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The role of Hedgehog signaling in fibrogenic liver repair
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This study suggests that injury-related activation of the hedgehog pathway modulates several important aspects of repair, containing hepatic accumulation of myofibroblasts and liver fibrosis
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• Choi SS, Omenetti A, Syn WK, Diehl AM (2011) The role of Hedgehog signaling in fibrogenic liver repair. Int J Biochem Cell Biol 43:238–244. This study suggests that injury-related activation of the hedgehog pathway modulates several important aspects of repair, containing hepatic accumulation of myofibroblasts and liver fibrosis
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Expression of the developmental sonic hedgehog (Shh) signalling pathway is up-regulated in chronic lung fibrosis and the Shh receptor patched 1 is present in circulating T lymphocytes
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The story of Notch and chronic kidney disease
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Sharma S, Sirin Y, Susztak K (2011) The story of Notch and chronic kidney disease. Curr Opin Nephrol Hypertens 20:56–61
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New insights into the mechanism of notch signalling in fibrosis
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This review summarizes the structure and activation of the Notch family members, and focuses on recent findings regarding the role of Notch in organ fibrogenesis, in humans and in animal models
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•• Kavian N, Servettaz A, Weill B, Batteux F (2012) New insights into the mechanism of notch signalling in fibrosis. Open Rheumatol J 6:96–102. This review summarizes the structure and activation of the Notch family members, and focuses on recent findings regarding the role of Notch in organ fibrogenesis, in humans and in animal models
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Notch signalling regulates fibroblast activation and collagen release in systemic sclerosis
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Notch signaling is activated in SSc and plays an important role in fibroblast activation and collagen release
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• Dees C, Tomcik M, Zerr P, Akhmetshina A, Horn A, Palumbo K, Beyer C, Zwerina J, Distler O, Schett G, Distler JH (2011) Notch signalling regulates fibroblast activation and collagen release in systemic sclerosis. Ann Rheum Dis 70:1304–1310. Notch signaling is activated in SSc and plays an important role in fibroblast activation and collagen release
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Kavian N, Servettaz A, Mongaret C, Wang A, Nicco C, Chereau C, Grange P, Vuiblet V, Birembaut P, Diebold MD, Weill B, Dupin N, Batteux F (2010) Targeting ADAM-17/notch signaling abrogates the development of systemic sclerosis in a murine model. Arthritis Rheum 62:3477–3487
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Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis
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Plantier L, Crestani B, Wert SE, Dehoux M, Zweytick B, Guenther A, Whitsett JA (2011) Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis. Thorax 66:651–657
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The Notch pathway in podocytes plays a role in the development of glomerular disease
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Niranjan T, Bielesz B, Gruenwald A, Ponda MP, Kopp JB, Thomas DB, Susztak K (2008) The Notch pathway in podocytes plays a role in the development of glomerular disease. Nat Med 14:290–298
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Murea M, Park JK, Sharma S, Kato H, Gruenwald A, Niranjan T, Si H, Thomas DB, Pullman JM, Melamed ML, Susztak K (2010) Expression of Notch pathway proteins correlates with albuminuria, glomerulosclerosis, and renal function. Kidney Int 78:514–522
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Dees C, Zerr P, Tomcik M, Beyer C, Horn A, Akhmetshina A, Palumbo K, Reich N, Zwerina J, Sticherling M, Mattson MP, Distler O, Schett G, Distler JH (2011) Inhibition of Notch signaling prevents experimental fibrosis and induces regression of established fibrosis. Arthritis Rheum 63:1396–1404
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Saad S, Stanners SR, Yong R, Tang O, Pollock CA (2010) Notch mediated epithelial to mesenchymal transformation is associated with increased expression of the Snail transcription factor. Int J Biochem Cell Biol 42:1115–1122
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Epithelial Notch signaling regulates interstitial fibrosis development in the kidneys of mice and humans
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This study suggests that Notch activation is associated with EMT of cultured tubular cells and is sufficient to induce severe tubulointerstitial fibrosis in vivo
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• Bielesz B, Sirin Y, Si H, Niranjan T, Gruenwald A, Ahn S, Kato H, Pullman J, Gessler M, Haase VH, Susztak K (2010) Epithelial Notch signaling regulates interstitial fibrosis development in the kidneys of mice and humans. J Clin Invest 120:4040–4054. This study suggests that Notch activation is associated with EMT of cultured tubular cells and is sufficient to induce severe tubulointerstitial fibrosis in vivo
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Waters AM, Wu MY, Onay T, Scutaru J, Liu J, Lobe CG, Quaggin SE, Piscione TD (2008) Ectopic notch activation in developing podocytes causes glomerulosclerosis. J Am Soc Nephrol 19:1139–1157
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•• Decleves AE, Sharma K (2014) Novel targets of antifibrotic and anti-inflammatory treatment in CKD. Nat Rev Nephrol 10:257–267. This review highlights new insights into the mechanisms that contribute to CKD and approaches that might facilitate the development of disease-arresting therapies for CKD
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•• Tampe D, Zeisberg M (2014) Potential approaches to reverse or repair renal fibrosis. Nat Rev Nephrol 10:226–237. This review summarizes strategies targeting various components of the fibrotic pathway, from signaling molecules including TGF-β, PI3K, and chemokines to microRNAs
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