Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Research Project (R01)
Project #
1R01DK050697-01
Application #
2151748
Study Section
General Medicine A Subcommittee 2 (GMA)
Project Start
1996-02-01
Project End
1999-01-31
Budget Start
1996-02-01
Budget End
1997-01-31
Support Year
1
Fiscal Year
1996
Total Cost
Indirect Cost
Name
University of California San Francisco
Department
Psychiatry
Type
Schools of Medicine
DUNS #
073133571
City
San Francisco
State
CA
Country
United States
Zip Code
94143
Shah, Sohela; Sanford, Ukina R; Vargas, Julie C et al. (2010) Strain background modifies phenotypes in the ATP8B1-deficient mouse. PLoS One 5:e8984
Pawlikowska, Ludmila; Strautnieks, Sandra; Jankowska, Irena et al. (2010) Differences in presentation and progression between severe FIC1 and BSEP deficiencies. J Hepatol 53:170-8
Strautnieks, Sandra S; Byrne, Jane A; Pawlikowska, Ludmila et al. (2008) Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families. Gastroenterology 134:1203-14
Knisely, A S; Strautnieks, Sandra S; Meier, Yvonne et al. (2006) Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 44:478-86
Paulusma, Coen C; Groen, Annemiek; Kunne, Cindy et al. (2006) Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport. Hepatology 44:195-204
Klomp, Leo W J; Vargas, Julie C; van Mil, Saskia W C et al. (2004) Characterization of mutations in ATP8B1 associated with hereditary cholestasis. Hepatology 40:27-38
van Mil, Saskia W C; van der Woerd, Wendy L; van der Brugge, Gerda et al. (2004) Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11. Gastroenterology 127:379-84
Carlton, Victoria E H; Pawlikowska, Ludmila; Bull, Laura N (2004) Molecular basis of intrahepatic cholestasis. Ann Med 36:606-17
Pawlikowska, Ludmila; Groen, Annemiek; Eppens, Elaine F et al. (2004) A mouse genetic model for familial cholestasis caused by ATP8B1 mutations reveals perturbed bile salt homeostasis but no impairment in bile secretion. Hum Mol Genet 13:881-92
Bull, Laura N (2002) Hereditary forms of intrahepatic cholestasis. Curr Opin Genet Dev 12:336-42

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