title: A fluorescent probe for GM1 gangliosidosis related β-galactosidase: N-(dansylamino)hexylaminocarbonylpentyl-1,5-dideoxy-1,5-imino-D-galactitol.
abstract: 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.
authors.affiliation: Carbohydrate Chemistry Team, Industrial Research Limited, PO Box 31-310, 5040 Lower Hutt, New Zealand.
authors.forename: Richard F G
authors.initials: RF
authors.lastname: Fröhlich
authors.forename: Katrin
authors.initials: K
authors.lastname: Fantur
authors.forename: Richard H
authors.initials: RH
authors.lastname: Furneaux
authors.forename: Eduard
authors.initials: E
authors.lastname: Paschke
authors.forename: Arnold E
authors.initials: AE
authors.lastname: Stütz
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Wicki
authors.forename: Stephen G
authors.initials: SG
authors.lastname: Withers
authors.forename: Tanja M
authors.initials: TM
authors.lastname: Wrodnigg
mesh_terms: D060054:Agrobacterium / Q000201:enzymology; D004926:Escherichia coli / Q000201:enzymology; D005347:Fibroblasts / Q000166:cytology / Q000201:enzymology / Q000473:pathology; D005456:Fluorescent Dyes / Q000737:chemistry*; D004376:Galactitol / Q000737:chemistry*; D016537:Gangliosidosis, GM1 / Q000175:diagnosis* / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D006801:Humans; D052119:Lysosomal-Associated Membrane Protein 2 / Q000032:analysis; D008856:Microscopy, Fluorescence; D009154:Mutation; D001616:beta-Galactosidase / Q000032:analysis / Q000235:genetics / Q000378:metabolism*
chemical_list: D005456:Fluorescent Dyes; D052119:Lysosomal-Associated Membrane Protein 2; D004376:Galactitol; D001616:beta-Galactosidase