Alpha-galactosidase A


  • Accession: P06280
  • Swissprot: AGAL_HUMAN
  • Organism: Homo sapiens
  • Gene: GLA
  • Target class: Enzyme

Drug Relations:

Migalastat is a pharmacological chaperone that is designed to selectively and reversibly bind with high affinity to the active sites of certain mutant forms of alpha-Gal A, the genotypes of which are referred to as amenable mutations. Migalastat binding stabilizes these mutant forms of alpha-Gal A in the endoplasmic reticulum and facilitates their proper trafficking to lysosomes where dissociation of migalastat restores alpha-Gal A activity, leading to the catabolism of GL-3 and related substrates. Bioactivity details MOA