2. Metabolic Enzyme/Protease
  3. Glycosidase
  4. Migalastat hydrochloride

Migalastat (hydrochloride)  (Synonyms: GR181413A)

製品番号: HY-14929A Assay: 99.70%
COA 取扱説明書 Technical Support

Migalastat (GR181413A free base) hydrochloride 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.

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Migalastat hydrochloride

Migalastat (hydrochloride) 構造式

CAS 番号 : 75172-81-5

容量 価格（税別） 在庫状況 数量
Solid + Solvent (Highly Recommended)
10 mM * 1 mL in Water
ready for reconstitution
 USD 82 在庫あり
Solution
10 mM * 1 mL in Water USD 82 在庫あり
Solid
5 mg $75 在庫あり
10 mg $115 在庫あり
25 mg $230 在庫あり
50 mg $370 在庫あり
100 mg $600 在庫あり
250 mg $1200 在庫あり
500 mg   お問い合わせ  
1 g   お問い合わせ  

* アイテムを追加する前、数量をご選択ください

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Based on 1 publication(s) in Google Scholar

Other Forms of Migalastat hydrochloride:

Migalastat (hydrochloride) 関連抗体

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製品説明

Migalastat (GR181413A free base) hydrochloride 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].

IC50 & Target

IC50: 0.04 μM (human α-Gal A)[1]; Ki: 0.04 μM (human α-Gal A)[1]
体外実験

Both IC50 and Ki values of Migalastat hydrochloride (GR181413A) toward human lysosomal a-Gal A are 0.04 μM[1].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

Migalastat (hydrochloride) 関連抗体

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.
体内実験

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].

MedChemExpress (MCE) 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)[2]
Dosage: 3 mg/kg
Administration: Oral gavage; every day for 4 weeks
Result: Reduced Globotriaosylceramide (Gb3) storage remarkably in kidney of mice.
分子量

199.63

分子式

C6H14ClNO4
CAS 番号
Appearance

Solid

Color

White to light brown

SMILES

O[C@H]1[C@@H](CO)NC[C@H](O)[C@H]1O.Cl
輸送条件

Room temperature in continental US; may vary elsewhere.
保管条件

4°C, sealed storage, away from moisture

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

H2O : ≥ 200 mg/mL (1001.85 mM)

*"≥" means soluble, but saturation unknown.

Preparing
Stock Solutions
Concentration Solvent Mass 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
View the Complete Stock Solution Preparation Table

* Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
Storage method and period of stock solution: -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture). When stored at -80°C, please use it within 6 months. When stored at -20°C, please use it within 1 month.

* Note: If you choose water as the stock solution, please dilute it to the working solution, then filter and sterilize it with a 0.22 μm filter before use.

  • Molarity Calculator

  • Dilution Calculator

Mass (g) = Concentration (mol/L) × Volume (L) × Molecular Weight (g/mol)

Mass
=
Concentration
×
Volume
×
Molecular Weight *

Concentration (start) × Volume (start) = Concentration (final) × Volume (final)

一般には略語で表示されます：C1V1 = C2V2

濃度 （開始）

C1

×
体積 （開始）

V1

=
濃度 （終了）

C2

×
体積 （終了）

V2

体内:

For the following dissolution methods, please prepare the working solution directly. It is recommended to prepare fresh solutions and use them promptly within a short period of time.
The percentages shown for the solvents indicate their volumetric ratio in the final prepared solution. If precipitation or phase separation occurs during preparation, heat and/or sonication can be used to aid dissolution.

  • Protocol 1

    Add each solvent one by one:  PBS

    Solubility: 100 mg/mL (500.93 mM); Clear solution; Need ultrasonic

In Vivo Dissolution Calculator
Please enter the basic information of animal experiments:

Dosage

mg/kg

Animal weight
(per animal)

g

Dosing volume
(per animal)

μL

Number of animals

Recommended: Prepare an additional quantity of animals to account for potential losses during experiments.
Calculation results:
Working solution concentration: mg/mL
This product has good water solubility, please refer to the measured solubility data in water/PBS/Saline for details.
The concentration of the stock solution you require exceeds the measured solubility. The following solution is for reference only.If necessary, please contact MedChemExpress (MCE).
純度とドキュメンテーション

純度: 99.70%

COA (250 KB) HNMR (521 KB)

取扱説明書 (2659 KB)
参考文献

Complete Stock Solution Preparation Table

* Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
Storage method and period of stock solution: -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture). When stored at -80°C, please use it within 6 months. When stored at -20°C, please use it within 1 month.

Optional Solvent Concentration Solvent Mass 1 mg 5 mg 10 mg 25 mg
H2O 1 mM 5.0093 mL 25.0463 mL 50.0927 mL 125.2317 mL
5 mM 1.0019 mL 5.0093 mL 10.0185 mL 25.0463 mL
10 mM 0.5009 mL 2.5046 mL 5.0093 mL 12.5232 mL
15 mM 0.3340 mL 1.6698 mL 3.3395 mL 8.3488 mL
20 mM 0.2505 mL 1.2523 mL 2.5046 mL 6.2616 mL
25 mM 0.2004 mL 1.0019 mL 2.0037 mL 5.0093 mL
30 mM 0.1670 mL 0.8349 mL 1.6698 mL 4.1744 mL
40 mM 0.1252 mL 0.6262 mL 1.2523 mL 3.1308 mL
50 mM 0.1002 mL 0.5009 mL 1.0019 mL 2.5046 mL
60 mM 0.0835 mL 0.4174 mL 0.8349 mL 2.0872 mL
80 mM 0.0626 mL 0.3131 mL 0.6262 mL 1.5654 mL
100 mM 0.0501 mL 0.2505 mL 0.5009 mL 1.2523 mL

* Note: If you choose water as the stock solution, please dilute it to the working solution, then filter and sterilize it with a 0.22 μm filter before use.

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Migalastat hydrochloride Related Classifications

  • Molarity Calculator

  • Dilution Calculator

The molarity calculator equation

Mass (g) = Concentration (mol/L) × Volume (L) × Molecular Weight (g/mol)

質量   濃度   体積   分子量 *
= × ×

The dilution calculator equation

Concentration (start) × Volume (start) = Concentration (final) × Volume (final)

一般には略語で表示されます：C1V1 = C2V2

濃度 （開始） × 体積 （開始） = 濃度 （終了） × 体積 （終了）
× = ×
C1   V1   C2   V2
Help & FAQs
  • Do most proteins show cross-species activity?

    Species cross-reactivity must be investigated individually for each product. Many human cytokines will produce a nice response in mouse cell lines, and many mouse proteins will show activity on human cells. Other proteins may have a lower specific activity when used in the opposite species.

Keywords:

Migalastat75172-81-5GR181413AGlycosidaseFabry diseaseorally activeα-galactosidase Aα-Gal AglobotriaosylceramideGb3globotriaosylsphingosinelyso-Gb1-deoxygalactonojirimycinDGJInhibitorinhibitorinhibit

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