Article

Cholangiocyte primary cilia in liver health and disease.

Mayo Clinic College of Medicine, Department of Internal Medicine, Rochester, Minnesota 55905, USA.
Developmental Dynamics (impact factor: 2.54). 05/2008; 237(8):2007-12. DOI:10.1002/dvdy.21530 pp.2007-12
Source: PubMed

ABSTRACT 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.

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Keywords

apical plasma membrane
 
bile ductal lumen
 
cell types
 
chemo-sensory functions
 
Cholangiocyte cilia
 
cholangiocyte primary cilia
 
cholangiocytes
 
ciliary mechano-
 
cystic
 
epithelial cells
 
fibrotic liver diseases
 
functions
 
genes encoding ciliary-associated proteins
 
genes encoding polycystin-1
 
intrahepatic bile ducts
 
liver diseases
 
polycystin-2
 
primary cilia
 
processes