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自噬与神经退行性疾病:斑马鱼模型系统。

Mitophagy and neurodegeneration: the zebrafish model system.

机构信息

Department of Comparative Biomedical Sciences; The Royal Veterinary College; University of London; London, UK; Department of Pharmacology; University College London School of Pharmacy; London, UK.

出版信息

Autophagy. 2013 Nov 1;9(11):1693-709. doi: 10.4161/auto.25082. Epub 2013 Aug 8.

Abstract

Autophagy is responsible for the degradation of cytoplasmic components and organelles such as mitochondria. The selective degradation of damaged mitochondria by autophagy is termed mitophagy, and is an important quality control mechanism. Neurons, being highly specialized cells, are particularly susceptible to defects of autophagy. Impairments in mitochondrial function and their dynamics are present in many neurodegenerative diseases, and modulators of both mitochondrial physiology and autophagy present themselves as promising therapeutic targets. Zebrafish are now established as a valuable tool for disease modeling. A wide variety of genetic and molecular techniques can be employed to highlight pathogenic processes and dissect disease pathways. This review will explore the role that zebrafish have so far played in our understanding of mitophagy in neurodegeneration, and will discuss how they might be used to drive the wider mitophagy field forward.

摘要

自噬负责细胞质成分和细胞器(如线粒体)的降解。自噬对受损线粒体的选择性降解称为线粒体自噬,是一种重要的质量控制机制。神经元作为高度特化的细胞,特别容易受到自噬缺陷的影响。许多神经退行性疾病中存在线粒体功能和动力学的损伤,线粒体生理学和自噬的调节剂成为有前途的治疗靶点。斑马鱼现已成为疾病建模的有用工具。可以采用多种遗传和分子技术来突出发病过程并剖析疾病途径。本综述将探讨斑马鱼在我们对神经退行性变中线粒体自噬的理解中所起的作用,并讨论如何利用它们推动更广泛的线粒体自噬领域的发展。

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