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SHR Neuro Krebs Kardio Lipid

Schalli, M; Tysoe, C; Fischer, R; Pabst, BM; Thonhofer, M; Paschke, E; Rappitsch, T; Stütz, AE; Tschernutter, M; Windischhofer, W; Withers, SG.
N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols: A new family of activity promotors for a GM1-gangliosidosis related human lysosomal β-galactosidase mutant.
Carbohydr Res. 2017; 443-444(2):15-22
Web of Science PubMed FullText FullText_MUG

 

Autor/innen der Med Uni Graz:
Pabst Bettina
Paschke Eduard
Tschernutter Marion
Windischhofer Werner
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Abstract:
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 β-galactosidase mutant R201C, thus representing a new structural type of pharmacological chaperones for this lysosomal storage disease. Copyright © 2017 Elsevier Ltd. All rights reserved.
Find related publications in this database (using NLM MeSH Indexing)
Cyclopentanes - chemical synthesis
Cyclopentanes - chemistry
Cyclopentanes - pharmacology
Enzyme Inhibitors - chemical synthesis
Enzyme Inhibitors - chemistry
Enzyme Inhibitors - pharmacology
Gangliosidosis, GM1 - enzymology
Gangliosidosis, GM1 - genetics
Humans -
Models, Molecular -
Molecular Conformation -
Mutation -
beta-Galactosidase - antagonists & inhibitors
beta-Galactosidase - genetics

Find related publications in this database (Keywords)
Aminocyclopentane
Carbafuranose
Galactosidase inhibitor
Pharmacological chaperone
G(M1)-Gangliosidosis
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