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我们能从蝌蚪身上了解到的关于纤毛病和气道疾病的知识:利用非洲爪蟾的系统生物学研究纤毛和黏液纤毛上皮。

What we can learn from a tadpole about ciliopathies and airway diseases: Using systems biology in Xenopus to study cilia and mucociliary epithelia.

作者信息

Walentek Peter, Quigley Ian K

机构信息

Department of Molecular and Cell Biology; Genetics, Genomics and Development Division; Developmental and Regenerative Biology Group, University of California, Berkeley, California, 94720.

Salk Institute for Biological Studies, La Jolla, California, 92037.

出版信息

Genesis. 2017 Jan;55(1-2). doi: 10.1002/dvg.23001.

DOI:10.1002/dvg.23001
PMID:28095645
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5276738/
Abstract

Over the past years, the Xenopus embryo has emerged as an incredibly useful model organism for studying the formation and function of cilia and ciliated epithelia in vivo. This has led to a variety of findings elucidating the molecular mechanisms of ciliated cell specification, basal body biogenesis, cilia assembly, and ciliary motility. These findings also revealed the deep functional conservation of signaling, transcriptional, post-transcriptional, and protein networks employed in the formation and function of vertebrate ciliated cells. Therefore, Xenopus research can contribute crucial insights not only into developmental and cell biology, but also into the molecular mechanisms underlying cilia related diseases (ciliopathies) as well as diseases affecting the ciliated epithelium of the respiratory tract in humans (e.g., chronic lung diseases). Additionally, systems biology approaches including transcriptomics, genomics, and proteomics have been rapidly adapted for use in Xenopus, and broaden the applications for current and future translational biomedical research. This review aims to present the advantages of using Xenopus for cilia research, highlight some of the evolutionarily conserved key concepts and mechanisms of ciliated cell biology that were elucidated using the Xenopus model, and describe the potential for Xenopus research to address unresolved questions regarding the molecular mechanisms of ciliopathies and airway diseases.

摘要

在过去几年中,非洲爪蟾胚胎已成为一种极其有用的模式生物,用于在体内研究纤毛和纤毛上皮的形成与功能。这带来了一系列发现,阐明了纤毛细胞特化、基体生物发生、纤毛组装和纤毛运动的分子机制。这些发现还揭示了脊椎动物纤毛细胞形成和功能中所采用的信号传导、转录、转录后和蛋白质网络在功能上的深度保守性。因此,非洲爪蟾研究不仅能为发育生物学和细胞生物学提供关键见解,还能为纤毛相关疾病(纤毛病)以及影响人类呼吸道纤毛上皮的疾病(如慢性肺部疾病)的分子机制提供重要认识。此外,包括转录组学、基因组学和蛋白质组学在内的系统生物学方法已迅速适用于非洲爪蟾研究,并拓宽了当前和未来转化生物医学研究的应用范围。本综述旨在介绍使用非洲爪蟾进行纤毛研究的优势,突出一些利用非洲爪蟾模型阐明的纤毛细胞生物学中进化保守的关键概念和机制,并描述非洲爪蟾研究在解决有关纤毛病和气道疾病分子机制的未解决问题方面的潜力。

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本文引用的文献

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Role of epidermal cilia in development of the Australian lungfish, Neoceratodus forsteri (Osteichthyes: Dipnoi).表皮纤毛在澳大利亚肺鱼(Neoceratodus forsteri)(硬骨鱼纲:角齿鱼目)发育中的作用。
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Ciliary transcription factors and miRNAs precisely regulate Cp110 levels required for ciliary adhesions and ciliogenesis.睫状转录因子和微小RNA精确调节睫状黏附及纤毛发生所需的Cp110水平。
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The ciliopathy-associated CPLANE proteins direct basal body recruitment of intraflagellar transport machinery.与纤毛病相关的CPLANE蛋白指导鞭毛内运输机制的基体募集。
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p73 Is Required for Multiciliogenesis and Regulates the Foxj1-Associated Gene Network.多纤毛形成需要p73并调节与Foxj1相关的基因网络。
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