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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| Formato: | article |
| Lenguaje: | EN |
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MDPI AG
2021
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| Acceso en línea: | https://doaj.org/article/469f616d9cdd4f35916ca85fc417d351 |
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| Sumario: | 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. |
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