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The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization
Aggregation of the 140-residue protein α-synuclein (αSN) is a key factor in the etiology of Parkinson’s disease. Although the intensely anionic C-terminal domain (CTD) of αSN does not form part of the amyloid core region or affect membrane binding ability, truncation or reduction of charges in the C...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8831632/ https://www.ncbi.nlm.nih.gov/pubmed/35145226 http://dx.doi.org/10.1038/s42003-022-03059-8 |
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author | Farzadfard, Azad Pedersen, Jannik Nedergaard Meisl, Georg Somavarapu, Arun Kumar Alam, Parvez Goksøyr, Louise Nielsen, Morten Agertoug Sander, Adam Frederik Knowles, Tuomas P. J. Pedersen, Jan Skov Otzen, Daniel Erik |
author_facet | Farzadfard, Azad Pedersen, Jannik Nedergaard Meisl, Georg Somavarapu, Arun Kumar Alam, Parvez Goksøyr, Louise Nielsen, Morten Agertoug Sander, Adam Frederik Knowles, Tuomas P. J. Pedersen, Jan Skov Otzen, Daniel Erik |
author_sort | Farzadfard, Azad |
collection | PubMed |
description | Aggregation of the 140-residue protein α-synuclein (αSN) is a key factor in the etiology of Parkinson’s disease. Although the intensely anionic C-terminal domain (CTD) of αSN does not form part of the amyloid core region or affect membrane binding ability, truncation or reduction of charges in the CTD promotes fibrillation through as yet unknown mechanisms. Here, we study stepwise truncated CTDs and identify a threshold region around residue 121; constructs shorter than this dramatically increase their fibrillation tendency. Remarkably, these effects persist even when as little as 10% of the truncated variant is mixed with the full-length protein. Increased fibrillation can be explained by a substantial increase in self-replication, most likely via fragmentation. Paradoxically, truncation also suppresses toxic oligomer formation, and oligomers that can be formed by chemical modification show reduced membrane affinity and cytotoxicity. These remarkable changes correlate to the loss of negative electrostatic potential in the CTD and highlight a double-edged electrostatic safety guard. |
format | Online Article Text |
id | pubmed-8831632 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-88316322022-02-24 The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization Farzadfard, Azad Pedersen, Jannik Nedergaard Meisl, Georg Somavarapu, Arun Kumar Alam, Parvez Goksøyr, Louise Nielsen, Morten Agertoug Sander, Adam Frederik Knowles, Tuomas P. J. Pedersen, Jan Skov Otzen, Daniel Erik Commun Biol Article Aggregation of the 140-residue protein α-synuclein (αSN) is a key factor in the etiology of Parkinson’s disease. Although the intensely anionic C-terminal domain (CTD) of αSN does not form part of the amyloid core region or affect membrane binding ability, truncation or reduction of charges in the CTD promotes fibrillation through as yet unknown mechanisms. Here, we study stepwise truncated CTDs and identify a threshold region around residue 121; constructs shorter than this dramatically increase their fibrillation tendency. Remarkably, these effects persist even when as little as 10% of the truncated variant is mixed with the full-length protein. Increased fibrillation can be explained by a substantial increase in self-replication, most likely via fragmentation. Paradoxically, truncation also suppresses toxic oligomer formation, and oligomers that can be formed by chemical modification show reduced membrane affinity and cytotoxicity. These remarkable changes correlate to the loss of negative electrostatic potential in the CTD and highlight a double-edged electrostatic safety guard. Nature Publishing Group UK 2022-02-10 /pmc/articles/PMC8831632/ /pubmed/35145226 http://dx.doi.org/10.1038/s42003-022-03059-8 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Article Farzadfard, Azad Pedersen, Jannik Nedergaard Meisl, Georg Somavarapu, Arun Kumar Alam, Parvez Goksøyr, Louise Nielsen, Morten Agertoug Sander, Adam Frederik Knowles, Tuomas P. J. Pedersen, Jan Skov Otzen, Daniel Erik The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
title | The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
title_full | The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
title_fullStr | The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
title_full_unstemmed | The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
title_short | The C-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
title_sort | c-terminal tail of α-synuclein protects against aggregate replication but is critical for oligomerization |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8831632/ https://www.ncbi.nlm.nih.gov/pubmed/35145226 http://dx.doi.org/10.1038/s42003-022-03059-8 |
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