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C. elegans to model autophagy-related human disorders.
Progress in Molecular Biology and Translational Science Pub Date : 2020-04-06 , DOI: 10.1016/bs.pmbts.2020.01.007
Shi Quan Wong 1 , Anita V Kumar 1 , Joslyn Mills 1 , Louis R Lapierre 1
Affiliation  

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.



中文翻译:

秀丽隐杆线虫模拟自噬相关的人类疾病。

自噬是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。功能失调的自噬从根本上与人类各种疾病和病理的发展有关。使用线虫秀丽隐杆线虫作为模型系统来研究自噬已经改善了我们对其调节和在生物生理中的功能的理解。在这里,我们审查了秀丽隐杆线虫自噬途径的遗传,功能和法规守恒我们描述了在这种极其有用的模型生物中量化和研究自噬过程的工具。我们进一步讨论如何修改这些线虫以模拟自噬相关的人类疾病,并强调从此类模型获得的重要见解。总之,我们着重指出秀丽隐杆线虫作为了解自噬相关人类疾病的遗传和分子基础的特殊工具的优势。

更新日期:2020-04-06
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