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

Richard F G Fröhlich; Katrin Fantur; Richard H Furneaux; Eduard Paschke; Arnold E Stütz; Jacqueline Wicki; Stephen G Withers; Tanja M Wrodnigg

doi:10.1016/j.bmcl.2011.09.012

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

Bioorg Med Chem Lett. 2011 Nov 15;21(22):6872-5. doi: 10.1016/j.bmcl.2011.09.012. Epub 2011 Sep 10.

Authors

Richard F G Fröhlich¹, Katrin Fantur, Richard H Furneaux, Eduard Paschke, Arnold E Stütz, Jacqueline Wicki, Stephen G Withers, Tanja M Wrodnigg

Affiliation

¹ Carbohydrate Chemistry Team, Industrial Research Limited, PO Box 31-310, 5040 Lower Hutt, New Zealand.

PMID: 21974950
DOI: 10.1016/j.bmcl.2011.09.012

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.

MeSH terms

Agrobacterium / enzymology
Escherichia coli / enzymology
Fibroblasts / cytology
Fibroblasts / enzymology
Fibroblasts / pathology
Fluorescent Dyes / chemistry*
Galactitol / chemistry*
Gangliosidosis, GM1 / diagnosis*
Gangliosidosis, GM1 / enzymology*
Gangliosidosis, GM1 / genetics
Gangliosidosis, GM1 / pathology
Humans
Lysosomal-Associated Membrane Protein 2 / analysis
Microscopy, Fluorescence
Mutation
beta-Galactosidase / analysis
beta-Galactosidase / genetics
beta-Galactosidase / metabolism*

Substances

Fluorescent Dyes
Lysosomal-Associated Membrane Protein 2
Galactitol
beta-Galactosidase