The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications
Neurodegenerative disorders are not only characterized by specific patterns of cell loss but the presence and accumulation of various pathological proteins—both of which correlate with disease evolution. There is now mounting evidence to suggest that these pathological proteins present with toxic, a...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2017-11-01
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| Series: | Frontiers in Neuroscience |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/article/10.3389/fnins.2017.00647/full |
| _version_ | 1828517974072360960 |
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| author | Maria Masnata Francesca Cicchetti Francesca Cicchetti |
| author_facet | Maria Masnata Francesca Cicchetti Francesca Cicchetti |
| author_sort | Maria Masnata |
| collection | DOAJ |
| description | Neurodegenerative disorders are not only characterized by specific patterns of cell loss but the presence and accumulation of various pathological proteins—both of which correlate with disease evolution. There is now mounting evidence to suggest that these pathological proteins present with toxic, at times prion-like, properties and can therefore seed pathology in neighboring as well remotely connected healthy neurons as they spread across the brain. What is less clear, at this stage, is how much this actually contributes to, and drives, the core pathogenic events. In this review, we present a comprehensive, up-to-date summary of the reported in vitro studies that support the spreading and seeding capacities of pathological proteins, with an emphasis on mutant huntingtin protein in the context of Huntington's disease, although in vivo work remains to be performed to validate this theory in this particular disease. We have further reviewed these findings in light of their potential implications for the development of novel therapeutic approaches. |
| first_indexed | 2024-12-11T18:48:52Z |
| format | Article |
| id | doaj.art-a6b28adc7579483cab60adee52a59e97 |
| institution | Directory Open Access Journal |
| issn | 1662-453X |
| language | English |
| last_indexed | 2024-12-11T18:48:52Z |
| publishDate | 2017-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neuroscience |
| spelling | doaj.art-a6b28adc7579483cab60adee52a59e972022-12-22T00:54:22ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2017-11-011110.3389/fnins.2017.00647309481The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic ImplicationsMaria Masnata0Francesca Cicchetti1Francesca Cicchetti2Axe Neurosciences, Centre de Recherche du CHU de Québec-Université Laval, Quebec, QC, CanadaAxe Neurosciences, Centre de Recherche du CHU de Québec-Université Laval, Quebec, QC, CanadaDépartement de Psychiatrie & Neurosciences, Université Laval, Quebec, QC, CanadaNeurodegenerative disorders are not only characterized by specific patterns of cell loss but the presence and accumulation of various pathological proteins—both of which correlate with disease evolution. There is now mounting evidence to suggest that these pathological proteins present with toxic, at times prion-like, properties and can therefore seed pathology in neighboring as well remotely connected healthy neurons as they spread across the brain. What is less clear, at this stage, is how much this actually contributes to, and drives, the core pathogenic events. In this review, we present a comprehensive, up-to-date summary of the reported in vitro studies that support the spreading and seeding capacities of pathological proteins, with an emphasis on mutant huntingtin protein in the context of Huntington's disease, although in vivo work remains to be performed to validate this theory in this particular disease. We have further reviewed these findings in light of their potential implications for the development of novel therapeutic approaches.http://journal.frontiersin.org/article/10.3389/fnins.2017.00647/fullHuntington's diseaseParkinson's diseaseAlzheimer's diseaseprionstauβ-amyloid |
| spellingShingle | Maria Masnata Francesca Cicchetti Francesca Cicchetti The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications Frontiers in Neuroscience Huntington's disease Parkinson's disease Alzheimer's disease prions tau β-amyloid |
| title | The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications |
| title_full | The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications |
| title_fullStr | The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications |
| title_full_unstemmed | The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications |
| title_short | The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications |
| title_sort | evidence for the spread and seeding capacities of the mutant huntingtin protein in in vitro systems and their therapeutic implications |
| topic | Huntington's disease Parkinson's disease Alzheimer's disease prions tau β-amyloid |
| url | http://journal.frontiersin.org/article/10.3389/fnins.2017.00647/full |
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