Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases

Age-related misfolding and aggregation of disease-linked proteins in selective brain regions is a characteristic of neurodegenerative diseases. Although neuropathological aggregates that characterize these various diseases are found at sites other than synapses, increasing evidence supports the idea...

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Main Author: Gunnar K. Gouras
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:International Journal of Cell Biology
Online Access:http://dx.doi.org/10.1155/2013/141083
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author Gunnar K. Gouras
author_facet Gunnar K. Gouras
author_sort Gunnar K. Gouras
collection DOAJ
description Age-related misfolding and aggregation of disease-linked proteins in selective brain regions is a characteristic of neurodegenerative diseases. Although neuropathological aggregates that characterize these various diseases are found at sites other than synapses, increasing evidence supports the idea that synapses are where the pathogenesis begins. Understanding these diseases is hampered by our lack of knowledge of what the normal functions of these proteins are and how they are affected by aging. Evidence has supported the idea that neurodegenerative disease-linked proteins have a common propensity for prion protein-like cell-to-cell propagation. However, it is not thought that the prion-like quality of these proteins/peptides that allows their cell-to-cell transmission implies a role for human-to-human spread in common age-related neurodegenerative diseases. It will be important to better understand the molecular and cellular mechanisms governing the role of these aggregating proteins in neural function, especially at synapses, how their propagation occurs and how pathogenesis is promoted by aging.
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spelling doaj-art-ed572e1a9a6543d59646a8ad671cc06d2025-02-03T05:45:30ZengWileyInternational Journal of Cell Biology1687-88761687-88842013-01-01201310.1155/2013/141083141083Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative DiseasesGunnar K. Gouras0Department of Experimental Medical Science, Experimental Dementia Research Unit, Wallenberg Neuroscience Center, Lund University, 221 84 Lund, SwedenAge-related misfolding and aggregation of disease-linked proteins in selective brain regions is a characteristic of neurodegenerative diseases. Although neuropathological aggregates that characterize these various diseases are found at sites other than synapses, increasing evidence supports the idea that synapses are where the pathogenesis begins. Understanding these diseases is hampered by our lack of knowledge of what the normal functions of these proteins are and how they are affected by aging. Evidence has supported the idea that neurodegenerative disease-linked proteins have a common propensity for prion protein-like cell-to-cell propagation. However, it is not thought that the prion-like quality of these proteins/peptides that allows their cell-to-cell transmission implies a role for human-to-human spread in common age-related neurodegenerative diseases. It will be important to better understand the molecular and cellular mechanisms governing the role of these aggregating proteins in neural function, especially at synapses, how their propagation occurs and how pathogenesis is promoted by aging.http://dx.doi.org/10.1155/2013/141083
spellingShingle Gunnar K. Gouras
Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases
International Journal of Cell Biology
title Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases
title_full Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases
title_fullStr Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases
title_full_unstemmed Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases
title_short Convergence of Synapses, Endosomes, and Prions in the Biology of Neurodegenerative Diseases
title_sort convergence of synapses endosomes and prions in the biology of neurodegenerative diseases
url http://dx.doi.org/10.1155/2013/141083
work_keys_str_mv AT gunnarkgouras convergenceofsynapsesendosomesandprionsinthebiologyofneurodegenerativediseases