Regulation of ciliary trafficking of polycystin-2 and the pathogenesis of autosomal dominant polycystic kidney disease.
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.
Cai
Yiqiang
Y
Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06510, USA. yiqiang.cai@yale.edu
Tang
Zhangui
Z
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
D011956:Receptors, Cell Surface; D050396:TRPP Cation Channels; C451750:PKHD1 protein, human