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It interacts with many other proteins, including plasma membrane proteins, modulating cell function. It is expressed at low levels in normal adult kidney cells but is increased in kidney diseases, playing important roles in inflammation and in the development of glomerular and interstitial fibrosis in chronic disease. This review reports the evidence for its expression in human and animal models of chronic kidney disease and summarizes data showing that anti\u2010<jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content> therapy can successfully attenuate fibrotic changes in several such models, suggesting that therapies targeting <jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content> and events downstream of it in renal cells may be useful for the treatment of human kidney fibrosis. Connective tissue growth factor stimulates the development of fibrosis in the kidney in many ways including activating cells to increase extracellular matrix synthesis, inducing cell cycle arrest and hypertrophy, and prolonging survival of activated cells. The relationship between <jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content> and the pro\u2010fibrotic factor <jats:styled-content style=\"fixed-case\">TGF<\/jats:styled-content>\u03b2 is examined and mechanisms by which <jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content> promotes signalling by the latter are discussed. No specific cellular receptors for <jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content> have been discovered but it interacts with and activates several plasma membrane proteins including low\u2010density lipoprotein receptor\u2010related protein (<jats:styled-content style=\"fixed-case\">LRP<\/jats:styled-content>)\u20101, <jats:styled-content style=\"fixed-case\">LRP<\/jats:styled-content>\u20106, tropomyosin\u2010related kinase <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>, integrins and heparan sulphate proteoglycans. Intracellular signalling and downstream events triggered by such interactions are reviewed. Finally, the relationships between <jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content> and several anti\u2010fibrotic factors, such as bone morphogenetic factor\u20104 (<jats:styled-content style=\"fixed-case\">BMP<\/jats:styled-content>4), <jats:styled-content style=\"fixed-case\">BMP<\/jats:styled-content>7, hepatocyte growth factor, <jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content>3 and <jats:styled-content style=\"fixed-case\">O<\/jats:styled-content>ncostatin <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>, are discussed. 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