By Jong Hoon Park, Curie Ahn (eds.)
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Extra info for Cystogenesis
In vertebrates, most motile cilia are displayed on the ependymal cells of brain ventricles, the epithelial cells of trachea and lung, and the cells of the oviducts (Michaud and Yoder 2006). These motile cilia are involved in the movement of mucus or the circulation of body fluid, whereas primary cilia function in the sensing of extracellular stimulus and signal transduction. In fact, recent studies have revealed that various proteins including ion channels, receptors, and transporters involved in signal transduction are localized to the ciliary membrane and basal body (Satir et al.
Moreover, these secreted factors can also induce proliferation. Thus, the induction of inflammation and fibrosis by hyperproliferation further accelerates cell proliferation, resulting in a vicious cycle among proliferation, inflammation, and fibrosis. Some reports have shown that the inhibition of cytokines that are involved in the up-regulation of inflammation and fibrosis could be a therapeutic strategy for PKD (Kaltschmidt et al. 2000; Brosius and He 2015; Chea and Lee 2009; Berthier et al.
Biochim Biophys Acta 1812(10):1327–1336. 012 Obermuller N, Morente N, Kranzlin B, Gretz N, Witzgall R (2001) A possible role for metalloproteinases in renal cyst development. Am J Physiol Renal Physiol 280(3):F540–F550 Olsan EE, Mukherjee S, Wulkersdorfer B, Shillingford JM, Giovannone AJ, Todorov G, Song X, Pei Y, Weimbs T (2011) Signal transducer and activator of transcription-6 (STAT6) inhibition suppresses renal cyst growth in polycystic kidney disease. Proc Natl Acad Sci U S A 108(44):18067–18072.
Cystogenesis by Jong Hoon Park, Curie Ahn (eds.)