Synthesis of C-5a-substituted derivatives of 4-epi-isofagomine: notable β-galactosidase inhibitors and activity promotors of GM1-gangliosidosis related human lysosomal β-galactosidase mutant R201C.

Martin Thonhofer, Patrick Weber, Andres Gonzalez Santana, Christina Tysoe, Roland Fischer, Bettina M. Pabst, Eduard Paschke, Michael Schalli, Arnold Stütz, Marion Tschernutter, Werner Windischhofer, Stephen G. Withers

Publikation: Beitrag in einer FachzeitschriftArtikel


From an easily available partially protected analog of 1-deoxy-L-gulo-nojirimycin, by chain-branching at C-4 and suitable modification, lipophilic analogs of the powerful β-D-galactosidase inhibitor 4-epi-isofagomine have been prepd. New compds. exhibit considerably improved inhibitory activities when compared with the unsubstituted parent compd. and may serve as leads toward new pharmacol. chaperones for GM1-gangliosidosis and Morquio B disease. [on SciFinder(R)]
Seiten (von - bis)71-80
FachzeitschriftCarbohydrate Research
PublikationsstatusVeröffentlicht - 2016


  • deoxygulonojirimycin galactosidase inhibitor isofagomine synthesis Morquio imino alditol
  • human lysosomal galactosidase inhibitor gangliosidosis isofagomine synthesis

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