By Jong Hoon Park, Curie Ahn (eds.)
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Extra info for Cystogenesis
In PKD, increases in the activity of molecules such as growth factor and TGF-β have been reported to occur and promote fibrosis. Therefore, the inflammation and fibrosis responses have been suggested as therapeutic targets for PKD. In order to guide further studies, this review indicates the roles of inflammatory and fibrosis signaling in PKD. 1 Introduction Inflammation is a kind of protective mechanism involved in the response to foreign organisms. When inflammation occurs, it typically shows redness, heat, pain, swelling, and so on.
446 Ta MH, Harris DC, Rangan GK (2013) Role of interstitial inflammation in the pathogenesis of polycystic kidney disease. Nephrology 18(5):317–330. 12045 Tak PP, Firestein GS (2001) NF-kappaB: a key role in inflammatory diseases. J Clin Invest 107(1):7–11. 1172/JCI11830 Talbot JJ, Shillingford JM, Vasanth S, Doerr N, Mukherjee S, Kinter MT, Watnick T, Weimbs T (2011) Polycystin-1 regulates STAT activity by a dual mechanism. Proc Natl Acad Sci U S A 108(19):7985–7990. 1103816108 Talbot JJ, Song X, Wang X, Rinschen MM, Doerr N, LaRiviere WB, Schermer B, Pei YP, Torres VE, Weimbs T (2014) The cleaved cytoplasmic tail of polycystin-1 regulates Src-dependent STAT3 activation.
1994), is elevated in jck mice that show PKD (Surendran et al. 2002). In Gpr48–/– mice that develop PKD, renal fibrosis was shown to accompany the activation of the Wnt signaling pathway (Dang et al. 2014). Also, Wnt signaling exerts effects on primary cilia and regulates cilia formation (Cisternas et al. 2014). Transgenic mice with genetic knock outs of the ciliary proteins Kif3a and Bbs1 showed a hyperactivation of Wnt signaling compared with normal mice (Corbit et al. 2008). Likewise, the up-regulation of Wnt/β-catenin signaling induces an increased expression of the gene encoding fibronectin, which is closely related to fibrosis (Cisternas et al.
Cystogenesis by Jong Hoon Park, Curie Ahn (eds.)