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Disulfide Bonding in Neurodegenerative Misfolding Diseases
In recent years an increasing number of neurodegenerative diseases has been linked to the misfolding of a specific protein and its subsequent accumulation into aggregated species, often toxic to the cell. Of all the factors that affect the behavior of these proteins, disulfide bonds are likely to be...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Hindawi Publishing Corporation
2013
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3747422/ https://www.ncbi.nlm.nih.gov/pubmed/23983694 http://dx.doi.org/10.1155/2013/318319 |
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author | Mossuto, Maria Francesca |
author_facet | Mossuto, Maria Francesca |
author_sort | Mossuto, Maria Francesca |
collection | PubMed |
description | In recent years an increasing number of neurodegenerative diseases has been linked to the misfolding of a specific protein and its subsequent accumulation into aggregated species, often toxic to the cell. Of all the factors that affect the behavior of these proteins, disulfide bonds are likely to be important, being very conserved in protein sequences and being the enzymes devoted to their formation among the most conserved machineries in mammals. Their crucial role in the folding and in the function of a big fraction of the human proteome is well established. The role of disulfide bonding in preventing and managing protein misfolding and aggregation is currently under investigation. New insights into their involvement in neurodegenerative diseases, their effect on the process of protein misfolding and aggregation, and into the role of the cellular machineries devoted to disulfide bond formation in neurodegenerative diseases are emerging. These studies mark a step forward in the comprehension of the biological base of neurodegenerative disorders and highlight the numerous questions that still remain open. |
format | Online Article Text |
id | pubmed-3747422 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-37474222013-08-27 Disulfide Bonding in Neurodegenerative Misfolding Diseases Mossuto, Maria Francesca Int J Cell Biol Review Article In recent years an increasing number of neurodegenerative diseases has been linked to the misfolding of a specific protein and its subsequent accumulation into aggregated species, often toxic to the cell. Of all the factors that affect the behavior of these proteins, disulfide bonds are likely to be important, being very conserved in protein sequences and being the enzymes devoted to their formation among the most conserved machineries in mammals. Their crucial role in the folding and in the function of a big fraction of the human proteome is well established. The role of disulfide bonding in preventing and managing protein misfolding and aggregation is currently under investigation. New insights into their involvement in neurodegenerative diseases, their effect on the process of protein misfolding and aggregation, and into the role of the cellular machineries devoted to disulfide bond formation in neurodegenerative diseases are emerging. These studies mark a step forward in the comprehension of the biological base of neurodegenerative disorders and highlight the numerous questions that still remain open. Hindawi Publishing Corporation 2013 2013-08-01 /pmc/articles/PMC3747422/ /pubmed/23983694 http://dx.doi.org/10.1155/2013/318319 Text en Copyright © 2013 Maria Francesca Mossuto. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Mossuto, Maria Francesca Disulfide Bonding in Neurodegenerative Misfolding Diseases |
title | Disulfide Bonding in Neurodegenerative Misfolding Diseases |
title_full | Disulfide Bonding in Neurodegenerative Misfolding Diseases |
title_fullStr | Disulfide Bonding in Neurodegenerative Misfolding Diseases |
title_full_unstemmed | Disulfide Bonding in Neurodegenerative Misfolding Diseases |
title_short | Disulfide Bonding in Neurodegenerative Misfolding Diseases |
title_sort | disulfide bonding in neurodegenerative misfolding diseases |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3747422/ https://www.ncbi.nlm.nih.gov/pubmed/23983694 http://dx.doi.org/10.1155/2013/318319 |
work_keys_str_mv | AT mossutomariafrancesca disulfidebondinginneurodegenerativemisfoldingdiseases |