Article
N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols: A new family of activity promotors for a GM1-gangliosidosis related human lysosomal β-galactosidase mutant.
Carbohydrate research - 18 Apr 2017
Schalli Michael, Tysoe Christina, Fischer Roland, Pabst Bettina M, Thonhofer Martin, Paschke Eduard, Rappitsch Tanja, Stütz Arnold E, Tschernutter Marion, Windischhofer Werner, Withers Stephen G
Abstract excerpt
From 1,2;3,4-di-O-isopropylidene-α-D-galactopyranose, a series of highly functionalized (hydroxymethyl)cyclopentanes was easily available. In line with reports by Reymond and Jäger on similar structures, these amine containing basic carbasugars are potent inhibitors of β-D-galactosidases and, for the first time, could be shown to act as pharmacological chaperones for GM1-gangliosidosis-associated lysosomal acid...
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