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Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome

Exfoliation syndrome is largely considered an age-related disease that presents with fibrillar aggregates in the front part of the eye. A growing body of literature has investigated structural diversity of amyloids and fibrillar aggregates associated with neurodegenerative disease. However, in case...

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Autores principales: Ghaffari Sharaf, Mehdi, Amidian, Sara, Rathod, Vineet, Crichton, Andrew, Damji, Karim F., Wille, Holger, Unsworth, Larry D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7522999/
https://www.ncbi.nlm.nih.gov/pubmed/32985538
http://dx.doi.org/10.1038/s41598-020-72737-6
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author Ghaffari Sharaf, Mehdi
Amidian, Sara
Rathod, Vineet
Crichton, Andrew
Damji, Karim F.
Wille, Holger
Unsworth, Larry D.
author_facet Ghaffari Sharaf, Mehdi
Amidian, Sara
Rathod, Vineet
Crichton, Andrew
Damji, Karim F.
Wille, Holger
Unsworth, Larry D.
author_sort Ghaffari Sharaf, Mehdi
collection PubMed
description Exfoliation syndrome is largely considered an age-related disease that presents with fibrillar aggregates in the front part of the eye. A growing body of literature has investigated structural diversity of amyloids and fibrillar aggregates associated with neurodegenerative disease. However, in case of exfoliation syndrome, there is a dearth of information on the biophysical characteristics of these fibrils and structural polymorphisms. Herein, structural diversity of fibrils isolated from the anterior lens capsule of patients was evaluated using transmission electron microscopy techniques. It was apparent that, despite having a low sample number of different patients, there exists a wide range of fibril morphologies. As it is not precisely understood how these fibrils form, or what they are composed of, it is difficult to postulate a mechanism responsible for these differences in fibril structure. However, it is apparent that there is a wider range of fibril structure than initially appreciated. Moreover, these data may suggest the variance in fibril structure arises from patient-specific fibril composition and/or formation mechanisms.
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spelling pubmed-75229992020-09-29 Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome Ghaffari Sharaf, Mehdi Amidian, Sara Rathod, Vineet Crichton, Andrew Damji, Karim F. Wille, Holger Unsworth, Larry D. Sci Rep Article Exfoliation syndrome is largely considered an age-related disease that presents with fibrillar aggregates in the front part of the eye. A growing body of literature has investigated structural diversity of amyloids and fibrillar aggregates associated with neurodegenerative disease. However, in case of exfoliation syndrome, there is a dearth of information on the biophysical characteristics of these fibrils and structural polymorphisms. Herein, structural diversity of fibrils isolated from the anterior lens capsule of patients was evaluated using transmission electron microscopy techniques. It was apparent that, despite having a low sample number of different patients, there exists a wide range of fibril morphologies. As it is not precisely understood how these fibrils form, or what they are composed of, it is difficult to postulate a mechanism responsible for these differences in fibril structure. However, it is apparent that there is a wider range of fibril structure than initially appreciated. Moreover, these data may suggest the variance in fibril structure arises from patient-specific fibril composition and/or formation mechanisms. Nature Publishing Group UK 2020-09-28 /pmc/articles/PMC7522999/ /pubmed/32985538 http://dx.doi.org/10.1038/s41598-020-72737-6 Text en © The Author(s) 2020 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 licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence 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 licence, visit http://creativecommons.org/licenses/by/4.0/.
spellingShingle Article
Ghaffari Sharaf, Mehdi
Amidian, Sara
Rathod, Vineet
Crichton, Andrew
Damji, Karim F.
Wille, Holger
Unsworth, Larry D.
Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
title Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
title_full Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
title_fullStr Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
title_full_unstemmed Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
title_short Structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
title_sort structural polymorphisms in fibrillar aggregates associated with exfoliation syndrome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7522999/
https://www.ncbi.nlm.nih.gov/pubmed/32985538
http://dx.doi.org/10.1038/s41598-020-72737-6
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