Inborn Errors in Bile Acid Synthesis

Shefer, Sarah

Abstract

Funding Agency

• Agency: National Institute of Health (NIH)
• Institute: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
• Type: Method to Extend Research in Time (MERIT) Award (R37)
• Project #: 5R37DK026756-18
• Application #: 2137924
• Study Section: Special Emphasis Panel (NSS)
• Project Start: 1980-12-01
• Project End: 1996-11-30
• Budget Start: 1995-12-31
• Budget End: 1996-11-30
• Support Year: 18
• Fiscal Year: 1996

Institution

• Name: University of Medicine & Dentistry of NJ
• Department: Internal Medicine/Medicine
• Type: Schools of Medicine
• DUNS #: 605799469
• City: Newark
• State: NJ
• Country: United States
• Zip Code: 07107

Publications

Hirayama, Takeshi; Honda, Akira; Matsuzaki, Yasushi et al. (2006) Hypercholesterolemia in rats with hepatomas: increased oxysterols accelerate efflux but do not inhibit biosynthesis of cholesterol. Hepatology 44:602-11

Li, Hai; Chen, Frank; Shang, Quan et al. (2005) FXR-activating ligands inhibit rabbit ASBT expression via FXR-SHP-FTF cascade. Am J Physiol Gastrointest Liver Physiol 288:G60-6

Dubrac, Sandrine; Lear, Steven R; Ananthanarayanan, Meena et al. (2005) Role of CYP27A in cholesterol and bile acid metabolism. J Lipid Res 46:76-85

Honda, Akira; Salen, Gerald; Matsuzaki, Yasushi et al. (2005) Disrupted coordinate regulation of farnesoid X receptor target genes in a patient with cerebrotendinous xanthomatosis. J Lipid Res 46:287-96

Honda, Akira; Yoshida, Tadashi; Xu, Guorong et al. (2004) Significance of plasma 7alpha-hydroxy-4-cholesten-3-one and 27-hydroxycholesterol concentrations as markers for hepatic bile acid synthesis in cholesterol-fed rabbits. Metabolism 53:42-8

Xu, Guorong; Pan, Lu-Xing; Li, Hai et al. (2004) Dietary cholesterol stimulates CYP7A1 in rats because farnesoid X receptor is not activated. Am J Physiol Gastrointest Liver Physiol 286:G730-5

Erickson, Sandra K; Lear, Steven R; Deane, Sean et al. (2003) Hypercholesterolemia and changes in lipid and bile acid metabolism in male and female cyp7A1-deficient mice. J Lipid Res 44:1001-9

Xu, Guorong; Li, Hai; Pan, Lu-Xing et al. (2003) FXR-mediated down-regulation of CYP7A1 dominates LXRalpha in long-term cholesterol-fed NZW rabbits. J Lipid Res 44:1956-62

Sehayek, Ephraim; Wang, Rong; Ono, Jennie G et al. (2003) Localization of the PE methylation pathway and SR-BI to the canalicular membrane: evidence for apical PC biosynthesis that may promote biliary excretion of phospholipid and cholesterol. J Lipid Res 44:1605-13

Pullinger, Clive R; Eng, Celeste; Salen, Gerald et al. (2002) Human cholesterol 7alpha-hydroxylase (CYP7A1) deficiency has a hypercholesterolemic phenotype. J Clin Invest 110:109-17