Hepatobiliary complications of cystic fibrosis
Author:
Publisher
Springer Science and Business Media LLC
Subject
Gastroenterology,General Medicine
Link
http://link.springer.com/content/pdf/10.1007/s11894-004-0013-6.pdf
Reference55 articles.
1. Dodge JA, Morison S, Lewis PA, et al.: Incidence, population, and survival of cystic fibrosis in the UK, 1968–95. UK Cystic Fibrosis Survey Management Committee. Arch Dis Child 1997, 77:493–496.
2. Gabriel SE, Brigman KN, Koller BH, et al.: Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science 1994, 266:107–109.
3. Riordan JR, Rommens JM, Kerem B-S, et al.: Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989, 245:1066–1073. This study identified the gene defect in CF. The gene, located on chromosome 7, codes for a protein known as the cystic fibrosis transmembrane conductance regulator (CFTR). A mutation at position 508 in the first nucleotide binding fold accounts for the most common mutation in CF. Identification of the gene defect in CF provided critical breakthroughs in our understanding of the pathogenesis of this disease.
4. Cohn JA, Strong TA, Picciotto MA, et al.: Localization of CFTR in human bile duct epithelial cells. Gastroenterology 1993, 105:1857–1864. This study demonstrated that, in liver, CFTR is localized to the apical membrane of bile duct epithelial cells, or cholangiocytes. It is not found in hepatic parenchymal cells (hepatocytes) or other cells of the liver. The role of CFTR in biliary epithelium provided insight into the mechanisms of normal bile formation.
5. Fitz JG, Basavappa S, McGill J, et al.: Regulation of membrane chloride currents in rat bile duct epithelial cells. J Clin Invest 1993, 91:319–328. This paper described the regulatory mechanisms involved in Cl− secretion in the apical membrane of biliary epithelial cells. Distinct Cl− conductances were described, including both Ca2+-and cAMP-activated channels. The study demonstrated that cAMP-regulated Cl− secretion is associated with endogenous expression of CFTR.
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