Figure 2
Effect of 1-deoxy-galactonojirimycin on various mutant of alpha-galactosidase expressed in COS-7 cells. Western blot analysis. Cells were cultured in the presence (+) or absence (-) of 20 uM 1-deoxy-galactonojirimycin for 2 days prior to western blot analysis. Panel A: WT-, COS-7 transfected with wild type AGAL (20 micrograms of cell extract); WT+, COS-7 transfected with wild type AGAL and treated with 20 micromolar 1-deoxy-galactonojirimycin (15 micrograms of cell extract); preWT/COS, COS-7 tranfected with void vector (20 micrograms) plus alpha-galactosidase precursor form (Fabrazyme® 20 nanograms), preWT, alpha-galactosidase precursor form (Fabrazyme® 20 nanograms); M, marker;. Panel B,C,D: COS-7 transfected with void vector (COS7), wild-type (WT) or mutant alpha-galactosidase vectors (10 micrograms) not treated (-) or treated with 1-deoxy-galactonojirimycin (+).