title: Expression of polycystins and fibrocystin on primary cilia of lung cells.
abstract: Mutations in polycystin-1, polycystin-2, or fibrocystin account for autosomal dominant or recessive polycystic kidney disease. Renal cystogenesis is linked to abnormal localization and function of these cystoproteins in renal primary cilia. They are also expressed in extrarenal tissues in which their functions are unclear. Here we found that human type-II alveolar epithelial A549, airway submucosal Calu-3 cells, and rat bronchioles contain primary or multiple cilia in which we detected these cystoproteins. At sub-confluency, polycystin-1 was expressed on plasma membrane, while polycystin-2 was localized to the ER of resting cells. Both polycystins were detected on the spindle and mid-body of mitotic cells, while fibrocystin was on centrosome throughout cell cycle. Polycystins and fibrocystin may participate in regulating mucociliary sensing and transport within pulmonary airways.
authors.affiliation: a Membrane Protein Disease Research Group, Department of Physiology, University of Alberta, 7-29 Medical Sciences Building, Edmonton, AB T6G 2H7, Canada.
authors.forename: Qiaolin
authors.initials: Q
authors.lastname: Hu
authors.forename: Yuliang
authors.initials: Y
authors.lastname: Wu
authors.forename: Jingfeng
authors.initials: J
authors.lastname: Tang
authors.forename: Wang
authors.initials: W
authors.lastname: Zheng
authors.forename: Qian
authors.initials: Q
authors.lastname: Wang
authors.forename: Drew
authors.initials: D
authors.lastname: Nahirney
authors.forename: Marek
authors.initials: M
authors.lastname: Duszyk
authors.forename: Shaohua
authors.initials: S
authors.lastname: Wang
authors.forename: Jian-Cheng
authors.initials: JC
authors.lastname: Tu
authors.forename: Xing-Zhen
authors.initials: XZ
authors.lastname: Chen
mesh_terms: D000818:Animals; D002453:Cell Cycle / Q000502:physiology; D045744:Cell Line, Tumor; D018385:Centrosome / Q000378:metabolism; D002923:Cilia / Q000235:genetics / Q000378:metabolism; D006224:Cricetinae; D005786:Gene Expression Regulation / Q000502:physiology*; D006801:Humans; D008168:Lung / Q000166:cytology / Q000378:metabolism*; D051381:Rats; D011956:Receptors, Cell Surface / Q000096:biosynthesis* / Q000235:genetics; D050396:TRPP Cation Channels / Q000096:biosynthesis* / Q000235:genetics
chemical_list: D011956:Receptors, Cell Surface; D050396:TRPP Cation Channels; C451750:PKHD1 protein, human; C479541:PKHD1 protein, rat
keywords: A549; Calu-3; cils primaires; fibrocystin; fibrocystine; lung; polycystin; polycystine; poumon; primary cilium