机译:The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms
Amicus Therapeut, Cranbury, NJ 08512 USA;
Washington Univ, Sch Med, Dept Med, St Louis, MO 63110 USA;
lysosome; pharmacological chaperone; lysosomal storage disease; CHEMICAL CHAPERONE THERAPY; LYSOSOMAL GLUCOCEREBROSIDASE; STORAGE DISORDERS; DISEASE MUTATIONS; MIGLUSTAT; CELLS; TRAFFICKING; TYPE-1; ENZYME; MATURATION;