title: Analysis of Pathological Activities of CCN Proteins in Fibrotic Diseases: Kidney Fibrosis.
abstract: Renal fibrosis is characterized by glomerulosclerosis and tubulointerstitial fibrosis. Transforming growth factor-β (TGF-β) is postulated to play a central role in the development of both fibrotic processes. Extracellular matrix proteins, particularly type I collagen and fibronectin, accumulate in the tissue during renal fibrogenesis. CCN2, also known as connective tissue growth factor (CTGF), is increased in the setting of fibrosis and modulates a number of downstream signaling pathways involved in the fibrogenic properties of TGF-β. Unilateral ureteral obstruction is one of the most widely used models of renal tubulointerstitial fibrosis. Herein, we describe unilateral ureteral obstruction in mice as an animal model of renal fibrosis and methods for immunohistochemical analyses of extracellular matrix proteins and CCN2. In addition, we describe the construction of podocyte-specific CCN2-transgenic mice for analyzing mesangial matrix expansion and glomerulosclerosis.
authors.affiliation: Department of Nephrology, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Yokoi
authors.affiliation: Department of Nephrology, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan. mmuko@kumamoto-u.ac.jp.|Department of Nephrology, Kumamoto University Graduate School of Medical Sciences, Kumamoto, 860-8556, Japan. mmuko@kumamoto-u.ac.jp.
authors.forename: Masashi
authors.initials: M
authors.lastname: Mukoyama
mesh_terms: D000818:Animals; D055511:CCN Intercellular Signaling Proteins / Q000235:genetics* / Q000378:metabolism*; D004195:Disease Models, Animal; D016326:Extracellular Matrix Proteins / Q000378:metabolism; D005355:Fibrosis; D005822:Genetic Vectors / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D007674:Kidney Diseases / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D008565:Membrane Proteins / Q000235:genetics; D051379:Mice; D008822:Mice, Transgenic; D011401:Promoter Regions, Genetic; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000378:metabolism; D016212:Transforming Growth Factor beta / Q000378:metabolism
chemical_list: D055511:CCN Intercellular Signaling Proteins; D016326:Extracellular Matrix Proteins; D008565:Membrane Proteins; D016212:Transforming Growth Factor beta; C113267:nephrin; D020797:Receptor, Platelet-Derived Growth Factor beta
keywords: CCN2; Connective tissue growth factor; Diabetic nephropathy; Podocytes; Renal fibrosis; TGF-β; Transgenic mice