排序方式: 共有1条查询结果,搜索用时 437 毫秒
1
1.
Sarmed H. Kathem Ashraf M. Mohieldin Shakila Abdul-Majee Sajida H. Ismail Qaiss H. Altaei Ibrahim K. Alshimmari Mohanned M. Alsaidi Hussein Khammas Andromeda M. Nauli Bina Joe Surya M. Nauli 《老年心脏病学杂志》2014,11(1):63-73
Background Ciliopathies are a group of diseases associated with abnormal structure or function of primary cilia. Ciliopathies include polycystic kidney disease (PKD), a pathology associated with vascular hypertension. We previously showed that cilia length regulates cilia function, and cilia function is required for nitric oxide (NO) biosynthesis in endothelial cells. Because patients with PKD show abnormal sensory cilia function, the aim of our current study was to search for a targeted therapy focused on primary cilia, which we refer to as 'cilio- therapy'. Methods and Results In the present studies, our in vitro analyses refined fenoldopam as an equipotent and more specific dopa- minergic agonist to regulate cilia length and function. Our in vivo studies indicated that fenoldopam increased cilia length and serum NO thereby reducing blood pressure in a PKD mouse model. Our crossover, multicenter, double-blind and placebo-controlled clinical study further indicated that cilia-targeting therapy showed an overall reduction in mean arterial pressure in PKD patients. Conclusions Overall, our studies provide the first evidence of ciliotherapy as an innovative intervention in patients with abnormal primary cilia. 相似文献
1