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Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination

Prion disorders are infectious, neurodegenerative diseases that affect humans and animals. Susceptibility to some prion diseases such as kuru or the new variant of Creutzfeldt-Jakob disease in humans and scrapie in sheep and goats is influenced by polymorphisms of the coding region of the prion prot...

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Detalles Bibliográficos
Autores principales: Sisó, Sílvia, González, Lorenzo, Jeffrey, Martin
Formato: Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2905956/
https://www.ncbi.nlm.nih.gov/pubmed/20652006
http://dx.doi.org/10.1155/2010/747892
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author Sisó, Sílvia
González, Lorenzo
Jeffrey, Martin
author_facet Sisó, Sílvia
González, Lorenzo
Jeffrey, Martin
author_sort Sisó, Sílvia
collection PubMed
description Prion disorders are infectious, neurodegenerative diseases that affect humans and animals. Susceptibility to some prion diseases such as kuru or the new variant of Creutzfeldt-Jakob disease in humans and scrapie in sheep and goats is influenced by polymorphisms of the coding region of the prion protein gene, while other prion disorders such as fatal familial insomnia, familial Creutzfeldt-Jakob disease, or Gerstmann-Straussler-Scheinker disease in humans have an underlying inherited genetic basis. Several prion strains have been demonstrated experimentally in rodents and sheep. The progression and pathogenesis of disease is influenced by both genetic differences in the prion protein and prion strain. Some prion diseases only affect the central nervous system whereas others involve the peripheral organs prior to neuroinvasion. Many experiments undertaken in different species and using different prion strains have postulated common pathways of neuroinvasion. It is suggested that prions access the autonomic nerves innervating peripheral organs and tissues to finally reach the central nervous system. We review here published data supporting this view and additional data suggesting that neuroinvasion may concurrently or independently involve the blood vascular system.
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spelling pubmed-29059562010-07-22 Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination Sisó, Sílvia González, Lorenzo Jeffrey, Martin Interdiscip Perspect Infect Dis Review Article Prion disorders are infectious, neurodegenerative diseases that affect humans and animals. Susceptibility to some prion diseases such as kuru or the new variant of Creutzfeldt-Jakob disease in humans and scrapie in sheep and goats is influenced by polymorphisms of the coding region of the prion protein gene, while other prion disorders such as fatal familial insomnia, familial Creutzfeldt-Jakob disease, or Gerstmann-Straussler-Scheinker disease in humans have an underlying inherited genetic basis. Several prion strains have been demonstrated experimentally in rodents and sheep. The progression and pathogenesis of disease is influenced by both genetic differences in the prion protein and prion strain. Some prion diseases only affect the central nervous system whereas others involve the peripheral organs prior to neuroinvasion. Many experiments undertaken in different species and using different prion strains have postulated common pathways of neuroinvasion. It is suggested that prions access the autonomic nerves innervating peripheral organs and tissues to finally reach the central nervous system. We review here published data supporting this view and additional data suggesting that neuroinvasion may concurrently or independently involve the blood vascular system. Hindawi Publishing Corporation 2010 2010-06-23 /pmc/articles/PMC2905956/ /pubmed/20652006 http://dx.doi.org/10.1155/2010/747892 Text en Copyright © 2010 Sílvia Sisó et al. 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
Sisó, Sílvia
González, Lorenzo
Jeffrey, Martin
Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination
title Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination
title_full Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination
title_fullStr Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination
title_full_unstemmed Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination
title_short Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination
title_sort neuroinvasion in prion diseases: the roles of ascending neural infection and blood dissemination
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2905956/
https://www.ncbi.nlm.nih.gov/pubmed/20652006
http://dx.doi.org/10.1155/2010/747892
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