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利用秀丽隐杆线虫模拟自噬相关的人类疾病。

C. elegans to model autophagy-related human disorders.

作者信息

Wong Shi Quan, Kumar Anita V, Mills Joslyn, Lapierre Louis R

机构信息

Department of Molecular Biology, Cell Biology, and Biochemistry, Brown University, Providence, RI, United States.

Department of Molecular Biology, Cell Biology, and Biochemistry, Brown University, Providence, RI, United States.

出版信息

Prog Mol Biol Transl Sci. 2020;172:325-373. doi: 10.1016/bs.pmbts.2020.01.007. Epub 2020 Apr 6.

Abstract

Autophagy is a highly conserved degradation process that clears damaged intracellular macromolecules and organelles in order to maintain cellular health. Dysfunctional autophagy is fundamentally linked to the development of various human disorders and pathologies. The use of the nematode Caenorhabditis elegans as a model system to study autophagy has improved our understanding of its regulation and function in organismal physiology. Here, we review the genetic, functional, and regulatory conservation of the autophagy pathway in C. elegans and we describe tools to quantify and study the autophagy process in this incredibly useful model organism. We further discuss how these nematodes have been modified to model autophagy-related human diseases and underscore the important insights obtained from such models. Altogether, we highlight the strengths of C. elegans as an exceptional tool to understand the genetic and molecular foundations underlying autophagy-related human diseases.

摘要

自噬是一种高度保守的降解过程,可清除受损的细胞内大分子和细胞器,以维持细胞健康。自噬功能失调与多种人类疾病和病理状况的发生发展有着根本联系。利用线虫秀丽隐杆线虫作为模型系统来研究自噬,增进了我们对其在机体生理学中的调节和功能的理解。在此,我们综述了秀丽隐杆线虫自噬途径的遗传、功能和调节保守性,并描述了在这种极其有用的模式生物中量化和研究自噬过程的工具。我们进一步讨论了如何对这些线虫进行改造以模拟自噬相关的人类疾病,并强调了从这些模型中获得的重要见解。总之,我们突出了秀丽隐杆线虫作为理解自噬相关人类疾病潜在遗传和分子基础的卓越工具的优势。

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