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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2022
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.
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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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