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引用次数: 0
摘要
在过去的 20 年中，人类单基因遗传疾病及其自身免疫表型被定义为 I 型干扰素（IFN）活性缺陷或增强的基础疾病。这些疾病描述了 I 型干扰素在自然条件下的影响，并证明只有 I 型干扰素活性的狭窄窗口才是有益的。I 型 IFN 不足易使人类患上危及生命的病毒性疾病（尽管数量出乎意料地少），而 I 型 IFN 在呼吸道和脑部病毒感染的免疫中起着核心作用。也许与直觉相反，I 型干扰素过多似乎是更多自身炎症和/或自身免疫疾病的基础，这些疾病被称为 I 型干扰素病，其研究揭示了 I 型干扰素信号诱导过程中涉及的多种分子程序。这些观察结果表明，将 I 型干扰素活性控制在生理范围内可能对预防和治疗病毒性和炎症性疾病有临床意义。
Human life within a narrow range: The lethal ups and downs of type I interferons
The past 20 years have seen the definition of human monogenic disorders and their autoimmune phenocopies underlying either defective or enhanced type I interferon (IFN) activity. These disorders delineate the impact of type I IFNs in natural conditions and demonstrate that only a narrow window of type I IFN activity is beneficial. Insufficient type I IFN predisposes humans to life-threatening viral diseases (albeit unexpectedly few) with a central role in immunity to respiratory and cerebral viral infection. Excessive type I IFN, perhaps counterintuitively, appears to underlie a greater number of autoinflammatory and/or autoimmune conditions known as type I interferonopathies, whose study has revealed multiple molecular programs involved in the induction of type I IFN signaling. These observations suggest that the manipulation of type I IFN activity to within a physiological range may be clinically relevant for the prevention and treatment of viral and inflammatory diseases.
期刊介绍：
Science Immunology is a peer-reviewed journal that publishes original research articles in the field of immunology. The journal encourages the submission of research findings from all areas of immunology, including studies on innate and adaptive immunity, immune cell development and differentiation, immunogenomics, systems immunology, structural immunology, antigen presentation, immunometabolism, and mucosal immunology. Additionally, the journal covers research on immune contributions to health and disease, such as host defense, inflammation, cancer immunology, autoimmunity, allergy, transplantation, and immunodeficiency. Science Immunology maintains the same high-quality standard as other journals in the Science family and aims to facilitate understanding of the immune system by showcasing innovative advances in immunology research from all organisms and model systems, including humans.
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