Sophie Vieweg
École Polytechnique Fédérale de Lausanne
10 Papers
21 Citations
Sophie Vieweg is an academic researcher from École Polytechnique Fédérale de Lausanne. The author has contributed to research in topics: Huntingtin & Huntingtin Protein. The author has an hindex of 6, co-authored 10 publications. Previous affiliations of Sophie Vieweg include Max Planck Society.
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Papers
Nanoscale studies link amyloid maturity with polyglutamine diseases onset
Francesco Simone Ruggeri,Francesco Simone Ruggeri,Sophie Vieweg,Urszula Cendrowska,Giovanni Longo,Anass Chiki,Hilal A. Lashuel,Hilal A. Lashuel,Giovanni Dietler +8 more
TL;DR: This work investigates the structural, mechanical and morphological properties of fibrillar aggregates at the single molecule and nanometer scale using the first exon of the Huntingtin protein as a model system (Exon1) to provide novel insights into the molecular and structural basis of Huntingtin Exon1 aggregation and toxicity.
Mutant Exon1 Huntingtin Aggregation is Regulated by T3 Phosphorylation-Induced Structural Changes and Crosstalk between T3 Phosphorylation and Acetylation at K6.
Anass Chiki,Sean M. DeGuire,Francesco Simone Ruggeri,Domenico Sanfelice,Annalisa Ansaloni,Zhe Ming Wang,Urszula Cendrowska,Ritwik Burai,Sophie Vieweg,Annalisa Pastore,Giovanni Dietler,Hilal A. Lashuel +11 more
TL;DR: The results demonstrate that phosphorylation at T3 stabilizes the α-helical conformation of the N-terminal 17 amino acids (Nt17) and significantly inhibits the aggregation of mutant Httex1 and suggest that its aggregation and possibly its function(s) are controlled by regulatory mechanisms involving crosstalk between different PTMs.
Unraveling the complexity of amyloid polymorphism using gold nanoparticles and cryo-EM.
Urszula Cendrowska,Paulo Jacob Silva,Nadine Ait-Bouziad,Marie Müller,Zekiye Pelin Guven,Sophie Vieweg,Anass Chiki,Lynn Radamaker,Senthil Kumar,Marcus Fändrich,Francesco Tavanti,Maria Cristina Menziani,Alfredo Alexander-Katz,Francesco Stellacci,Hilal A. Lashuel +14 more
TL;DR: NPs that efficiently label amyloid fibrils produced in vitro or isolated from postmortem tissues, under hydrating conditions and in such a way as to unmask their polymorphism and morphological features are described, consistent with the emerging view that the physiologic milieu is a key determinant of amyloids fibril strains.
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An Intein-based Strategy for the Production of Tag-free Huntingtin Exon 1 Proteins Enables New Insights into the Polyglutamine Dependence of Httex1 Aggregation and Fibril Formation
TL;DR: This work reports an intein-based strategy that allows, for the first time, the rapid and efficient production of native tag-free Httex1 with polyQ repeats ranging from 7Q to 49Q, and reveals the inability of Ht Tex1–7Q/15Q to undergo amyloid fibril formation and an inverse correlation between fibrils length and polyQ repeat length.
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The Nt17 Domain and its Helical Conformation Regulate the Aggregation, Cellular Properties and Neurotoxicity of Mutant Huntingtin Exon 1.
Sophie Vieweg,Anne-Laure Mahul-Mellier,Francesco Simone Ruggeri,Nathan Riguet,Sean M. DeGuire,Anass Chiki,Urszula Cendrowska,Giovanni Dietler,Hilal A. Lashuel +8 more
TL;DR: In this paper, the authors investigated the effect of removing Nt17 or modulating its local structure on the membrane interactions, neuronal uptake, and toxicity of monomeric or fibrillar Httex1.
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