Department of Neuroscience of Disease, Brain Research Institute, Niigata University, Niigata 951-8585, Japan.
Int J Mol Sci. 2023 Apr 11;24(8):7079. doi: 10.3390/ijms24087079.
Neurodegenerative disorders are characterized by the progressive loss of neuronal structure or function, resulting in memory loss and movement disorders. Although the detailed pathogenic mechanism has not been elucidated, it is thought to be related to the loss of mitochondrial function in the process of aging. Animal models that mimic the pathology of a disease are essential for understanding human diseases. In recent years, small fish have become ideal vertebrate models for human disease due to their high genetic and histological homology to humans, ease of in vivo imaging, and ease of genetic manipulation. In this review, we first outline the impact of mitochondrial dysfunction on the progression of neurodegenerative diseases. Then, we highlight the advantages of small fish as model organisms, and present examples of previous studies regarding mitochondria-related neuronal disorders. Lastly, we discuss the applicability of the turquoise killifish, a unique model for aging research, as a model for neurodegenerative diseases. Small fish models are expected to advance our understanding of the mitochondrial function in vivo, the pathogenesis of neurodegenerative diseases, and be important tools for developing therapies to treat diseases.
神经退行性疾病的特征是神经元结构或功能的进行性丧失，导致记忆丧失和运动障碍。虽然详细的发病机制尚未阐明，但据认为与衰老过程中线粒体功能的丧失有关。模拟疾病病理学的动物模型对于理解人类疾病至关重要。近年来，小鱼由于与人类具有高度的遗传和组织学同源性、易于进行体内成像以及易于进行基因操作，已成为人类疾病的理想脊椎动物模型。在这篇综述中，我们首先概述了线粒体功能障碍对神经退行性疾病进展的影响。然后，我们强调了小鱼作为模型生物的优势，并介绍了以前关于与线粒体相关的神经元疾病的研究实例。最后，我们讨论了作为衰老研究独特模型的 turquoise killifish 作为神经退行性疾病模型的适用性。小鱼模型有望促进我们对体内线粒体功能、神经退行性疾病发病机制的理解，并成为治疗疾病的重要工具。
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