| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 1370273 | Bioorganic & Medicinal Chemistry Letters | 2011 | 4 Pages |
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.
Graphical abstractBiological 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.Figure optionsDownload full-size imageDownload as PowerPoint slide
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Authors
Richard F.G. Fröhlich, Katrin Fantur, Richard H. Furneaux, Eduard Paschke, Arnold E. Stütz, Jacqueline Wicki, Stephen G. Withers, Tanja M. Wrodnigg,