** = Publikationen gelistet in SCI/SSCI/Pubmed
** Weber, P; Fischer, R; Nasseri, SA; Pabst, BM; Prasch, H; Stuetz, AE; Thonhofer, M; Withers, SG; Windischhofer, W; Wrodnigg, TM
N-Methyl-N-Alkylaminocyclopentanes: Powerful and Selective β-
HELV CHIM ACTA. 2024;
Doi: 10.1002/hlca.202300219
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** Weber, P; Thonhofer, M; Averill, S; Davies, GJ; Santana, AG; Müller, P; Nasseri, SA; Offen, WA; Pabst, BM; Paschke, E; Schalli, M; Torvisco, A; Tschernutter, M; Tysoe, C; Windischhofer, W; Withers, SG; Wolfsgruber, A; Wrodnigg, TM; Stütz, AE
Mechanistic Insights into the Chaperoning of Human Lysosomal-Galactosidase Activity: Highly Functionalized Aminocyclopentanes and C-5a-Substituted Derivatives of 4-epi-Isofagomine.
Molecules. 2020; 25(17):
Doi: 10.3390/molecules25174025
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** Weber, P; Nasseri, SA; Pabst, BM; Torvisco, A; Müller, P; Paschke, E; Tschernutter, M; Windischhofer, W; Withers, SG; Wrodnigg, TM; Stütz, AE
Potent GH20 N-Acetyl-β-d-hexosaminidase Inhibitors: N-Substituted 3-acetamido-4-amino-5-hydroxymethyl-cyclopentanediols.
Molecules. 2018; 23(3): 708
Doi: 10.3390/molecules23030708
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** Lebl, R; Thonhofer, M; Tysoe, C; Pabst, BM; Schalli, M; Weber, P; Paschke, E; Stütz, AE; Tschernutter, M; Windischhofer, W; Withers, SG
A Morita-Baylis-Hillman based route to C-5a-chain-extended 4-epi-isofagomine type glycosidase inhibitors.
Carbohydr Res. 2017; 442(15):31-40
Doi: 10.1016/j.carres.2017.03.003
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** 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(15):15-22
Doi: 10.1016/j.carres.2017.03.009
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** Schalli, M; Weber, P; Tysoe, C; Pabst, BM; Thonhofer, M; Paschke, E; Stütz, AE; Tschernutter, M; Windischhofer, W; Withers, SG
A new type of pharmacological chaperone for GM1-gangliosidosis related human lysosomal β-galactosidase: N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols.
Bioorg Med Chem Lett. 2017; 27(15):3431-3435
Doi: 10.1016/j.bmcl.2017.05.086
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** Thonhofer, M; Weber, P; Gonzalez Santana, A; Tysoe, C; Fischer, R; Pabst, BM; Paschke, E; Schalli, M; Stütz, AE; Tschernutter, M; Windischhofer, W; Withers, SG
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.
Carbohydr Res. 2016; 429(15):71-80
Doi: 10.1016/j.carres.2016.03.020
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** Thonhofer, M; Weber, P; Santana, AG; Fischer, R; Pabst, BM; Paschke, E; Schalli, M; Stütz, AE; Tschernutter, M; Windischhofer, W; Withers, SG
Synthesis of C-5a-chain extended derivatives of 4-epi-isofagomine: Powerful β-galactosidase inhibitors and low concentration activators of GM1-gangliosidosis-related human lysosomal β-galactosidase.
Bioorg Med Chem Lett. 2016; 26(5):1438-1442
Doi: 10.1016/j.bmcl.2016.01.059
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** Fantur, KM; Wrodnigg, TM; Stütz, AE; Pabst, BM; Paschke, E
Fluorous iminoalditols act as effective pharmacological chaperones against gene products from GLB₁ alleles causing GM1-gangliosidosis and Morquio B disease.
J Inherit Metab Dis. 2012; 35(3):495-503
Doi: 10.1007/s10545-011-9409-2
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** Fantur, K; Hofer, D; Schitter, G; Steiner, AJ; Pabst, BM; Wrodnigg, TM; Stütz, AE; Paschke, E
DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human G(M1)-gangliosidosis fibroblasts.
Mol Genet Metab. 2010; 100(3):262-268
Doi: 10.1016/j.ymgme.2010.03.019
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