The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases

Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a class of fatal neurodegenerative diseases caused by the entry and spread of infectious prion proteins (PrP<sup>Sc</sup>) in the central nervous system (CNS). These diseases are endemic to certain mammalian animal...

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Autor principal:	Anthony E. Kincaid
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	PrP<sup>Sc</sup> TSE prions nasal cavity prion pathogenesis olfactory system Microbiology QR1-502
Acceso en línea:	https://doaj.org/article/469f616d9cdd4f35916ca85fc417d351
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spelling	oai:doaj.org-article:469f616d9cdd4f35916ca85fc417d3512021-11-25T19:14:12ZThe Role of the Nasal Cavity in the Pathogenesis of Prion Diseases10.3390/v131122871999-4915https://doaj.org/article/469f616d9cdd4f35916ca85fc417d3512021-11-01T00:00:00Zhttps://www.mdpi.com/1999-4915/13/11/2287https://doaj.org/toc/1999-4915Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a class of fatal neurodegenerative diseases caused by the entry and spread of infectious prion proteins (PrP<sup>Sc</sup>) in the central nervous system (CNS). These diseases are endemic to certain mammalian animal species that use their sense of smell for a variety of purposes and therefore expose their nasal cavity (NC) to PrP<sup>Sc</sup> in the environment. Prion diseases that affect humans are either inherited due to a mutation of the gene that encodes the prion protein, acquired by exposure to contaminated tissues or medical devices, or develop without a known cause (referred to as <i>sporadic</i>). The purpose of this review is to identify components of the NC that are involved in prion transport and to summarize the evidence that the NC serves as a route of entry (centripetal spread) and/or a source of shedding (centrifugal spread) of PrP<sup>Sc</sup>, and thus plays a role in the pathogenesis of the TSEs.Anthony E. KincaidMDPI AGarticlePrP<sup>Sc</sup>TSEprionsnasal cavityprion pathogenesisolfactory systemMicrobiologyQR1-502ENViruses, Vol 13, Iss 2287, p 2287 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	PrP<sup>Sc</sup> TSE prions nasal cavity prion pathogenesis olfactory system Microbiology QR1-502
spellingShingle	PrP<sup>Sc</sup> TSE prions nasal cavity prion pathogenesis olfactory system Microbiology QR1-502 Anthony E. Kincaid The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases
description	Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a class of fatal neurodegenerative diseases caused by the entry and spread of infectious prion proteins (PrP<sup>Sc</sup>) in the central nervous system (CNS). These diseases are endemic to certain mammalian animal species that use their sense of smell for a variety of purposes and therefore expose their nasal cavity (NC) to PrP<sup>Sc</sup> in the environment. Prion diseases that affect humans are either inherited due to a mutation of the gene that encodes the prion protein, acquired by exposure to contaminated tissues or medical devices, or develop without a known cause (referred to as <i>sporadic</i>). The purpose of this review is to identify components of the NC that are involved in prion transport and to summarize the evidence that the NC serves as a route of entry (centripetal spread) and/or a source of shedding (centrifugal spread) of PrP<sup>Sc</sup>, and thus plays a role in the pathogenesis of the TSEs.
format	article
author	Anthony E. Kincaid
author_facet	Anthony E. Kincaid
author_sort	Anthony E. Kincaid
title	The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases
title_short	The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases
title_full	The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases
title_fullStr	The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases
title_full_unstemmed	The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases
title_sort	role of the nasal cavity in the pathogenesis of prion diseases
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/469f616d9cdd4f35916ca85fc417d351
work_keys_str_mv	AT anthonyekincaid theroleofthenasalcavityinthepathogenesisofpriondiseases AT anthonyekincaid roleofthenasalcavityinthepathogenesisofpriondiseases
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The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases

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