Kathem Sarmed H, Mohieldin Ashraf M, Nauli Surya M
College of Pharmacy and Pharmaceutical Sciences, University of Toledo, Toledo, Ohio ; College of Pharmacy, University of Baghdad, Baghdad, Iraq.
College of Pharmacy and Pharmaceutical Sciences, University of Toledo, Toledo, Ohio.
AIMS Mol Sci. 2014;1(1):27-46. doi: 10.3934/molsci.2013.1.27.
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited genetic disorder that results in progressive renal cyst formation with ultimate loss of renal function and other systemic disorders. These systemic disorders include abnormalities in cardiovascular, portal, pancreatic and gastrointestinal systems. ADPKD is considered to be among the ciliopathy diseases due to the association with abnormal primary cilia function. In order to understand the full course of primary cilia and its association with ADPKD, the structure, functions and role of primary cilia have been meticulously investigated. As a result, the focus on primary cilia has emerged to support the vital roles of primary cilia in ADPKD. The primary cilia have been shown to have not only a mechanosensory function but also a chemosensory function. Both structural and functional defects in primary cilia result in cystic kidney disease and vascular hypertension. Thus, the mechanosenory and chemosensory functions will be analyzed in regards to ADPKD.
常染色体显性多囊肾病（ADPKD）是一种遗传性基因疾病，会导致肾脏逐渐形成囊肿，最终肾功能丧失，并引发其他全身性疾病。这些全身性疾病包括心血管、门静脉、胰腺和胃肠道系统的异常。由于与原发性纤毛功能异常有关，ADPKD被认为属于纤毛病疾病。为了了解原发性纤毛的全过程及其与ADPKD的关联，人们对原发性纤毛的结构、功能和作用进行了细致研究。结果，对原发性纤毛的关注开始凸显，以支持其在ADPKD中的重要作用。原发性纤毛不仅具有机械传感功能，还具有化学传感功能。原发性纤毛的结构和功能缺陷都会导致多囊肾病和血管性高血压。因此，将针对ADPKD分析其机械传感和化学传感功能。
