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Cholangiocyte primary cilia in liver health and disease
Author(s) -
Masyuk Anatoliy I.,
Masyuk Tatyana V.,
LaRusso Nicholas F.
Publication year - 2008
Publication title -
developmental dynamics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.634
H-Index - 141
eISSN - 1097-0177
pISSN - 1058-8388
DOI - 10.1002/dvdy.21530
Subject(s) - cilium , cholangiocyte , biology , ciliogenesis , microbiology and biotechnology , intrahepatic bile ducts , epithelial polarity , gene , intraflagellar transport , apical membrane , motile cilium , cell , epithelium , medicine , endocrinology , genetics , bile duct , flagellum
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, P2Y 12 , 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. Developmental Dynamics 237:2007–2012, 2008. © 2008 Wiley‐Liss, Inc.