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CNS disease associated with enhanced type I interferon signalling
The Lancet Neurology ( IF 46.5 ) Pub Date : 2024-10-16 , DOI: 10.1016/s1474-4422(24)00263-1
Yanick J Crow

The ability to mount an interferon-mediated innate immune response is essential in protection against neurotropic viruses, but antiviral type I interferons also have neurotoxic potential. The production of type I interferons can be triggered by self-derived nucleic acids, and the brain can be susceptible to inappropriate upregulation of type I interferon signalling. Homoeostatic dysregulation of type I interferons has been implicated in rare inborn errors of immunity (referred to as type I interferonopathies) and more common neurodegenerative disorders (eg, Parkinson's disease, Alzheimer's disease, and amyotrophic lateral sclerosis). Recent developments include new insights into the pathogenesis of these disorders that involve dysregulated type I interferon signalling, as well as advances in their diagnosis and management. The role of type I interferons in brain cellular health suggests the future therapeutic potential of approaches that target these interferons and their signalling.

中文翻译:


与 I 型干扰素信号增强相关的 CNS 疾病



产生干扰素介导的先天免疫反应的能力对于抵御嗜神经病毒至关重要,但抗病毒 I 型干扰素也具有神经毒性潜力。I 型干扰素的产生可由自身来源的核酸触发,大脑易受 I 型干扰素信号的不适当上调的影响。I 型干扰素的稳态失调与罕见的先天性免疫缺陷(称为 I 型干扰素病)和更常见的神经退行性疾病(如帕金森病、阿尔茨海默病和肌萎缩侧索硬化症)有关。最近的进展包括对这些涉及 I 型干扰素信号失调的疾病发病机制的新见解,以及其诊断和管理的进展。I 型干扰素在脑细胞健康中的作用表明了针对这些干扰素及其信号传导的方法的未来治疗潜力。
更新日期:2024-10-17
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