Medchemexpress LLC Migalastat hydrochloride (GR181413A) | 75172-81-5 | 99.7% | 199.63 | 100 MG

Supplier:  Medchemexpress LLC 100MGHY14929A

Migalastat hydrochloride (GR181413A) is an orally active α-galactosidase A molecular chaperone. It functions by binding to the active site of certain unstable mutant forms of α-galactosidase A, which facilitates their transport to the lysosome. Once in the acidic environment of the lysosome, Migalastat dissociates, allowing the mutant α-galactosidase A to exhibit biological activity.

• Orally active α-galactosidase A molecular chaperone
• IC50 value of 0.04 μM for human α-Gal A
• Facilitates transport of unstable mutant α-galactosidase A to the lysosome
• Enables mutant α-galactosidase A to exhibit biological activity
• Reduces Gb3 accumulation and lysosome volume in EHK cells mutated α-Gal A
• Increases α-Gal A activity in heart, kidney, spleen, and liver in transgenic mice
• Reduces lyso-Gb3 levels in kidney, heart, and skin in transgenic mice

Catalog No. 50-003-64798