朊病毒疾病与内质网应激途径的相关性及治疗追求

IF 0.7 Cell Pathology Pub Date : 2017-08-28 DOI:10.1515/ersc-2017-0003
Tarah Satterfield, J. Pritchett, Sarah Cruz, Kyeorda Kemp
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引用次数: 3

摘要

背景:传染性海绵状脑病是一种罕见的神经退行性疾病,其特征是神经元细胞丧失、星形细胞增多和斑块形成。这些疾病的病原体被认为是异常折叠的朊病毒,其特征是从正常的细胞朊病毒蛋白(PrPc)到异常形式(PrPTSE)的构象变化。尽管有证据表明正常的朊病毒蛋白会导致这些疾病。未折叠蛋白反应是一系列与内质网(ER)中未折叠蛋白积累有关的解决应激的保守途径,已被证明在调节朊病毒疾病的发展中发挥作用。方法:本综述根据与朊病毒蛋白合成和转化相关的当前研究的相关性选择论文,确定朊病毒疾病与内质网应激之间的各种联系,并报道与内质网应激和朊病毒疾病相关的当前和潜在治疗方法。结论:了解朊病毒的形成机制,内质网应激与朊病毒疾病的进展有关,对朊病毒疾病的治疗具有重要意义。因此,靶向内质网或内质网中参与应激反应的途径可能有助于我们治疗朊病毒疾病。
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Prion disease and endoplasmic reticulum stress pathway correlations and treatment pursuits
Abstract Background: Transmissible spongiform encephalopathies are a collection of rare neurodegenerative disorders characterized by loss of neuronal cells, astrocytosis, and plaque formation. The causative agent of these diseases is thought to be abnormally folded prions and is characterized by a conformational change from normal, cellular prion protein (PrPc) to the abnormal form (PrPTSE). Although, there is evidence that normal prion protein can contribute to these disorders. The unfolded protein response, a conserved series of pathways involved in resolving stress associated with unfolded protein accumulation in the Endoplasmic Reticulum (ER), has been shown to play a role in regulating the development of prion diseases. Methods: This review chose papers based on their relevance to current studies involved in prion protein synthesis and transformation, identifies various links between prion diseases and ER stress, and reports on current and potential treatments as they relate to ER stress and prion diseases. Conclusion: For the advancement of prion disease treatment, it is important to understand the mechanisms involved in prion formation, and ER stress is implicated in prion disease progression. Therefore, targeting the ER or pathways involved in response to stress in the ER may help us treat prion diseases.
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