The Roles of Primary cilia in Polycystic Kidney Disease.
The Roles of Primary cilia in Polycystic Kidney Disease.
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DOI：
10.3934/molsci.2013.1.27
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发表时间：
2014
影响因子：
1.4
通讯作者：
Nauli SM
中科院分区：
文献类型：
--
作者：
Kathem SH;Mohieldin AM;Nauli SM
常染色体显性遗传性多囊肾病（ADPKD）是一种遗传性疾病，可导致进行性肾囊肿形成，最终丧失肾功能和其他全身性疾病。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。ADPKD被认为是纤毛疾病之一，因为它与初级纤毛功能异常有关。为了了解初级纤毛的全过程及其与ADPKD的关系，对初级纤毛的结构、功能和作用进行了细致的研究。因此，对初级纤毛的关注已经出现，以支持初级纤毛在ADPKD中的重要作用。初级纤毛不仅具有机械感觉功能，而且具有化学感觉功能。初级纤毛的结构和功能缺陷导致囊性肾病和血管性高血压。因此，将分析ADPKD的机械感觉和化学感觉功能。
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.