Transcriptional Basis for Hepatic Fibrosis in Cystic Fibrosis–associated Liver Disease

Autor: Peter Lewindon, Ristan M. Greer, Grant A. Ramm, Richard M. Williamson, Ross W. Shepherd, Anita C. Hoskins, Tamara N. Pereira

Publikováno v: Journal of Pediatric Gastroenterology & Nutrition. 54:328-335

Liver disease contributes to significant morbidity and mortality in cystic fibrosis (CF). Although all patients with CF express the defective CF transmembrane conductance regulator in cholangiocytes, many develop asymptomatic fibrosing liver disease.

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::84daa99d61446a08aa0edb52189f1b3f
https://doi.org/10.1097/mpg.0b013e3182432034

Enhanced length-dependent Ca2+ activation in fish cardiomyocytes permits a large operating range of sarcomere lengths

Autor: Olivier Cazorla, Simon M. Patrick, Holly A. Shiels, Anita C. Hoskins, Ed White, Jonathan C. Kentish

Publikováno v: Journal of Molecular and Cellular Cardiology
Journal of Molecular and Cellular Cardiology, Elsevier, 2010, 48 (5), pp.917-924. ⟨10.1016/j.yjmcc.2010.02.008⟩

International audience; Length-tension relationship Skinned cardiac myocytes Phosphorylation Myosin binding protein C (MyBPC) Myosin light chain-2 (MLC-2) TnI TnT Fish myocytes continue to develop active tension when stretched to sarcomere lengths (S

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::88b3f8aa5fbb83c4d79e4b70f59733f3
https://hal.umontpellier.fr/hal-01824348

Fibrogenesis in pediatric cholestatic liver disease: role of taurocholate and hepatocyte-derived monocyte chemotaxis protein-1 in hepatic stellate cell recruitment

Autor: Tamara N. Pereira, Agnieszka D. Ney, Sonia A. Greco, James D. Doecke, Anita C. Hoskins, Peter J. Meikle, Bruno Turlin, Peter Lewindon, Kim R. Bridle, Grant A. Ramm, Ross W. Shepherd

Publikováno v: Hepatology (Baltimore, Md.). 49(2)

Cholestatic liver diseases, such as cystic fibrosis (CF) liver disease and biliary atresia, predominate as causes of childhood cirrhosis. Despite diverse etiologies, the stereotypic final pathway involves fibrogenesis where hepatic stellate cells (HS

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4cab9fc6028ecd51db6546932898da9c
https://pubmed.ncbi.nlm.nih.gov/19115220

Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis

Autor: Peter Lewindon, Anita C. Hoskins, Tamara N. Pereira, Ross W. Shepherd, Kenneth D.R. Setchell, J. L. Smith, Grant A. Ramm, Nancy C. O'Connell

Publikováno v: Hepatology (Baltimore, Md.). 39(6)

Focal biliary cirrhosis causes significant morbidity and mortality in cystic fibrosis (CF). Although the mechanisms of pathogenesis remain unclear, bile acids have been proposed as potential mediators of liver injury. This study examined bile acid co

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ca8953d774bd722afab81841c51a6404
https://pubmed.ncbi.nlm.nih.gov/15185309

The Role of Hepatic Stellate Cells and Transforming Growth Factor-β1 in Cystic Fibrosis Liver Disease

Autor: Anita C. Hoskins, Peter Lewindon, Tamara N. Pereira, Kim R. Bridle, Grant A. Ramm, Ross W. Shepherd, Richard M. Williamson

Liver disease causes significant morbidity and mortality from multilobular cirrhosis in patients with cystic fibrosis. Abnormal bile transport and biliary fibrosis implicate abnormal biliary physiology in the pathogenesis of cystic fibrosis-associate

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::318b439ab97b5d9b00458b2596b8bf86
https://europepmc.org/articles/PMC1850885/