title: Analysis of Pathological Activities of CCN Proteins in Fibrotic Diseases: Liver Fibrosis.
abstract: Hepatic fibrosis is a complex pathology arising from chronic injury. Pathological features are dominated by the excessive production of extracellular matrix proteins, particularly collagens which are deposited as insoluble scar material that can compromise tissue function. Fibrosis in the liver can often be assessed by staining for collagen in tissue sections and this is an approach that is widely used for grading of fibrosis in human biopsies. However, the recognition of the molecular components that drive fibrosis, including CCN proteins, and the involvement of hepatic stellate cells (HSC) as the principal collagen-producing cells in fibrosing liver, has resulted in a wide variety of molecular and cellular approaches to study the pathogenesis of fibrosis both in vivo and in vitro.
authors.affiliation: The Research Institute at Nationwide Children's Hospital, Research Building 2, 700 Children's Drive, Columbus, OH, 43205, USA. Li.chen@nationwidechildrens.org.
authors.forename: Li
authors.initials: L
authors.lastname: Chen
authors.affiliation: The Research Institute at Nationwide Children's Hospital, Research Building 2, 700 Children's Drive, Columbus, OH, 43205, USA.|Molecular, Cellular, and Developmental Biology Program, The Ohio State University, Columbus, OH, 43212, USA.|Department of Surgery, Wexner Medical Center, The Ohio State University, Columbus, OH, 43212, USA.
authors.forename: David R
authors.initials: DR
authors.lastname: Brigstock
mesh_terms: D000199:Actins / Q000235:genetics / Q000378:metabolism; D000818:Animals; D015415:Biomarkers; D055511:CCN Intercellular Signaling Proteins / Q000235:genetics* / Q000378:metabolism*; D047369:Chromatin Immunoprecipitation; D024042:Collagen Type I / Q000235:genetics / Q000378:metabolism; D055513:Connective Tissue Growth Factor / Q000235:genetics / Q000378:metabolism; D004195:Disease Models, Animal; D004797:Enzyme-Linked Immunosorbent Assay; D015870:Gene Expression; D017930:Genes, Reporter; D055166:Hepatic Stellate Cells / Q000166:cytology / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008103:Liver Cirrhosis / Q000209:etiology / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D008297:Male; D051379:Mice; D011401:Promoter Regions, Genetic; D034741:RNA, Small Interfering / Q000235:genetics; D015398:Signal Transduction
chemical_list: D000199:Actins; D015415:Biomarkers; D055511:CCN Intercellular Signaling Proteins; D024042:Collagen Type I; D034741:RNA, Small Interfering; C544013:alpha-smooth muscle actin, mouse; D055513:Connective Tissue Growth Factor
keywords: Chromatin immunoprecipitation; ELISA; Electrophoretic mobility shift assay; Immunohistochemistry; Immunoprecipitation; In situ hybridization; RT-PCR