TY - JOUR
T1 - Cholangiocyte primary cilia in liver health and disease
AU - Masyuk, Anatoliy I.
AU - Masyuk, Tatyana V.
AU - LaRusso, Nicholas F.
PY - 2008/8
Y1 - 2008/8
N2 - 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.
AB - 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.
KW - Chemosensors
KW - Cholangiociliopathies
KW - Cholangiocytes
KW - Liver
KW - Mechanosensors
KW - Osmosensors
KW - Primary cilia
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U2 - 10.1002/dvdy.21530
DO - 10.1002/dvdy.21530
M3 - Review article
C2 - 18407555
AN - SCOPUS:49449094484
SN - 1058-8388
VL - 237
SP - 2007
EP - 2012
JO - Developmental Dynamics
JF - Developmental Dynamics
IS - 8
ER -