| 摘要: |
| [摘要] 先天免疫作为机体抵御病原体入侵的第一道防线,在维持免疫系统稳态、识别并清除入侵病原体方面发挥重要作用。然而,在无菌条件下,先天免疫异常激活可能引发自身炎症性疾病。慢性-复发性-多灶性-溶骨性骨髓炎(CRMO)作为自身炎症性疾病的典型代表,在临床诊疗方面仍存在诸多亟待解决的基础理论和临床管理问题。CRMO小鼠模型Pstpip2-/-在自身炎症性疾病和CRMO研究领域中被广泛应用,并加深针对CRMO疾病在分子细胞水平的调控机制的理解。该文总结了一般性自身炎症性疾病的临床表现特征和分类,重点探讨膜结构调控蛋白PSTPIP2在CRMO Pstpip2-/-小鼠模型中的作用,旨在为解析溶骨性骨髓炎的发病机制及制订治疗策略提供参考。 |
| 关键词: 固有免疫 自身炎症性疾病 溶骨性骨髓炎 膜结构调控蛋白PSTPIP2 |
| DOI:10.3969/j.issn.1674-3806.2026.02.09 |
| 分类号:R 593 |
| 基金项目:国家自然科学基金项目(编号:82170173,82371789,U25A2050);天津市教委科研计划项目(编号:2022KJ194) |
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| Research progress in the autoinflammatory disease osteolytic osteomyelitis and membrane-regulating protein PSTPIP2 |
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HUO Qingran1,2,3, DONG Ge1,2,3, CAI Zhigang1,2,3
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1.Department of Pharmacology, School of Basic Medical Science, Tianjin Medical University, Tianjin 300070, China; 2.State Key Laboratory of Experimental Hematology, Tianjin 300070, China; 3.Tianjin Key Laboratory of Inflammatory Biology, Tianjin 300070, China
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| Abstract: |
| [Abstract] Innate immunity, as the first line of defense for the body against the invasion of pathogens, plays a significant role in maintaining the homeostasis of the immune system, and in recognizing and eliminating the invading pathogens. However, under aseptic conditions, the abnormal activation of innate immunity may induce autoinflammatory diseases. Chronic recurrent multifocal osteomyelitis(CRMO), as a typical representative of autoinflammatory diseases, still has many fundamental theoretical and clinical management problems that need to be urgently solved in clinical diagnosis and treatment. The Pstpip2-/- mouse model is widely used in the research fields of autoinflammatory diseases and CRMO, which facilitates the understanding of the regulatory mechanisms of CRMO at the molecular and cellular levels. This paper summarizes the clinical manifestations and classifications of general autoinflammatory diseases, with a focus on discussing the role of membrane-regulating protein proline-serine-threonine-phosphatase-interacting protein 2(PSTPIP2) in the Pstpip2-/- mouse model of CRMO, aiming to provide a reference for analyzing the pathogenesis of osteolytic osteomyelitis and formulating therapeutic strategies. |
| Key words: Innate immunity Autoinflammatory diseases Osteolytic osteomyelitis Membrane-regulating protein proline-serine-threonine-phosphatase-interacting protein 2(PSTPIP2) |