A fluorescent probe for GM1 gangliosidosis related β-galactosidase: N-(Dansylamino)hexylaminocarbonylpentyl-1,5-dideoxy-1,5-imino- d-galactitol

Biological evaluation of N-(dansylamino)hexylaminocarbonylpentyl-1,5-dideoxy-1,5-imino- d-galactitol on residual β-galactosidase activities in fibroblasts of GM1 gangliosidosis patient cells and co-localisation experiments and lysosome associated membrane protein 2 in healthy skin fibroblast cells are reported. N-(Dansylamino)hexylaminocarbonylpentyl-1,5-dideoxy-1,5-imino- d-galactitol, a strong competitive inhibitor of β-galactosidase, enhances residual β-galactosidase activities in fibroblasts and serves as lead en route to diagnostic compounds for tracking the fate of mutant β-gal as well as aberrant GM1 gangliosides by live cell imaging.