Marra Amanda N, Li Yue, Wingert Rebecca A
Department of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, University of Notre Dame, Notre Dame, IN, 46556, USA.
Genesis. 2016 Sep;54(9):457-69. doi: 10.1002/dvg.22957. Epub 2016 Jul 25.
Cilia arose early during eukaryotic evolution, and their structural components are highly conserved from the simplest protists to complex metazoan species. In recent years, the role of cilia in the ontogeny of vertebrate organs has received increasing attention due to a staggering correlation between human disease and dysfunctional cilia. In particular, the presence of cilia in both the developing and mature kidney has become a deep area of research due to ciliopathies common to the kidney, such as polycystic kidney disease (PKD). Interestingly, mutations in genes encoding proteins that localize to the cilia cause similar cystic phenotypes in kidneys of various vertebrates, suggesting an essential role for cilia in kidney organogenesis and homeostasis as well. Importantly, the genes so far identified in kidney disease have conserved functions across species, whose kidneys include both primary and motile cilia. Here, we aim to provide a comprehensive description of cilia and their role in kidney development, as well as highlight the usefulness of the zebrafish embryonic kidney as a model to further understand the function of cilia in kidney health.
纤毛在真核生物进化早期就已出现,其结构成分从最简单的原生生物到复杂的后生动物物种都高度保守。近年来,由于人类疾病与功能失调的纤毛之间存在惊人的相关性,纤毛在脊椎动物器官个体发育中的作用受到了越来越多的关注。特别是,由于肾脏常见的纤毛病,如多囊肾病(PKD),发育中的肾脏和成熟肾脏中纤毛的存在已成为一个深入的研究领域。有趣的是,编码定位于纤毛的蛋白质的基因突变在各种脊椎动物的肾脏中会导致类似的囊性表型,这表明纤毛在肾脏器官发生和体内平衡中也起着重要作用。重要的是,迄今为止在肾脏疾病中鉴定出的基因在不同物种中具有保守功能,这些物种的肾脏包括初级纤毛和运动纤毛。在这里,我们旨在全面描述纤毛及其在肾脏发育中的作用,并强调斑马鱼胚胎肾脏作为进一步了解纤毛在肾脏健康中功能的模型的有用性。