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The Roles of Primary cilia in Polycystic Kidney Disease. 原发性纤毛在多囊肾病中的作用。
IF 1.4 Q4 BIOCHEMISTRY & MOLECULAR BIOLOGY Pub Date : 2014-01-01 DOI: 10.3934/molsci.2013.1.27
Sarmed H Kathem, Ashraf M Mohieldin, Surya M Nauli

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)是一种遗传性遗传病,导致进行性肾囊肿形成,最终肾功能丧失和其他全身性疾病。这些全身性疾病包括心血管、门静脉、胰腺和胃肠系统的异常。由于与原发性纤毛功能异常有关,ADPKD被认为是纤毛病之一。为了了解初级纤毛的整个过程及其与ADPKD的关系,我们对初级纤毛的结构、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。原发性纤毛的结构和功能缺陷可导致囊性肾病和血管性高血压。因此,机械感觉和化学感觉功能将在ADPKD方面进行分析。
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引用次数: 45
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AIMS Molecular Science
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