The cholangiocyte primary cilium in health and disease

Adrian Mansini, Estanislao Peixoto, Kristen M. Thelen, Cesar Gaspari, Sujeong Jin, Sergio Gradilone

Research output: Contribution to journalReview articlepeer-review

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Cholangiocytes, like most cells, express primary cilia extending from their membranes. These organelles function as antennae which detect stimuli from bile and transmit the information into cells regulating several signaling pathways involved in secretion, proliferation and apoptosis. The ability of primary cilia to detect different signals is provided by ciliary associated proteins which are expressed in its membrane. Defects in the structure and/or function of these organelles lead to cholangiociliopathies that result in cholangiocyte hyperproliferation, altered fluid secretion and absorption. Since primary cilia dysfunction has been observed in several epithelial tumors, including cholangiocarcinoma (CCA), primary cilia have been proposed as tumor suppressor organelles. In addition, the loss of cilia is associated with dysregulation of several molecular pathways resulting in CCA development and progression. Thus, restoration of the primary cilia may be a potential therapeutic approach for several ciliopathies and CCA.

Original languageEnglish (US)
Pages (from-to)1245-1253
Number of pages9
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Issue number4
StatePublished - Apr 2018

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© 2017 Elsevier B.V.


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