Abstract：
Mitochondria are singular cell organelles essential for many cellular functions, which includes responding to stress, regulating calcium levels, maintaining protein homeostasis, and coordinating apoptosis response. The vitality of cells, therefore, hinges on the optimal functioning of these dynamic organelles. Mitochondrial Quality Control Mechanisms (MQCM) play a pivotal role in ensuring the integrity and functionality of mitochondria. Perturbations in these mechanisms have been closely associated with the pathogenesis of neurodegenerative disorders such as Parkinson\'s disease, Alzheimer\'s disease, Huntington\'s disease, and amyotrophic lateral sclerosis. Compelling evidence suggests that targeting specific pathways within the MQCM could potentially offer a therapeutic avenue for rescuing mitochondrial integrity and mitigating the progression of neurodegenerative diseases. The intricate interplay of cellular stress, protein misfolding, and impaired quality control mechanisms provides a nuanced understanding of the underlying pathology. Consequently, unravelling the specific MQCM dysregulation in neurodegenerative disorders becomes paramount for developing targeted therapeutic strategies. This review delves into the impaired MQCM pathways implicated in neurodegenerative disorders and explores emerging therapeutic interventions. By shedding light on pharmaceutical and genetic manipulations aimed at restoring MQCM efficiency, the discussion aims to provide insights into novel strategies for ameliorating the progression of neurodegenerative diseases. Understanding and addressing mitochondrial quality control mechanisms not only underscore their significance in cellular health but also offer a promising frontier for advancing therapeutic approaches in the realm of neurodegenerative disorders.
摘要：
线粒体是许多细胞功能所必需的单个细胞器，其中包括对压力的反应，调节钙水平，维持蛋白质稳态，协调细胞凋亡反应。细胞的活力，因此,取决于这些动态细胞器的最佳功能。线粒体质量控制机制（MQCM）在确保线粒体的完整性和功能性中起着关键作用。这些机制的干扰与帕金森病等神经退行性疾病的发病机制密切相关。老年痴呆症,亨廷顿病,和肌萎缩侧索硬化症.令人信服的证据表明，靶向MQCM内的特定途径可能为挽救线粒体完整性和减轻神经退行性疾病的进展提供治疗途径。细胞压力的复杂相互作用，蛋白质折叠错误，受损的质量控制机制提供了对潜在病理的细致理解。因此,解开神经退行性疾病中特定的MQCM失调对于制定有针对性的治疗策略至关重要。这篇综述深入研究了与神经退行性疾病有关的MQCM通路受损，并探讨了新兴的治疗干预措施。通过阐明旨在恢复MQCM效率的药物和遗传操作，该讨论旨在为改善神经退行性疾病进展的新策略提供见解。理解和解决线粒体质量控制机制不仅强调了它们在细胞健康中的重要性，而且为推进神经退行性疾病领域的治疗方法提供了有希望的前沿。
