Cholangiocyte primary cilia in liver health and disease

Anatoliy I. Masyuk, Tatyana V. Masyuk, Nicholas F. LaRusso

Research output: Contribution to journalReview article

94 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)2007-2012
Number of pages6
JournalDevelopmental Dynamics
Volume237
Issue number8
DOIs
StatePublished - Aug 1 2008

Keywords

  • Chemosensors
  • Cholangiociliopathies
  • Cholangiocytes
  • Liver
  • Mechanosensors
  • Osmosensors
  • Primary cilia

ASJC Scopus subject areas

  • Developmental Biology

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