gal A[1].

Name	deoxygalactonojirimycin, hydrochloride
Synonyms	capric acid-d19 MIGALASTAT perdeuterodecanoic acid Capric acid-d19,OH (3S,5S,2R,4R)-2-(HYDROXYMETHYL)PIPERIDINE-3,4,5-TRIOL Decanoic-d19 acid decanoic acid-d19

Description	Migalastat (GR181413A (free base)) is an orally active and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC50 of 0.04 μM for human α-Gal A[1].
Related Catalog	Research Areas >> Others Signaling Pathways >> Others >> Others
Target	IC50: 0.04 μM (human α-Gal A)[1]; Ki: 0.04 μM (human α-Gal A)[1]
In Vitro	Both IC50 and Ki values of Migalastat toward human lysosomal a-Gal A are 0.04 μM[1]. Cell Viability Assay[4] Cell Line: EHK cells mutated α-Gal A Concentration: 10 μM Incubation Time: 9 days Result: Reduced Gb3 accumulation and lysosome volume.
In Vivo	Fabry disease is an X-linked recessive disorder caused by the deficient activity of α-galactosidase A[2]. 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)[2]. Migalastat shows the half-life of less than 1 day in all major issues in TgM for 2 weeks pretreatment[2]. 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[3]. 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)[2] Dosage: 3 mg/kg Administration: Oral gavage; every day for 4 weeks Result: Reduced Globotriaosylceramide (Gb3) storage remarkably in kidney of mice.
References	[1]. Asano N, et al. In vitro 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. [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. [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. [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.

Density	1.456g/cm3
Boiling Point	361.1ºC at 760 mmHg
Molecular Formula	C₆H₁₃NO₄
Molecular Weight	163.17200
Flash Point	197.3ºC
Exact Mass	163.08400
PSA	92.95000
Vapour Pressure	1.13E-06mmHg at 25°C
Index of Refraction	1.582

Hazard Codes	Xi
Risk Phrases	R36/37/38
Safety Phrases	S26-S36