RNA as the stone guest of protein aggregation

Mostra el registre complet Registre parcial de l'ítem

  • dc.contributor.author Louka, Alexandra
  • dc.contributor.author Zacco, Elsa
  • dc.contributor.author Temussi, Piero Andrea
  • dc.contributor.author Tartaglia, Gian Gaetano
  • dc.contributor.author Pastore, Annalisa
  • dc.date.accessioned 2020-11-23T07:04:52Z
  • dc.date.available 2020-11-23T07:04:52Z
  • dc.date.issued 2020
  • dc.description.abstract The study of prions as infectious aggregates dates several decades. From its original formulation, the definition of a prion has progressively changed to the point that many aggregation-prone proteins are now considered bona fide prions. RNA molecules, not included in the original 'protein-only hypothesis', are also being recognized as important factors contributing to the 'prion behaviour', that implies the transmissibility of an aberrant fold. In particular, an association has recently emerged between aggregation and the assembly of prion-like proteins in RNA-rich complexes, associated with both physiological and pathological events. Here, we discuss the historical rising of the concept of prion-like domains, their relation to RNA and their role in protein aggregation. As a paradigmatic example, we present the case study of TDP-43, an RNA-binding prion-like protein associated with amyotrophic lateral sclerosis. Through this example, we demonstrate how the current definition of prions has incorporated quite different concepts making the meaning of the term richer and more stimulating. An important message that emerges from our analysis is the dual role of RNA in protein aggregation, making RNA, that has been considered for many years a 'silent presence' or the 'stone guest' of protein aggregation, an important component of the process.
  • dc.description.sponsorship UK Dementia Research Institute [RE1 3556], which is funded by the Medical Research Council, Alzheimer's Society and Alzheimer's Research UK; E.Z. was supported by a MINDED fellowship from the EU Horizon 2020 research and innovation program under the Marie Skłodowska-Curie [754490]. Funding for open access charge: UK Dementia Research Institute.
  • dc.format.mimetype application/pdf
  • dc.identifier.citation Louka A, Zacco E, Temussi PA, Tartaglia GG, Pastore A. RNA as the stone guest of protein aggregation. Nucleic Acids Res. 2020 Oct 17;48(21):11880-9. DOI: 10.1093/nar/gkaa822
  • dc.identifier.doi http://dx.doi.org/10.1093/nar/gkaa822
  • dc.identifier.issn 0305-1048
  • dc.identifier.uri http://hdl.handle.net/10230/45850
  • dc.language.iso eng
  • dc.publisher Oxford University Press
  • dc.relation.ispartof Nucleic Acids Res. 2020 Oct 17;48(21):11880-9
  • dc.relation.projectID info:eu-repo/grantAgreement/EC/H2020/754490
  • dc.rights © The Author(s) 2020. Published by Oxford University Press on behalf of Nucleic Acids Research. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
  • dc.rights.accessRights info:eu-repo/semantics/openAccess
  • dc.rights.uri http://creativecommons.org/licenses/by/4.0/
  • dc.title RNA as the stone guest of protein aggregation
  • dc.type info:eu-repo/semantics/article
  • dc.type.version info:eu-repo/semantics/publishedVersion

Col·leccions

Articles (Center for Genomic Regulation (CRG))
Documents OpenAIRE (Open Access Infrastructure for Research in Europe)