Transport of Prions in the Peripheral Nervous System: Pathways, Cell Types, and Mechanisms.

Transport of Prions in the Peripheral Nervous System: Pathways, Cell Types, and Mechanisms.
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朊病毒在外周神经系统中的转运:途径、细胞类型和机制。

DOI:
10.3390/v14030630
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发表时间:
2022-03-18
期刊:
Viruses
影响因子:
--
通讯作者:
Bartz JC
Bartz JC
中科院分区:
其他
文献类型:
--
作者:
Koshy SM;Kincaid AE;Bartz JC

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朊病毒疾病是发生在动物和人类中的可传播的蛋白质错误折叠疾病,其中内源性朊病毒蛋白PrPC经历构象变化成为称为PrPSc的自模板聚集体。PrPSc在中枢神经系统(CNS)中的形成导致神经胶质增生、海绵状组织增生和细胞功能障碍,最终导致宿主死亡。朊病毒通过神经解剖学网络从外周接种部位扩散到中枢神经系统结构。虽然已经确定内源性PrPC是朊病毒形成所必需的,并且朊病毒扩散的速率与缓慢的轴突运输一致,但PrPSc运输的机制细节仍然难以捉摸。目前的研究工作主要集中在与轴突相关的朊病毒运输的细胞机制。这包括阐明所涉及的特定细胞类型,亚细胞机制和在此过程中存在的潜在辅因子。
Prion diseases are transmissible protein misfolding disorders that occur in animals and humans where the endogenous prion protein, PrPC, undergoes a conformational change into self-templating aggregates termed PrPSc. Formation of PrPSc in the central nervous system (CNS) leads to gliosis, spongiosis, and cellular dysfunction that ultimately results in the death of the host. The spread of prions from peripheral inoculation sites to CNS structures occurs through neuroanatomical networks. While it has been established that endogenous PrPC is necessary for prion formation, and that the rate of prion spread is consistent with slow axonal transport, the mechanistic details of PrPSc transport remain elusive. Current research endeavors are primarily focused on the cellular mechanisms of prion transport associated with axons. This includes elucidating specific cell types involved, subcellular machinery, and potential cofactors present during this process.
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