Alpha 2.0
Login Register
» Authors » Eduard Paschke

Eduard Paschke

Explore the profile of Eduard Paschke including associated specialties, affiliations and a list of published articles. Areas
Snapshot
Articles 39
Citations 588
Followers 0
Related Specialties
Biochemistry
Pediatrics
Neurology
Top 10 Co-Authors
Arnold E Stutz
Stephen G Withers
Bettina M Pabst
Tanja M Wrodnigg
Martin Thonhofer
Barbara Plecko
Marion Tschernutter
Gere Sunder-Plassmann
Werner Windischhofer
Michael Schalli
Published In
Carbohydr Res
Bioorg Med Chem Lett
Hum Mutat
J Inherit Metab Dis
Molecules
Affiliations
Soon will be listed here.
Recent Articles
1.
Mechanistic Insights into the Chaperoning of Human Lysosomal-Galactosidase Activity: Highly Functionalized Aminocyclopentanes and -5a-Substituted Derivatives of 4--Isofagomine
Weber P, Thonhofer M, Averill S, Davies G, Gonzalez Santana A, Muller P, et al.
Molecules . 2020 Sep; 25(17). PMID: 32899288
Glycosidase inhibitors have shown great potential as pharmacological chaperones for lysosomal storage diseases. In light of this, a series of new cyclopentanoid β-galactosidase inhibitors were prepared and their inhibitory and...
2.
Morquio-B disease: Clinical and genetic characteristics of a distinct -related dysostosis multiplex
Abumansour I, Yuskiv N, Paschke E, Stockler-Ipsiroglu S
JIMD Rep . 2020 Feb; 51(1):30-44. PMID: 32071837
Background: Morquio-B disease (MBD) is a distinct -related dysostosis multiplex involving the trabecular parts of long bones and spine, presenting a mild phenocopy of -related Morquio-A disease. Methods: We analyzed...
3.
The Clinical and Molecular Spectrum of GM1 Gangliosidosis
Arash-Kaps L, Komlosi K, Seegraber M, Diederich S, Paschke E, Amraoui Y, et al.
J Pediatr . 2019 Nov; 215:152-157.e3. PMID: 31761138
Objective: To evaluate the clinical presentation of patients with GM1 gangliosidosis and to determine whether specific clinical or biochemical signs could lead to a prompt diagnosis. Study Design: We retrospectively...
4.
Potent GH20 N-Acetyl-β-d-hexosaminidase Inhibitors: N-Substituted 3-acetamido-4-amino-5-hydroxymethyl-cyclopentanediols
Weber P, Nasseri S, Pabst B, Torvisco A, Muller P, Paschke E, et al.
Molecules . 2018 Mar; 23(3). PMID: 29558439
From 1,2;3,4-di--isopropylidene-d-galactopyranose, a preliminary series of highly functionalized amino(hydroxymethyl)cyclopentanes was easily available. These amine-containing basic carbasugars featuring the d- configuration are potent inhibitors of the GH20 β-d-hexosaminidases probed and may...
5.
A new type of pharmacological chaperone for G-gangliosidosis related human lysosomal β-galactosidase: N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols
Schalli M, Weber P, Tysoe C, Pabst B, Thonhofer M, Paschke E, et al.
Bioorg Med Chem Lett . 2017 Jun; 27(15):3431-3435. PMID: 28600215
N-Functionalized amino(hydroxymethyl)cyclopentanetriols are potent inhibitors of β-d-galactosidases and, for the first time, could be shown to act as pharmacological chaperones for G-gangliosidosis-associated lysosomal acid β-galactosidase thus representing a new structural...
6.
N-Substituted 5-amino-1-hydroxymethyl-cyclopentanetriols: A new family of activity promotors for a G-gangliosidosis related human lysosomal β-galactosidase mutant
Schalli M, Tysoe C, Fischer R, Pabst B, Thonhofer M, Paschke E, et al.
Carbohydr Res . 2017 Mar; 443-444:15-22. PMID: 28319682
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...
7.
A Morita-Baylis-Hillman based route to C-5a-chain-extended 4-epi-isofagomine type glycosidase inhibitors
Lebl R, Thonhofer M, Tysoe C, Pabst B, Schalli M, Weber P, et al.
Carbohydr Res . 2017 Mar; 442:31-40. PMID: 28288345
By Morita-Baylis-Hillman reaction of 2,3-O-isopropylidene-D-glyceraldehyde with α,β-unsaturated carbonyl as well as hetero analogous carbonyl compounds such as acrylonitrile, suitable precursors of isofagomine and of 4-epi-isofagomine are available. Elaboration of the...
8.
Screening for Fabry Disease by Urinary Globotriaosylceramide Isoforms Measurement in Patients with Left Ventricular Hypertrophy
Gaggl M, Lajic N, Heinze G, Voigtlander T, Sunder-Plassmann R, Paschke E, et al.
Int J Med Sci . 2016 May; 13(5):340-6. PMID: 27226774
Background: Left ventricular hypertrophy (LVH) is a frequent echocardiographic feature in Fabry disease (FD) and in severe cases may be confused with hypertrophic cardiomyopathy (HCM) of other origin. The prevalence...
9.
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
Thonhofer M, Weber P, Gonzalez Santana A, Tysoe C, Fischer R, Pabst B, et al.
Carbohydr Res . 2016 Apr; 429:71-80. PMID: 27063389
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 prepared. New compounds exhibit...
10.
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
Thonhofer M, Weber P, Gonzalez Santana A, Fischer R, Pabst B, Paschke E, et al.
Bioorg Med Chem Lett . 2016 Feb; 26(5):1438-42. PMID: 26838810
From an easily available partially protected formal derivative of 1-deoxymannojirimycin, by hydroxymethyl chain-branching and further elaboration, lipophilic analogs of the powerful β-d-galactosidase inhibitor 4-epi-isofagomine have become available. New compounds exhibit...