Takeuchi, H.

nb-dnj activity disease y 455f glu activity chaperone lysosomal chemical patient enzyme replacement therapy evect therapy enzyme p 545l glucosidase protein concentration gsdii medium chemical chaperone therapy van der ploeg cos -7 cells 4 days wbroblast transport cell-line derivative storage glu species mutation compound glycogenosis type ii precursor lysosomal storage disorders reuser fabry disease fabry glycogen inhibitor transfected culture medium acid maltase dewciency a.j.j presence replacement brefeldin y 455f glu okumiya dewciency inhibition galactosidase cell homogenates species metabolism genetic chemical chaperone gsdii patients gaucher wild-type study nn-dnj lysosome biochem deoxynojirimycin absence rabbit polyclonal antibodies control 49–57 nd-dnj increase culture disorder 10 mol /l maturation butter 4- methylumbelliferyl pompe function glu cdna homogenate