... concentration of partially active GC vari-ants, as demonstrated by increased cellular GC activity,an increased concentration of lysosomal GC glyco-forms and increased colocalization of GC with ... mechanisms.Proc Natl Acad Sci USA 103, 13813–13818.29 Yu L, Ikeda K, Kato A, Adachi I, Godin G, CompainP, Martin O & Asano N (2006) alpha-1-C-octyl-1-de-oxynojirimycin as a pharmacological chaperone ... lysosomalconcentration, in which case new dosing and washoutregimens may be useful in restoring partial L444P GCactivity.Lastly, in contrast to ERT and like substrate reduc-tion therapy, a pharmacologic...