Neurodegenerative diseases (NDD) are incurable and the most prevalent cognitive and motor disorders of elderly. Mitochondria are essential for a wide range of cellular processes playing a pivotal role in a number of cellular functions like metabolism, intracellular signaling, apoptosis, and immunity. A plethora of evidence indicates the central role of mitochondrial functions in pathogenesis of many aging related NDD. Considering how mitochondria function in neurodegenerative diseases, oxidative stress, and mutations in mtDNA both contribute to aging. Many substantial reports suggested the involvement of numerous contributing factors including, mitochondrial dysfunction, oxidative stress, mitophagy, accumulation of somatic mtDNA mutations, compromised mitochondrial dynamics, and transport within axons in neurodegenerative disorders including Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, and Amyotrophic Lateral Sclerosis. Therapies therefore target fundamental mitochondrial processes such as energy metabolism, free-radical generation, mitochondrial biogenesis, mitochondrial redox state, mitochondrial dynamics, mitochondrial protein synthesis, mitochondrial quality control, and metabolism hold great promise to develop pharmacological based therapies in NDD. By emphasizing the most efficient pharmacological strategies to target dysfunction of mitochondria in the treatment of neurodegenerative diseases, this review serves the scientific community engaged in translational medical science by focusing on the establishment of novel, mitochondria-targeted treatment strategies.

神经退行性疾病（NDD）是无法治愈的，也是老年人最常见的认知和运动障碍。线粒体对于多种细胞过程至关重要，在新陈代谢、细胞内信号传导、细胞凋亡和免疫等许多细胞功能中发挥着关键作用。大量证据表明线粒体功能在许多与衰老相关的 NDD 发病机制中发挥着核心作用。考虑到线粒体在神经退行性疾病中的功能，氧化应激和 mtDNA 突变都会导致衰老。许多实质性报告表明，许多促成因素参与其中，包括线粒体功能障碍、氧化应激、线粒体自噬、体细胞 mtDNA 突变的积累、线粒体动力学受损以及神经退行性疾病（包括阿尔茨海默病、帕金森病、亨廷顿病和肌萎缩侧索硬化症）中的轴突内运输硬化。因此，针对基本线粒体过程（如能量代谢、自由基生成、线粒体生物发生、线粒体氧化还原状态、线粒体动力学、线粒体蛋白质合成、线粒体质量控制和代谢）的治疗为开发基于药理学的 NDD 疗法带来了巨大希望。通过强调在神经退行性疾病治疗中针对线粒体功能障碍的最有效的药理学策略，本综述通过重点建立新颖的线粒体靶向治疗策略，为从事转化医学的科学界提供服务。