Migalastat hydrochloride is a pharmacological chaperone that acts as a selective and competitive inhibitor of α-galactosidase (IC₅₀ = 40 nM). At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to α-galactosidase and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes.