The mitochondrial unfolded protein response (UPRmt) is rapidly gaining attention. While the CHOP (ATF4/5) axis of the UPRmt was the first to be described, other axes have subsequently been reported. Validation of this complex pathway in C. elegans has been extensively studied. However, validation of the UPRmt in mouse models of disease known to implicate mitochondrial reprogramming or dysfunction, such as cancer and neurodegeneration, respectively, is only beginning to emerge. This review summarizes recent findings and highlights the major role of the superoxide dismutase SOD1 in the communication between the mitochondria and the nucleus in these settings. While SOD1 has mostly been studied in the context of familial amyotrophic lateral sclerosis (fALS), recent studies suggest that SOD1 may be a potentially important mediator of the UPRmt and converge to emphasize an increasingly vital role of SOD1 as a therapeutic target in cancer.

中文翻译：

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SOD1与线粒体UPR在癌症和神经退行性变中的串扰。

线粒体展开的蛋白质反应（UPRmt）迅速引起人们的注意。虽然UPRmt的CHOP（ATF4 / 5）轴是第一个被描述的轴，但随后还报道了其他轴。秀丽隐杆线虫中这种复杂途径的验证已被广泛研究。但是，UPRmt在已知分别牵涉线粒体重编程或功能障碍的疾病（例如分别为癌症和神经退行性疾病）的小鼠模型中的验证才刚刚开始。这篇综述总结了最近的发现，并强调了在这些环境中超氧化物歧化酶SOD1在线粒体和细胞核之间的通讯中的主要作用。尽管SOD1大多是在家族性肌萎缩性侧索硬化症（fALS）的背景下进行研究的，