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There are 40101 results for: content related to: Inherited disorders of cholestasis in adulthood

  1. You have free access to this content
    Altered hepatobiliary gene expressions in PFIC1: ATP8B1 gene defect is associated with CFTR downregulation


    Volume 43, Issue 5, May 2006, Pages: 1125–1134, Christine Demeilliers, Emmanuel Jacquemin, Véronique Barbu, Martine Mergey, François Paye, Laura Fouassier, Nicolas Chignard, Chantal Housset and Nour-Eddine Lomri

    Version of Record online : 20 APR 2006, DOI: 10.1002/hep.21160

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    Canalicular ABC transporters and liver disease

    The Journal of Pathology

    Volume 226, Issue 2, January 2012, Pages: 300–315, Michael Nicolaou, Edward J Andress, Joseph K Zolnerciks, Peter H Dixon, Catherine Williamson and Kenneth J Linton

    Version of Record online : 1 DEC 2011, DOI: 10.1002/path.3019

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    Characterization of mutations in ATP8B1 associated with hereditary cholestasis


    Volume 40, Issue 1, July 2004, Pages: 27–38, Leo W. J. Klomp, Julie C. Vargas, Saskia W. C. van Mil, Ludmila Pawlikowska, Sandra S. Strautnieks, Michiel J. T. van Eijk, Jenneke A. Juijn, Carlos Pabón-Peña, Lauren B. Smith, Joseph A. DeYoung, Jane A. Byrne, Justijn Gombert, Gerda van der Brugge, Ruud Berger, Irena Jankowska, Joanna Pawlowska, Erica Villa, A. S. Knisely, Richard J. Thompson, Nelson B. Freimer, Roderick H. J. Houwen and Laura N. Bull

    Version of Record online : 30 JUN 2004, DOI: 10.1002/hep.20285

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    Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1


    Volume 50, Issue 5, November 2009, Pages: 1597–1605, Dineke E. Folmer, Vincent A. van der Mark, Kam S. Ho-Mok, Ronald P.J. Oude Elferink and Coen C. Paulusma

    Version of Record online : 7 JUL 2009, DOI: 10.1002/hep.23158

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    ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): Phenotypic differences between PFIC1 and PFIC2 and natural history


    Volume 51, Issue 5, May 2010, Pages: 1645–1655, Anne Davit-Spraul, Monique Fabre, Sophie Branchereau, Christiane Baussan, Emmanuel Gonzales, Bruno Stieger, Olivier Bernard and Emmanuel Jacquemin

    Version of Record online : 28 JAN 2010, DOI: 10.1002/hep.23539

  6. Exome sequencing reveals compound heterozygous mutations in ATP8B1 in a JAG1/NOTCH2 mutation-negative patient with clinically diagnosed Alagille syndrome

    American Journal of Medical Genetics Part A

    Volume 167, Issue 4, April 2015, Pages: 891–893, Christopher M. Grochowski, Ramakrishnan Rajagopalan, Alexandra M. Falsey, Kathleen M. Loomes, David A. Piccoli, Ian D. Krantz, Marcella Devoto and Nancy B. Spinner

    Version of Record online : 3 MAR 2015, DOI: 10.1002/ajmg.a.36946

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    miR-33 controls the expression of biliary transporters, and mediates statin- and diet-induced hepatotoxicity

    EMBO Molecular Medicine

    Volume 4, Issue 9, September 2012, Pages: 882–895, Ryan M. Allen, Tyler J. Marquart, Carolyn J. Albert, Frederick J. Suchy, David Q.-H. Wang, Meenakshisundaram Ananthanarayanan, David A. Ford and Ángel Baldán

    Version of Record online : 5 JUL 2012, DOI: 10.1002/emmm.201201228

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    Poster Session 1: Metabolic and Genetic Diseases


    Volume 60, Issue S1, October 2014, Pages: 523A–528A,

    Version of Record online : 1 OCT 2014, DOI: 10.1002/hep.27509

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    Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport


    Volume 44, Issue 1, July 2006, Pages: 195–204, Coen C. Paulusma, Annemiek Groen, Cindy Kunne, Kam S. Ho-Mok, Astrid L. Spijkerboer, D. Rudi de Waart, Frans J. Hoek, Heleen Vreeling, Kees A. Hoeben, Jan van Marle, Ludmila Pawlikowska, Laura N. Bull, Alan F. Hofmann, A. S. Knisely and Ronald P. J. Oude Elferink

    Version of Record online : 23 JUN 2006, DOI: 10.1002/hep.21212

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    Analysis of aberrant pre-messenger RNA splicing resulting from mutations in ATP8B1 and efficient in vitro rescue by adapted U1 small nuclear RNA


    Volume 61, Issue 4, April 2015, Pages: 1382–1391, Wendy L. van der Woerd, Johanna Mulder, Franco Pagani, Ulrich Beuers, Roderick H.J. Houwen and Stan F.J. van de Graaf

    Version of Record online : 23 FEB 2015, DOI: 10.1002/hep.27620

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    Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate


    Volume 51, Issue 1, January 2010, Pages: 286–296, Lieke M. van der Velden, Janneke M. Stapelbroek, Elmar Krieger, Peter V. E. van den Berghe, Ruud Berger, Patricia M. Verhulst, Joost C. M. Holthuis, Roderick H. J. Houwen, Leo W. J. Klomp and Stan F. J. van de Graaf

    Version of Record online : 4 SEP 2009, DOI: 10.1002/hep.23268

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    ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity


    Volume 47, Issue 1, January 2008, Pages: 268–278, Coen C. Paulusma, Dineke E. Folmer, Kam S. Ho-Mok, D. Rudi de Waart, Petra M. Hilarius, Arthur J. Verhoeven and Ronald P. J. Oude Elferink

    Version of Record online : 19 OCT 2007, DOI: 10.1002/hep.21950

  13. Recovery of graft steatosis and protein-losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child

    Pediatric Transplantation

    Volume 16, Issue 5, August 2012, Pages: E177–E182, Emanuele Nicastro, Xavier Stephenne, Françoise Smets, Fabio Fusaro, Catherine de Magnée, Raymond Reding and Etienne M. Sokal

    Version of Record online : 15 JUN 2011, DOI: 10.1111/j.1399-3046.2011.01514.x

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    Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis


    Volume 41, Issue 5, May 2005, Pages: 1160–1172, Verena Keitel, Martin Burdelski, Ulrich Warskulat, Thomas Kühlkamp, Dietrich Keppler, Dieter Häussinger and Ralf Kubitz

    Version of Record online : 19 APR 2005, DOI: 10.1002/hep.20682

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    Allograft steatohepatitis in progressive familial intrahepatic cholestasis type 1 after living donor liver transplantation

    Liver Transplantation

    Volume 15, Issue 6, June 2009, Pages: 610–618, Aya Miyagawa-Hayashino, Hiroto Egawa, Tohru Yorifuji, Makoto Hasegawa, Hironori Haga, Tatsuaki Tsuruyama, Mei-Chin Wen, Ryo Sumazaki, Toshiaki Manabe and Shinji Uemoto

    Version of Record online : 28 MAY 2009, DOI: 10.1002/lt.21686

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    P4 ATPases – The physiological relevance of lipid flipping transporters

    FEBS Letters

    Volume 584, Issue 13, July 02, 2010, Pages: 2708–2716, Coen C. Paulusma and Ronald P.J. Oude Elferink

    Version of Record online : 7 MAY 2010, DOI: 10.1016/j.febslet.2010.04.071

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    Progressive familial intrahepatic cholestasis: Genetic disorders of biliary transporters

    Journal of Gastroenterology and Hepatology

    Volume 20, Issue 6, June 2005, Pages: 807–817, MATTHEW J HARRIS, DAVID G LE COUTEUR and IRWIN M ARIAS

    Version of Record online : 2 MAR 2005, DOI: 10.1111/j.1440-1746.2005.03743.x

  18. Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis


    Accepted manuscript online: 27 DEC 2016, Kasper S. Wang, Greg Tiao, Lee M. Bass, Paula M. Hertel, Douglas Mogul, Nanda Kerkar, Matthew Clifton, Colleen Azen, Laura Bull, Philip Rosenthal, Dylan Stewart, Riccardo Superina, Ronen Arnon, Molly Bozic, Mary L. Brandt, Patrick A. Dillon, Annie Fecteau, Kishore Iyer, Binita Kamath, Saul Karpen, Frederick Karrer, Kathleen M. Loomes, Cara Mack, Peter Mattei, Alexander Miethke, Kyle Soltys, Yumirle P. Turmelle, Karen West, Jessica Zagory, Cat Goodhue, Benjamin L. Shneider and on behalf of theChildhood Liver Disease Research Network (ChiLDReN)

    DOI: 10.1002/hep.29019

  19. Progressive familial intrahepatic cholestasis: a single-center experience of living-donor liver transplantation during two decades in Japan

    Clinical Transplantation

    Volume 25, Issue 5, September/October 2011, Pages: 776–785, Tomohide Hori, Hiroto Egawa, Yasutsugu Takada, Mikiko Ueda, Fumitaka Oike, Yasuhiro Ogura, Seisuke Sakamoto, Mureo Kasahara, Kohei Ogawa, Aya Miyagawa-Hayashino, Yukihide Yonekawa, Tohru Yorifuji, Ken-Ichiro Watanabe, Hiraku Doi, Justin H. Nguyen, Feng Chen, Ann-Marie T. Baine, Lindsay B. Gardner and Shinji Uemoto

    Version of Record online : 16 DEC 2010, DOI: 10.1111/j.1399-0012.2010.01368.x

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    Functional analysis of the Notch ligand Jagged1 missense mutant proteins underlying Alagille syndrome

    The FEBS Journal

    Volume 279, Issue 12, June 2012, Pages: 2096–2107, Minoru Tada, Satsuki Itoh, Akiko Ishii-Watabe, Takuo Suzuki and Nana Kawasaki

    Version of Record online : 8 MAY 2012, DOI: 10.1111/j.1742-4658.2012.08595.x