title: Regulation of ciliary trafficking of polycystin-2 and the pathogenesis of autosomal dominant polycystic kidney disease.
abstract: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common human hereditary disorder characteristic of development of bilateral multiple fluid-filled kidney cysts. Accumulated evidence has suggested that primary cilium of renal epithelial cell plays a key role in cystogenesis. In this article we will give an overview on the basic information about polycystic kidney disease (PKD) and summarize the recent progresses in studies of regulation of polycystin-1 and -2 trafficking to cilia. We will also discuss the possible role of trafficking defects of polycystins on the pathogenesis of ADPKD.
authors.affiliation: Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06510, USA. yiqiang.cai@yale.edu
authors.forename: Yiqiang
authors.initials: Y
authors.lastname: Cai
authors.forename: Zhangui
authors.initials: Z
authors.lastname: Tang
mesh_terms: D000818:Animals; D002923:Cilia / Q000378:metabolism; D004847:Epithelial Cells / Q000378:metabolism; D006801:Humans; D007668:Kidney / Q000378:metabolism* / Q000473:pathology; D016891:Polycystic Kidney, Autosomal Dominant / Q000209:etiology / Q000235:genetics* / Q000378:metabolism; D021381:Protein Transport; D011956:Receptors, Cell Surface / Q000235:genetics / Q000378:metabolism / Q000502:physiology; D050396:TRPP Cation Channels / Q000235:genetics / Q000378:metabolism* / Q000502:physiology
chemical_list: D011956:Receptors, Cell Surface; D050396:TRPP Cation Channels; C451750:PKHD1 protein, human