... (causing anotherlysosomal storage disease) were shown to be foldingand trafficking mutants [16] before this was exploredas a possibility in GD. Galactose administrationincreased Q279E a- galactosidase ... a- galactosidase A residual activity in patient derived cells; thus, galactose was demonstrated to be first active-site-directed pharmacologic chaperonefor a lysosomal storage disease. Galactose administra-tion ... 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...