Published in Int J Mol Sci on August 04, 2014
Bile acids induce energy expenditure by promoting intracellular thyroid hormone activation. Nature (2006) 9.36
Targeted disruption of the nuclear receptor FXR/BAR impairs bile acid and lipid homeostasis. Cell (2000) 6.71
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med (2010) 6.23
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A (2009) 5.72
Cystic fibrosis. Lancet (2009) 5.40
Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr (2008) 5.19
EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol (2009) 4.45
Targeting bile-acid signalling for metabolic diseases. Nat Rev Drug Discov (2008) 4.43
Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology (2007) 4.33
Association of cystic fibrosis with abnormalities in fatty acid metabolism. N Engl J Med (2004) 4.09
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci U S A (2011) 3.87
Diagnosis and management of primary sclerosing cholangitis. Hepatology (2010) 3.60
A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. Nat Genet (1998) 3.33
Regulation of antibacterial defense in the small intestine by the nuclear bile acid receptor. Proc Natl Acad Sci U S A (2006) 3.05
Endocrine functions of bile acids. EMBO J (2006) 3.03
Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy. Hepatology (2010) 2.82
Farnesoid X receptor antagonizes nuclear factor kappaB in hepatic inflammatory response. Hepatology (2008) 2.79
Defects in gallbladder emptying and bile Acid homeostasis in mice with cystic fibrosis transmembrane conductance regulator deficiencies. Gastroenterology (2012) 2.77
High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology (2009) 2.75
Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome. Hepatology (2002) 2.74
Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J (2006) 2.54
Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care (2010) 2.52
Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros (2005) 2.46
High-dose ursodeoxycholic acid is associated with the development of colorectal neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Am J Gastroenterol (2011) 2.38
The phenotypic consequences of CFTR mutations. Ann Hum Genet (2003) 2.09
Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros (2011) 1.93
Genetic modifiers of liver disease in cystic fibrosis. JAMA (2009) 1.88
Expression and function of the bile acid receptor TGR5 in Kupffer cells. Biochem Biophys Res Commun (2008) 1.83
Evaluation of acoustic radiation force impulse elastography for fibrosis staging of chronic liver disease: a pilot study. Liver Int (2009) 1.82
Bile acids as regulators of hepatic lipid and glucose metabolism. Dig Dis (2010) 1.79
Liver disease and common-bile-duct stenosis in cystic fibrosis. N Engl J Med (1988) 1.79
Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile. Gastroenterology (2007) 1.73
Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2-/- mice. Hepatology (2009) 1.72
The G-protein-coupled bile acid receptor, Gpbar1 (TGR5), negatively regulates hepatic inflammatory response through antagonizing nuclear factor κ light-chain enhancer of activated B cells (NF-κB) in mice. Hepatology (2011) 1.71
Nonalcoholic fatty liver disease: US-based acoustic radiation force impulse elastography. Radiology (2010) 1.71
Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis. J Cyst Fibros (2013) 1.66
Non-invasive markers for hepatic fibrosis. BMC Gastroenterol (2011) 1.65
Intestinal microbiota determines development of non-alcoholic fatty liver disease in mice. Gut (2012) 1.64
High expression of the bile salt-homeostatic hormone fibroblast growth factor 19 in the liver of patients with extrahepatic cholestasis. Hepatology (2009) 1.62
Inhibition by cyclosporin A of adenosine triphosphate-dependent transport from the hepatocyte into bile. Gastroenterology (1993) 1.58
Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology (2007) 1.58
Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine. Infect Immun (2004) 1.53
Small intestine bacterial overgrowth. Gastroenterology (1979) 1.51
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J Pediatr (2007) 1.48
Advanced MRI methods for assessment of chronic liver disease. AJR Am J Roentgenol (2009) 1.39
Sclerosing cholangitis: a focus on secondary causes. Hepatology (2006) 1.37
Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut (2007) 1.29
Nutritional assessment and hepatic fatty acid composition in non-alcoholic fatty liver disease (NAFLD): a cross-sectional study. J Hepatol (2007) 1.26
LIGHT AND ELECTRON MICROSCOPIC EXAMINATION OF THE SMALL BOWEL OF CHILDREN WITH CYSTIC FIBROSIS. J Pediatr (1964) 1.21
Evidence of intestinal inflammation in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr (2010) 1.17
Metabolic phenotyping and systems biology approaches to understanding metabolic syndrome and fatty liver disease. Gastroenterology (2013) 1.16
The biliary HCO(3)(-) umbrella: a unifying hypothesis on pathogenetic and therapeutic aspects of fibrosing cholangiopathies. Hepatology (2010) 1.16
The bile acid receptor GPBAR-1 (TGR5) modulates integrity of intestinal barrier and immune response to experimental colitis. PLoS One (2011) 1.11
A biliary HCO3- umbrella constitutes a protective mechanism against bile acid-induced injury in human cholangiocytes. Hepatology (2012) 1.11
Dual farnesoid X receptor/TGR5 agonist INT-767 reduces liver injury in the Mdr2-/- (Abcb4-/-) mouse cholangiopathy model by promoting biliary HCO⁻₃ output. Hepatology (2011) 1.11
Evidence of chronic inflammation in morphologically normal small intestine of cystic fibrosis patients. Pediatr Res (2000) 1.09
Bile acid-induced apoptosis in hepatocytes is caspase-6-dependent. J Biol Chem (2008) 1.08
Gastrointestinal complications of cystic fibrosis. Clin Gastroenterol Hepatol (2012) 1.06
The Gordian Knot of dysbiosis, obesity and NAFLD. Nat Rev Gastroenterol Hepatol (2013) 1.06
The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. Hepatology (2000) 1.06
Cancer risk in cystic fibrosis: a 20-year nationwide study from the United States. J Natl Cancer Inst (2012) 1.05
Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation. Hepatology (2006) 1.04
Role of nuclear receptors for bile acid metabolism, bile secretion, cholestasis, and gallstone disease. Biochim Biophys Acta (2010) 1.01
Cystic fibrosis transmembrane conductance regulator knockout mice exhibit aberrant gastrointestinal microbiota. Gut Microbes (2012) 1.01
Cyclosporin A blocks bile acid synthesis in cultured hepatocytes by specific inhibition of chenodeoxycholic acid synthesis. Biochem J (1991) 1.00
Ischemic cholangitis. Mayo Clin Proc (1998) 1.00
Intestinal bile acid malabsorption in cystic fibrosis. Gut (1993) 1.00
Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study. Hepatology (1992) 0.98
Protective effect of bile acids on the onset of fructose-induced hepatic steatosis in mice. J Lipid Res (2010) 0.97
Crohn's disease and cystic fibrosis. Dig Dis Sci (1994) 0.96
Cystic fibrosis-associated liver disease. Best Pract Res Clin Gastroenterol (2010) 0.96
Clinical and genetic risk factors for cystic fibrosis-related liver disease. Pediatrics (1999) 0.94
Nuclear receptors as new perspective for the management of liver diseases. Gastroenterology (2011) 0.93
Non-invasive liver elastography (Fibroscan) for detection of cystic fibrosis-associated liver disease. J Cyst Fibros (2009) 0.92
Transient elastography in patients with cystic fibrosis. Pediatr Radiol (2010) 0.92
Serum proteome profiling identifies novel and powerful markers of cystic fibrosis liver disease. PLoS One (2013) 0.91
Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol (2002) 0.90
Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology (2004) 0.90
Outcome in cystic fibrosis liver disease. Am J Gastroenterol (2010) 0.90
Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease. J Hepatol (2004) 0.90
Small intestine bacterial overgrowth is frequent in cystic fibrosis: combined hydrogen and methane measurements are required for its detection. Acta Biochim Pol (2009) 0.89
Bile acid malabsorption in cystic fibrosis with and without pancreatic insufficiency. J Pediatr Gastroenterol Nutr (1984) 0.89
Metabolic syndrome: is immunosuppression to blame? Liver Transpl (2011) 0.89
CFTR depletion results in changes in fatty acid composition and promotes lipogenesis in intestinal Caco 2/15 cells. PLoS One (2010) 0.89
Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: a double blind prospective study. J Cyst Fibros (2014) 0.88
Effects of ivacaftor in patients with cystic fibrosis who carry the G551D mutation and have severe lung disease. Chest (2014) 0.88
Liver disease in adult patients with cystic fibrosis: a frequent and independent prognostic factor associated with death or lung transplantation. J Hepatol (2011) 0.88
Impact of lung transplantation on serum lipids in adults with cystic fibrosis. J Heart Lung Transplant (2011) 0.88
A pilot study of the characterization of hepatic tissue strain in children with cystic-fibrosis-associated liver disease (CFLD) by acoustic radiation force impulse imaging. Pediatr Radiol (2012) 0.87
Ultrasound evaluation of liver disease in cystic fibrosis as part of an annual assessment clinic: a 9-year review. Clin Radiol (2002) 0.86
ARFI and transient elastography for characterization of cystic fibrosis related liver disease: first longitudinal follow-up data in adult patients. J Cyst Fibros (2013) 0.86
Cystic fibrosis is a risk factor for celiac disease. Acta Biochim Pol (2010) 0.86
Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data. Liver Transpl (2011) 0.84
The biliary HCO(3)(-) umbrella: experimental evidence revisited. Curr Opin Gastroenterol (2012) 0.84
Surveillance for cystic fibrosis-associated hepatobiliary disease: early ultrasound changes and predisposing factors. J Pediatr (2003) 0.84
Biliary complications of cystic fibrosis. Gut (1992) 0.84
Non-invasive evaluation of cystic fibrosis related liver disease in adults with ARFI, transient elastography and different fibrosis scores. PLoS One (2012) 0.84
Farnesoid X receptor expression is decreased in colonic mucosa of patients with primary sclerosing cholangitis and colitis-associated neoplasia. Inflamm Bowel Dis (2013) 0.84
Genetics of cholestatic liver disease in 2010. Curr Opin Gastroenterol (2010) 0.83