DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE

Product Name: DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE
CAS No.: 108147-54-2
Chemical Name: DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE
Synonyms: MIGALASTAT;(2R,3S,4R,5S)-2-(hydroxymethyl)piperidine-3,4,5-triol;DGJ;Migalasta;GALACTOSTATIN HCL;DGJ, HYDROCHLORIDE;1-Deoxygalactonojirimycin;DEOXYGALACTONOJIRIMYCIN HCL;1-DEOXYGALACTONOJIRIMYCIN HCL;1,5-dideoxy-1,5-iminogalactitol
CBNumber: CB2252940
Molecular Formula: C6H13NO4
Formula Weight: 163.17
MOL File: 108147-54-2.mol

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DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE Property

Melting point:: 243-245 °C
Boiling point:: 361.1±42.0 °C(Predicted)
Density: 1.456±0.06 g/cm3(Predicted)
storage temp.: 2-8°C
pka: 13.77±0.70(Predicted)

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Safety

Hazard Codes: Xi
Risk Statements: 36/37/38
Safety Statements: 26-36
WGK Germany: 3

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Hazard and Precautionary Statements (GHS)

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N-Bromosuccinimide Price

Usbiological

Product number: 293236
Product name: 1-Deoxygalactonojirimycin
Packaging: 10g
Price: $36136
Updated: 2021/12/16

Medical Isotopes, Inc.

Product number: 8856
Product name: Deoxygalactonojirimycin, Hydrochloride
Packaging: 1mg
Price: $90
Updated: 2021/12/16

American Custom Chemicals Corporation

Product number: API0008119
Product name: MIGALASTAT
Purity: 95.00%
Packaging: 5MG
Price: $499.83
Updated: 2021/12/16

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DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE Chemical Properties,Usage,Production

Uses

Migalastat (GR181413A free base) is an orally active α-galactosidase A molecular chaperone, with an IC50 value of 0.04 μM for human α-Gal A. Migalastat binds to the active site of certain unstable mutant forms of α-galactosidase A, facilitating their transport to the lysosome. After dissociation in the acidic environment, Migalastat enables the mutant α-galactosidase A to exhibit biological activity[1].

Definition

ChEBI: Migalastat is a member of piperidines.

brand name

Treatment of Fabry disease.

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. DOI:10.1046/j.1432-1327.2000.01457.x
[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. DOI:10.1124/jpet.108.149054
[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. DOI:10.1371/journal.pone.0057631
[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. DOI:10.1093/hmg/ddy248

DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE Preparation Products And Raw materials

Raw materials

Preparation Products

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DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE Suppliers

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View Lastest Price from DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE manufacturers

Career Henan Chemical Co

Product: DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE 108147-54-2
Price: US $1.00/KG
Min. Order: 1KG
Purity: 98
Supply Ability: 200kg
Release date: 2019-09-10

108147-54-2, DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDERelated Search:

(R)-3-Hydroxypiperidine hydrocloride GALACTOSTATIN DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE cis-3,4-Dihydroxypiperidine trans-3,4-Dihydroxypiperidine DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE

GALACTOSTATIN HCL

DGJ

DGJ, HYDROCHLORIDE

DEOXYGALACTONOJIRIMYCIN HCL

DEOXYGALACTONOJIRIMYCIN, HYDROCHLORIDE

1-DEOXYGALACTONOJIRIMYCIN HCL

1,5-DIDEOXY-1,5-IMINO-D-GALACTITOL, HYDROCHLORIDE

3,4,5-Piperidinetriol, 2-(hydroxymethyl)-, (2R,3S,4R,5S)-

1,5-dideoxy-1,5-iminogalactitol

MIGALASTAT

(2R,3S,4R,5S)-2-(hydroxymethyl)piperidine-3,4,5-triol

Migalasta

1-Deoxygalactonojirimycin

(2R,3S,4R,5S)-2-(Hydroxymethyl)-3,4,5-piperidinetriol

108147-54-2

Biochemicals and Reagents

BioChemical

Galactosidase, alpha-

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