Nanoscale studies link amyloid maturity with polyglutamine diseases onset
<p dir="ltr">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 f...
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2016
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| _version_ | 1864513556424163328 |
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| author | F. S. Ruggeri (19738036) |
| author2 | S. Vieweg (19738039) U. Cendrowska (19738042) G. Longo (19095992) A. Chiki (19738045) H. A. Lashuel (19738048) G. Dietler (19738051) |
| author2_role | author author author author author author |
| author_facet | F. S. Ruggeri (19738036) S. Vieweg (19738039) U. Cendrowska (19738042) G. Longo (19095992) A. Chiki (19738045) H. A. Lashuel (19738048) G. Dietler (19738051) |
| author_role | author |
| dc.creator.none.fl_str_mv | F. S. Ruggeri (19738036) S. Vieweg (19738039) U. Cendrowska (19738042) G. Longo (19095992) A. Chiki (19738045) H. A. Lashuel (19738048) G. Dietler (19738051) |
| dc.date.none.fl_str_mv | 2016-08-08T03:00:00Z |
| dc.identifier.none.fl_str_mv | 10.1038/srep31155 |
| dc.relation.none.fl_str_mv | https://figshare.com/articles/journal_contribution/Nanoscale_studies_link_amyloid_maturity_with_polyglutamine_diseases_onset/27101623 |
| dc.rights.none.fl_str_mv | CC BY 4.0 info:eu-repo/semantics/openAccess |
| dc.subject.none.fl_str_mv | Biomedical and clinical sciences Neurosciences Huntington’s disease Protein aggregation Structural properties Mechanical properties Morphological properties |
| dc.title.none.fl_str_mv | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| dc.type.none.fl_str_mv | Text Journal contribution info:eu-repo/semantics/publishedVersion text contribution to journal |
| description | <p dir="ltr">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.</p><h2>Other Information</h2><p dir="ltr">Published in: Scientific Reports<br>License: <a href="https://creativecommons.org/licenses/by/4.0" target="_blank">https://creativecommons.org/licenses/by/4.0</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1038/srep31155" target="_blank">https://dx.doi.org/10.1038/srep31155</a></p> |
| eu_rights_str_mv | openAccess |
| id | Manara2_7ebc383e24e8d6dcc049acd2a5f54836 |
| identifier_str_mv | 10.1038/srep31155 |
| network_acronym_str | Manara2 |
| network_name_str | Manara2 |
| oai_identifier_str | oai:figshare.com:article/27101623 |
| publishDate | 2016 |
| repository.mail.fl_str_mv | |
| repository.name.fl_str_mv | |
| repository_id_str | |
| rights_invalid_str_mv | CC BY 4.0 |
| spelling | Nanoscale studies link amyloid maturity with polyglutamine diseases onsetF. S. Ruggeri (19738036)S. Vieweg (19738039)U. Cendrowska (19738042)G. Longo (19095992)A. Chiki (19738045)H. A. Lashuel (19738048)G. Dietler (19738051)Biomedical and clinical sciencesNeurosciencesHuntington’s diseaseProtein aggregationStructural propertiesMechanical propertiesMorphological properties<p dir="ltr">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.</p><h2>Other Information</h2><p dir="ltr">Published in: Scientific Reports<br>License: <a href="https://creativecommons.org/licenses/by/4.0" target="_blank">https://creativecommons.org/licenses/by/4.0</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1038/srep31155" target="_blank">https://dx.doi.org/10.1038/srep31155</a></p>2016-08-08T03:00:00ZTextJournal contributioninfo:eu-repo/semantics/publishedVersiontextcontribution to journal10.1038/srep31155https://figshare.com/articles/journal_contribution/Nanoscale_studies_link_amyloid_maturity_with_polyglutamine_diseases_onset/27101623CC BY 4.0info:eu-repo/semantics/openAccessoai:figshare.com:article/271016232016-08-08T03:00:00Z |
| spellingShingle | Nanoscale studies link amyloid maturity with polyglutamine diseases onset F. S. Ruggeri (19738036) Biomedical and clinical sciences Neurosciences Huntington’s disease Protein aggregation Structural properties Mechanical properties Morphological properties |
| status_str | publishedVersion |
| title | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| title_full | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| title_fullStr | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| title_full_unstemmed | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| title_short | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| title_sort | Nanoscale studies link amyloid maturity with polyglutamine diseases onset |
| topic | Biomedical and clinical sciences Neurosciences Huntington’s disease Protein aggregation Structural properties Mechanical properties Morphological properties |