Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis.
Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis.
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DOI：
10.1155/2013/746845
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发表时间：
2013-01-01
期刊：
影响因子：
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通讯作者：
Raoul, Cedric
中科院分区：
文献类型：
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作者：
Aebischer, Julianne;Bernard-Marissal, Nathalie;Raoul, Cedric
虽然对死亡受体的研究长期以来一直局限于免疫细胞，但过去十年已经提供了强有力的证据，证明它们与神经元死亡以及神经退行性疾病（如肌萎缩侧索硬化症（ALS））有关。ALS是一种致命的麻痹性疾病，主要影响大脑和脊髓中的运动神经元。与星形胶质细胞和小胶质细胞活化以及免疫细胞浸润相关的神经炎症过程伴随运动神经元变性，并支持疾病中非细胞自主机制的贡献。在动物模型和ALS患者中均观察到Fas、TNFR、LT-betaR和p75（NTR）信号传导的标志。本文综述了死亡受体在ALS中的作用以及运动神经元的选择性丧失与神经炎症之间的联系。它进一步表明了如何将最近的证据纳入最终的多方法治疗患者。
While studies on death receptors have long been restricted to immune cells, the last decade has provided a strong body of evidence for their implication in neuronal death and hence neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS). ALS is a fatal paralytic disorder that primarily affects motoneurons in the brain and spinal cord. A neuroinflammatory process, associated with astrocyte and microglial activation as well as infiltration of immune cells, accompanies motoneuron degeneration and supports the contribution of non-cell-autonomous mechanisms in the disease. Hallmarks of Fas, TNFR, LT-betaR, and p75(NTR) signaling have been observed in both animal models and ALS patients. This review summarizes to date knowledge of the role of death receptors in ALS and the link existing between the selective loss of motoneurons and neuroinflammation. It further suggests how this recent evidence could be included in an ultimate multiapproach to treat patients.