Nanoscale studies link amyloid maturity with polyglutamine diseases onset

F. S. Ruggeri, S. Vieweg, U. Cendrowska, G. Longo, A. Chiki, H. A. Lashuel, G. Dietler

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

The presence of expanded poly-glutamine (polyQ) repeats in proteins is directly linked to the pathogenesis of several neurodegenerative diseases, including Huntington's disease. However, the molecular and structural basis underlying the increased toxicity of aggregates formed by proteins containing expanded polyQ repeats remain poorly understood, in part due to the size and morphological heterogeneity of the aggregates they form in vitro. To address this knowledge gap and technical limitations, we investigated 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). Our findings demonstrate a direct correlation of the morphological and mechanical properties of Exon1 aggregates with their structural organization at the single aggregate and nanometric scale and provide novel insights into the molecular and structural basis of Huntingtin Exon1 aggregation and toxicity.

Original languageEnglish
Article number31155
JournalScientific Reports
Volume6
DOIs
Publication statusPublished - 8 Aug 2016

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Amyloid
Huntington Disease
Neurodegenerative Diseases
Exons
polyglutamine
Proteins

ASJC Scopus subject areas

  • General

Cite this

Ruggeri, F. S., Vieweg, S., Cendrowska, U., Longo, G., Chiki, A., Lashuel, H. A., & Dietler, G. (2016). Nanoscale studies link amyloid maturity with polyglutamine diseases onset. Scientific Reports, 6, [31155]. https://doi.org/10.1038/srep31155

Nanoscale studies link amyloid maturity with polyglutamine diseases onset. / Ruggeri, F. S.; Vieweg, S.; Cendrowska, U.; Longo, G.; Chiki, A.; Lashuel, H. A.; Dietler, G.

In: Scientific Reports, Vol. 6, 31155, 08.08.2016.

Research output: Contribution to journalArticle

Ruggeri, FS, Vieweg, S, Cendrowska, U, Longo, G, Chiki, A, Lashuel, HA & Dietler, G 2016, 'Nanoscale studies link amyloid maturity with polyglutamine diseases onset', Scientific Reports, vol. 6, 31155. https://doi.org/10.1038/srep31155
Ruggeri FS, Vieweg S, Cendrowska U, Longo G, Chiki A, Lashuel HA et al. Nanoscale studies link amyloid maturity with polyglutamine diseases onset. Scientific Reports. 2016 Aug 8;6. 31155. https://doi.org/10.1038/srep31155
Ruggeri, F. S. ; Vieweg, S. ; Cendrowska, U. ; Longo, G. ; Chiki, A. ; Lashuel, H. A. ; Dietler, G. / Nanoscale studies link amyloid maturity with polyglutamine diseases onset. In: Scientific Reports. 2016 ; Vol. 6.
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