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Microglia in prion diseases
The Journal of Clinical Investigation ( IF 15.9 ) Pub Date : 2017-07-17 , DOI: 10.1172/jci90605
Adriano Aguzzi , Caihong Zhu

Prion diseases are a group of progressive and fatal neurodegenerative disorders characterized by deposition of scrapie prion protein (PrPSc) in the CNS. This deposition is accompanied by neuronal loss, spongiform change, astrogliosis, and conspicuous microglial activation. Here, we argue that microglia play an overall neuroprotective role in prion pathogenesis. Several microglia-related molecules, such as Toll-like receptors (TLRs), the complement system, cytokines, chemokines, inflammatory regulators, and phagocytosis mediators, are involved in prion pathogenesis. However, the molecular mechanisms underlying the microglial response to prion infection are largely unknown. Consequently, we lack a comprehensive understanding of the regulatory network of microglial activation. On the positive side, recent findings suggest that therapeutic strategies modulating microglial activation and function may have merit in prion disease. Moreover, studies on the role of microglia in prion disease could deepen our understanding of neuroinflammation in a broad range of neurodegenerative disorders.



中文翻译:

小胶质细胞在pr病毒疾病中

on病毒疾病是一组进行性和致命性神经退行性疾病,其特征在于瘙痒病毒蛋白(PrPSc)在中枢神经系统中的沉积。这种沉积伴有神经元丢失,海绵状变化,星形胶质细胞增生和明显的小胶质细胞活化。在这里,我们认为小胶质细胞在病毒的发病机理中起着整体的神经保护作用。几种小胶质细胞相关分子,例如Toll样受体(TLR),补体系统,细胞因子,趋化因子,炎症调节剂和吞噬作用介质,都与in病毒的发病机理有关。但是,小胶质细胞对病毒感染的潜在分子机制尚不清楚。因此,我们对小胶质细胞激活的监管网络缺乏全面的了解。从积极的一面来看,最近的发现表明,调节小胶质细胞活化和功能的治疗策略可能在病毒疾病中具有优势。此外,关于小胶质细胞在病毒疾病中的作用的研究可以加深我们对广泛的神经退行性疾病中神经炎症的理解。

更新日期:2017-09-08
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