The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases.
The Role of the Nasal Cavity in the Pathogenesis of Prion Diseases.
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DOI：
10.3390/v13112287
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发表时间：
2021-11-16
期刊：
影响因子：
--
通讯作者：
Kincaid AE
中科院分区：
文献类型：
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作者：
Kincaid AE
朊病毒病（Prion diseases），又称传染性海绵状脑病（transmissible spongiform encephalopathies，TSE），是一类由感染性朊病毒蛋白（infectious prion proteins，PrPSc）进入中枢神经系统（central nervous system，CNS）并扩散引起的致死性神经退行性疾病。这些疾病是某些哺乳动物物种特有的，这些哺乳动物物种将其嗅觉用于各种目的，因此将其鼻腔（NC）暴露于环境中的PrPSc。影响人类的朊病毒疾病要么是由于编码朊病毒蛋白的基因突变而遗传的，要么是由于暴露于受污染的组织或医疗器械而获得的，要么是在没有已知原因的情况下发展的（称为散发性）。本综述的目的是确定参与朊病毒转运的NC组分，并总结NC作为PrPSc进入途径（向心扩散）和/或脱落来源（离心扩散）的证据，从而在TSE的发病机制中发挥作用。
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a class of fatal neurodegenerative diseases caused by the entry and spread of infectious prion proteins (PrPSc) 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 PrPSc 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 sporadic). 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 PrPSc, and thus plays a role in the pathogenesis of the TSEs.