Migalastathydrochloride , 98% , 75172-81-5
Synonym(s):
1,5-Dideoxy-1,5-imino-D -galactitol;Deoxygalactonojirimycin, Hydrochloride - CAS 75172-81-5 - Calbiochem;DGJ, 1,5-Dideoxy-1,5-imino-D-galactitol, Galactostatin, HCl;Migalastat
Pack Size | Price | Stock | Quantity |
10mg | RMB3165.60 | In Stock |
|
others | Enquire |
PRODUCT Properties
Melting point: | 260 |
storage temp. | 2-8°C |
solubility | Methanol (Slightly), Water (Slightly) |
form | White crystalline solid |
color | White to Brown |
Description and Uses
Migalastat, which is marketed by Amicus Therapeutics, received approval in the EU for the treatment of Fabry disease in adults and adolescents aged 16 or older. Fabry disease is caused by mutations of the enzyme α-galactosidase A (α-GAL A) that cause protein misfolding and prevents efficient metabolism of the glycosphingolipid globotriaosylceramide (GL3). Accumulation of GL3 in lysosomes, blood vessels, and various tissues ultimately leads to significant heart, kidney, and dermatological problems. Migalastat functions as a molecular chaperone to α- GAL A, engaging the enzyme and enabling it to adopt the proper conformation allowing for efficient breakdown of GL3. Because the standard of care prior to 2016 for treating Fabry disease was enzyme replacement therapy (ERT), migalastat’s approval in the EU represents an important advance for patients suffering from this disorder.
Deoxygalactonojirimycin hydrochloride has been used as an α-galactosidase A inhibitor to assess the enzymatic activity of α-galactosidase A. It has also been used as an α-galactosidase A inhibitor to study its effects on the mRNA levels in human embryonic kidney (HEK) cells and hippocampal neurons.
Safety
Symbol(GHS) | GHS07 |
Signal word | Warning |
Hazard statements | H302-H315-H319-H335 |
Precautionary statements | P261-P280-P301+P312-P302+P352-P305+P351+P338 |
Hazard Codes | Xi |
Risk Statements | 36/37/38 |
Safety Statements | 26-36 |
WGK Germany | 3 |
Hazard Note | Irritant |
HS Code | 29333990 |