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Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?

2021-06Journal article Open Access
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dc.contributor.authorMarcelo, Adriana
dc.contributor.authorKoppenol, Rebekah
dc.contributor.authorAlmeida, Luis Pedro
dc.contributor.authorMatos, Carlos A
dc.contributor.authorNóbrega, Clévio
dc.date.accessioned2021-07-07T09:22:34Z
dc.date.available2021-07-07T09:22:34Z
dc.date.issued2021-06
dc.description.abstractStress granules (SGs) are membraneless cell compartments formed in response to different stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other proteins coalesce together. SGs assembly is crucial for cell survival, since SGs are implicated in the regulation of translation, mRNA storage and stabilization and cell signalling, during stress. One defining feature of SGs is their dynamism, as they are quickly assembled upon stress and then rapidly dispersed after the stress source is no longer present. Recently, SGs dynamics, their components and their functions have begun to be studied in the context of human diseases. Interestingly, the regulated protein self-assembly that mediates SG formation contrasts with the pathological protein aggregation that is a feature of several neurodegenerative diseases. In particular, aberrant protein coalescence is a key feature of polyglutamine (PolyQ) diseases, a group of nine disorders that are caused by an abnormal expansion of PolyQ tract-bearing proteins, which increases the propensity of those proteins to aggregate. Available data concerning the abnormal properties of the mutant PolyQ disease-causing proteins and their involvement in stress response dysregulation strongly suggests an important role for SGs in the pathogenesis of PolyQ disorders. This review aims at discussing the evidence supporting the existence of a link between SGs functionality and PolyQ disorders, by focusing on the biology of SGs and on the way it can be altered in a PolyQ disease context.pt_PT
dc.description.sponsorshipALG-01-0145-FEDER-29480, SFRH/BD/133192/2017, SFRH/BD/133192/2017, SFRH/BD/148533/2019pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.doi10.1038/s41419-021-03873-8pt_PT
dc.identifier.issn2041-4889
dc.identifier.urihttp://hdl.handle.net/10400.1/16735
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherSpringer Naturept_PT
dc.relationCeftiofur Long Acting to Reduce Antibiotics
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectSpinocerebellar ataxia type-3pt_PT
dc.subjectN-terminal huntingtinpt_PT
dc.subjectOxidative stresspt_PT
dc.subjectMutant huntingtinpt_PT
dc.subjectCellular stresspt_PT
dc.subjectMolecular-mechanismspt_PT
dc.subjectNuclear inclusionspt_PT
dc.subjectAndrogen receptorpt_PT
dc.subjectAxonal-transportpt_PT
dc.subjectPhase-transitionpt_PT
dc.titleStress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?pt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.awardTitleCeftiofur Long Acting to Reduce Antibiotics
oaire.awardURIinfo:eu-repo/grantAgreement/EC/FP7/222424/EU
oaire.citation.issue6pt_PT
oaire.citation.startPage592pt_PT
oaire.citation.titleCell Death & Diseasept_PT
oaire.citation.volume12pt_PT
oaire.fundingStreamFP7
person.familyNameMarcelo
person.familyNameKoppenol
person.familyNameAlmeida
person.familyNameAlbuquerque Andrade de Matos
person.familyNameNóbrega
person.givenNameAdriana
person.givenNameRebekah
person.givenNameLuis Pedro
person.givenNameCarlos Adriano
person.givenNameClévio
person.identifier.ciencia-id7613-00C2-1621
person.identifier.ciencia-id2917-AE9E-33C1
person.identifier.ciencia-idDC14-C299-222A
person.identifier.ciencia-idC510-7F41-BAF8
person.identifier.orcid0000-0002-7327-0170
person.identifier.orcid0000-0002-1892-8670
person.identifier.orcid0000-0001-7805-9086
person.identifier.orcid0000-0002-9019-7569
person.identifier.orcid0000-0002-8312-5292
person.identifier.ridM-6047-2013
person.identifier.scopus-author-id37016980900
person.identifier.scopus-author-id24473454000
project.funder.identifierhttp://doi.org/10.13039/501100008530
project.funder.nameEuropean Commission
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
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