Department of Biological Sciences, University of Notre Dame, Notre Dame, USA.
Tissue Barriers. 2024 Oct;12(4):2309025. doi: 10.1080/21688370.2024.2309025. Epub 2024 Jan 28.
Cilia are hair-like structures found on the surface of nearly all vertebrate cell types where they have central roles in regulating development and orchestrating physiological events. There is growing interest in understanding the mechanisms of ciliogenesis due to the profound consequences that follow from the absence of proper ciliary function, which include diseases that affect the renal, respiratory, reproductive, nervous, visual, and digestive systems, among others. Now, a recent report has discerned new roles for the transcription factor ( in ciliated cell ontogeny within the embryonic zebrafish kidney and other tissues. Further, the team of researchers discovered that genetic ablation of murine homolog ERRγ in adult kidney epithelial cells led to shortened cilia, which precedes cystogenesis. These intriguing findings expand our fundamental understanding of the pathological basis of cilia defects, which is relevant for identifying future therapeutic targets for ciliopathies.
纤毛是几乎所有脊椎动物细胞表面都存在的毛发状结构,在调节发育和协调生理事件方面发挥着核心作用。由于缺乏适当的纤毛功能会带来深远的后果,包括影响肾脏、呼吸系统、生殖系统、神经系统、视觉系统和消化系统等疾病,因此人们越来越感兴趣于了解纤毛发生的机制。现在,最近的一份报告在胚胎斑马鱼肾脏和其他组织的纤毛细胞发生中发现了转录因子 (的新作用。此外,研究小组还发现,在成年肾脏上皮细胞中遗传消融鼠同源 ERRγ 导致纤毛缩短,这先于囊肿发生。这些有趣的发现扩展了我们对纤毛缺陷的病理基础的基本认识,这对于确定纤毛病的未来治疗靶点具有重要意义。