Iminosugar-Based Galactoside Mimics as Pharmacological Chaperones for Lysosomal b-Galactosidases
Prof. Estelle Galienne Institut de Chimie Organique et Analytique (ICOA), Université d’Orléans
Date: 26.04.2017 Time: 17:00 Place: HS H "Ulrich Santner", NTEG230E, Technische Universität Graz, Kopernikusgasse 24, 8010 Graz, Erdgeschoß
As part of our research program dedicated to the design of new iminosugars as therapeutic agents for lysosomal storage disorders, we synthesized a diversity of iminosugar-based galactoside mimics and investigated them as pharmacological chaperones for mutant forms of lysosomal b-galactosidases, responsible for such devastating diseases as GM1-gangliosidosis, Morquio B and Krabbe diseases. The synthesis and biological evaluation of C-alkylated iminosugars in the l-arabino, d-galacto and l-ribo series will be presented, as well as recent results concerning the synthesis and the remarkable chaperone activity of C-alkylated derivatives of 4-epi-isofagomine.