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In Situ Architecture and Cellular Interactions of PolyQ Inclusions

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dc.contributor.authorBäuerlein, Felix
dc.contributor.authorSaha, Itika
dc.contributor.authorMishra, Archana
dc.contributor.authorKalemanov, Maria
dc.contributor.authorMartinez-Sanchez, Antonio
dc.contributor.authorKlein, Rudiger
dc.contributor.authorDudanova, Irina
dc.contributor.authorHipp, Mark
dc.contributor.authorHartl, Ulrich
dc.contributor.authorBaumeister, Wolfgang
dc.contributor.authorFernandez-Busnadiego, Ruben
dc.contributor.departmentIngenierĂ­a de la InformaciĂłn y las Comunicaciones
dc.date.accessioned2023-12-19T08:21:03Z
dc.date.available2023-12-19T08:21:03Z
dc.date.issued2017-09-21
dc.description© 2017 Elsevier Inc. This document is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ This document is the accepted version of a published Work that appeared in final form in cell. To access the final edited and published work see https://doi.org/10.1016/j.cell.2017.08.009es
dc.description.abstractExpression of many disease-related aggregation-prone proteins results in cytotoxicity and the formation of large intracellular inclusion bodies. To gain insight into the role of inclusions in pathology and the in situ structure of protein aggregates inside cells, we employ advanced cryo-electron tomography methods to analyze the structure of inclusions formed by polyglutamine (polyQ)-expanded huntingtin exon 1 within their intact cellular context. In primary mouse neurons and immortalized human cells, polyQ inclusions consist of amyloid-like fibrils that interact with cellular endomembranes, particularly of the endoplasmic reticulum (ER). Interactions with these fibrils lead to membrane deformation, the local impairment of ER organization, and profound alterations in ER membrane dynamics at the inclusion periphery. These results suggest that aberrant interactions between fibrils and endomembranes contribute to the deleterious cellular effects of protein aggregation.es
dc.formatapplication/pdfes
dc.format.extent20es
dc.identifier.citationCell. Vol. 171, Issue 1, 21 September 2017, Pages 179-187.e10
dc.identifier.doihttps://doi.org/10.1016/j.cell.2017.08.009
dc.identifier.issnPrint: 0092-8674
dc.identifier.issnElectronic: 1097-4172
dc.identifier.urihttp://hdl.handle.net/10201/136738
dc.languageenges
dc.publisherElsevieres
dc.relationThis research has received funding from the European Commission (FP7 GA ERC-2012-SyG_318987–ToPAG) and the Munich Cluster for Systems Neurology (EXC 010 SyNergy). M.K. was supported by the Graduate School of Quantitative Biosciences Munich (GSC-1006).es
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.titleIn Situ Architecture and Cellular Interactions of PolyQ Inclusionses
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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