Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging
The misfolding, aggregation, and tissue accumulation of proteins are common events in diverse chronic diseases, known as protein misfolding disorders. Many of these diseases are associated with aging, but the mechanism for this connection is unknown. Recent evidence has shown that the formation and...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | International Journal of Cell Biology |
| Online Access: | http://dx.doi.org/10.1155/2013/638083 |
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| author | Karina Cuanalo-Contreras Abhisek Mukherjee Claudio Soto |
| author_facet | Karina Cuanalo-Contreras Abhisek Mukherjee Claudio Soto |
| author_sort | Karina Cuanalo-Contreras |
| collection | DOAJ |
| description | The misfolding, aggregation, and tissue accumulation of proteins are common events in diverse chronic diseases, known as protein misfolding disorders. Many of these diseases are associated with aging, but the mechanism for this connection is unknown. Recent evidence has shown that the formation and accumulation of protein aggregates may be a process frequently occurring during normal aging, but it is unknown whether protein misfolding is a cause or a consequence of aging. To combat the formation of these misfolded aggregates cells have developed complex and complementary pathways aiming to maintain protein homeostasis. These protective pathways include the unfolded protein response, the ubiquitin proteasome system, autophagy, and the encapsulation of damaged proteins in aggresomes. In this paper we review the current knowledge on the role of protein misfolding in disease and aging as well as the implication of deficiencies in the proteostasis cellular pathways in these processes. It is likely that further understanding of the mechanisms involved in protein misfolding and the natural defense pathways may lead to novel strategies for treatment of age-dependent protein misfolding disorders and perhaps aging itself. |
| format | Article |
| id | doaj-art-da069b522efc43889b0b2f3c5870f2a8 |
| institution | Kabale University |
| issn | 1687-8876 1687-8884 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Cell Biology |
| spelling | doaj-art-da069b522efc43889b0b2f3c5870f2a82025-08-20T03:24:25ZengWileyInternational Journal of Cell Biology1687-88761687-88842013-01-01201310.1155/2013/638083638083Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and AgingKarina Cuanalo-Contreras0Abhisek Mukherjee1Claudio Soto2Mitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, TX 77030, USAMitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, TX 77030, USAMitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, TX 77030, USAThe misfolding, aggregation, and tissue accumulation of proteins are common events in diverse chronic diseases, known as protein misfolding disorders. Many of these diseases are associated with aging, but the mechanism for this connection is unknown. Recent evidence has shown that the formation and accumulation of protein aggregates may be a process frequently occurring during normal aging, but it is unknown whether protein misfolding is a cause or a consequence of aging. To combat the formation of these misfolded aggregates cells have developed complex and complementary pathways aiming to maintain protein homeostasis. These protective pathways include the unfolded protein response, the ubiquitin proteasome system, autophagy, and the encapsulation of damaged proteins in aggresomes. In this paper we review the current knowledge on the role of protein misfolding in disease and aging as well as the implication of deficiencies in the proteostasis cellular pathways in these processes. It is likely that further understanding of the mechanisms involved in protein misfolding and the natural defense pathways may lead to novel strategies for treatment of age-dependent protein misfolding disorders and perhaps aging itself.http://dx.doi.org/10.1155/2013/638083 |
| spellingShingle | Karina Cuanalo-Contreras Abhisek Mukherjee Claudio Soto Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging International Journal of Cell Biology |
| title | Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging |
| title_full | Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging |
| title_fullStr | Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging |
| title_full_unstemmed | Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging |
| title_short | Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging |
| title_sort | role of protein misfolding and proteostasis deficiency in protein misfolding diseases and aging |
| url | http://dx.doi.org/10.1155/2013/638083 |
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