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  • Cholangiocyte primary cilia in liver health and disease #MMPMID18407555
  • Masyuk AI; Masyuk TV; LaRusso NF
  • Dev Dyn 2008[Aug]; 237 (8): 2007-12 PMID18407555show ga
  • The epithelial cells lining intrahepatic bile ducts (i.e., cholangiocytes), like many cell types in the body, have primary cilia extending from the apical plasma membrane into the bile ductal lumen. Cholangiocyte cilia express proteins such as polycystin-1, polycystin-2, fibrocystin, TRPV4, P2Y12, AC6, that account for ciliary mechano-, osmo-, and chemo-sensory functions; when these processes are disturbed by mutations in genes encoding ciliary-associated proteins, liver diseases (i.e., cholangiociliopathies) result. The cholangiociliopathies include but are not limited to cystic and fibrotic liver diseases associated with mutations in genes encoding polycystin-1, polycystin-2, and fibrocystin. In this review, we discuss the functions of cholangiocyte primary cilia, their role in the cholangiociliopathies, and potential therapeutic approaches.
  • |Animals[MESH]
  • |Bile Ducts, Intrahepatic/*cytology/*pathology[MESH]
  • |Chemoreceptor Cells/pathology/ultrastructure[MESH]
  • |Cilia/pathology/*physiology[MESH]
  • |Epithelial Cells/pathology/*ultrastructure[MESH]
  • |Humans[MESH]
  • |Liver Diseases/*pathology[MESH]
  • |Mechanoreceptors/pathology/ultrastructure[MESH]
  • |Water-Electrolyte Balance/physiology[MESH]

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  • suck abstract from ncbi

    2007 8.237 2008