Mitochondria, which are cell compartments that are widely present in eukaryotic cells, have been shown to be involved in a variety of synthetic, metabolic, and signaling processes, thereby playing a vital role in cells. The mitochondrial unfolded protein response (mtUPR) is a response in which mitochondria reverse the signal to the nucleus and maintain mitochondrial protein homeostasis when unfolded and misfolded proteins continue to accumulate. Multiple neurodegeneration diseases, including Alzheimer's disease (AD), Parkinson’s disease (PD), and familial amyotrophic lateral sclerosis (fALS), are public health challenges. Every year, countless efforts are expended trying to clarify the pathogenesis and treatment of neurological disorders, which are associated with mitochondrial dysfunction to some extent. Numerous studies have shown that mtUPR is involved in and plays an important role in the pathogenesis of neurological disorders, but the exact mechanism of the disorders is still unclear. Further study of the process of mtUPR in neurological disorders can help us more accurately understand their pathogenesis in order to provide new therapeutic targets. In this paper, we briefly review mtUPR signaling in Caenorhabditis elegans (C. elegans) and mammals and summarize the role of mtUPR in neurodegeneration diseases, including AD, PD and fALS.

线粒体是真核细胞中广泛存在的细胞区室，已被证明参与多种合成，代谢和信号传导过程，从而在细胞中起着至关重要的作用。线粒体未折叠蛋白应答（mtUPR）是一种响应，其中当未折叠和错误折叠的蛋白继续积累时，线粒体将信号逆转至细胞核并保持线粒体蛋白稳态。公共卫生挑战是多种神经退行性疾病，包括阿尔茨海默氏病（AD），帕金森氏病（PD）和家族性肌萎缩性侧索硬化症（fALS）。每年，为了阐明与线粒体功能障碍有关的神经系统疾病的发病机理和治疗方法，人们付出了无数的努力。大量研究表明，mtUPR参与神经系统疾病的发病机理并在其中发挥重要作用，但该疾病的确切机制仍不清楚。进一步研究mtUPR在神经系统疾病中的过程可以帮助我们更准确地了解其发病机制，从而提供新的治疗靶标。在本文中，我们简要回顾了mtUPR信号秀丽隐杆线虫（秀丽隐杆线虫）和哺乳动物，并总结了mtUPR在包括AD，PD和fALS在内的神经退行性疾病中的作用。