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dc.contributor.authorKuiper, E. F.Elsienapt_BR
dc.contributor.authorMattos, Eduardo Preusser dept_BR
dc.contributor.authorJardim, Laura Bannachpt_BR
dc.contributor.authorKampinga, Harmpt_BR
dc.contributor.authorBergink, Stevenpt_BR
dc.date.accessioned2018-11-28T02:45:42Zpt_BR
dc.date.issued2017pt_BR
dc.identifier.issn1662-453Xpt_BR
dc.identifier.urihttp://hdl.handle.net/10183/185182pt_BR
dc.description.abstractExpanded polyglutamine (polyQ) stretches in at least nine unrelated proteins lead to inherited neuronal dysfunction and degeneration. The expansion size in all diseases correlates with age at onset (AO) of disease and with polyQ protein aggregation, indicating that the expanded polyQ stretch is the main driving force for the disease onset. Interestingly, there is marked interpatient variability in expansion thresholds for a given disease. Between different polyQ diseases the repeat length vs. AO also indicates the existence of modulatory effects on aggregation of the upstream and downstream amino acid sequences flanking the Q expansion. This can be either due to intrinsic modulation of aggregation by the flanking regions, or due to differential interaction with other proteins, such as the components of the cellular protein quality control network. Indeed, several lines of evidence suggest that molecular chaperones have impact on the handling of different polyQ proteins. Here, we review factors differentially influencing polyQ aggregation: the Q-stretch itself, modulatory flanking sequences, interaction partners, cleavage of polyQ-containing proteins, and post-translational modifications, with a special focus on the role of molecular chaperones. By discussing typical examples of how these factors influence aggregation, we provide more insight on the variability of AO between different diseases as well as within the same polyQ disorder, on the molecular level.en
dc.format.mimetypeapplication/pdfpt_BR
dc.language.isoengpt_BR
dc.relation.ispartofFrontiers in neuroscience. Lausanne. vol. 11 (Mar. 2017), 145, 11 f.pt_BR
dc.rightsOpen Accessen
dc.subjectDoença de Huntingtonpt_BR
dc.subjectAggregationen
dc.subjectHuntington’s diseaseen
dc.subjectDoença de Machado-Josephpt_BR
dc.subjectMachado-Joseph diseaseen
dc.subjectChaperonas molecularespt_BR
dc.subjectMolecular chaperonesen
dc.subjectPolyglutamine diseaseen
dc.titleChaperones in polyglutamine aggregation : beyond the Q-stretchpt_BR
dc.typeArtigo de periódicopt_BR
dc.identifier.nrb001082068pt_BR
dc.type.originEstrangeiropt_BR


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