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在肌肉炎中,IFNγ会导致线粒体功能障碍和氧化应激

Catalina Abad¹, Iago Pinal-Fernandez^2,3, Clement Guillou⁴

¹Univ Rouen Normandie, Inserm, UMR1234, FOCIS Center of Excellence PAn'THER, F-76000, Rouen, France.

Nature communications

|June 26, 2024

概括

干扰素玛 (IFNγ) 通过引起线粒体功能障碍和氧化应激,驱动自身免疫性肌肉炎. 针对活性氧物种 (ROS) 或IFNγ可能为这种肌肉疾病提供新的治疗方法.

科学领域:

免疫学免疫学免疫学
线粒体生物学线粒体生物学
肌肉病理生理学肌肉病理生理学

背景情况:

异形性炎症性肌肉病变 (IIM) 是一种严重的自身免疫性疾病,其病因不明,治疗选择有限.
之前的研究表明,缺乏诱导性T细胞共刺激器 (Icos) 的小鼠会发展自发的干扰素马 (IFNγ) 驱动的神经炎,模仿人类的IIMs.

研究的目的:

研究IFNγ在IIM病原发生中的作用.
探索IFNγ,线粒体功能障碍和肌肉炎中的氧化应激之间的联系.

主要方法:

来自Icos缺陷小鼠肌肉组织的蛋白质和空间转录组分析.
评估线粒体结构和功能,以及氧化应激标志物.
用活性氧物种 (ROS) 缓冲器和抗IFNγ抗体进行治疗.
对 IIM 患者样本的转录组分析和体外人体肌肉细胞研究.

主要成果:

在Icos缺乏的小鼠中,IFNγ诱导了显著的肌纤维代谢失调,线粒体异常和氧化应激.
缓冲ROS缓解了肌肉炎,改善了线粒体功能,并减少了炎症.
抗IFNγ治疗减少了线粒体异常和氧化应激.
人类IIM数据证实了IFNγ和线粒体功能障碍之间的联系.

结论:

IFNγ在通过线粒体功能障碍和氧化应激驱动肌肉炎方面发挥着关键作用.
在线粒体功能障碍,ROS生产和肌肉炎炎炎症之间存在一个自我维持的循环.
准线粒体或ROS为肌肉炎提供了一个有前途的治疗策略.

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