Journal ArticleDOI
Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency.
A.S. Knisely,Sandra Strautnieks,Yvonne Meier,Bruno Stieger,J A Byrne,Bernard Portmann,Laura N. Bull,Ludmila Pawlikowska,Banu Bilezikçi,Figen Özçay,Aranka László,László Tiszlavicz,Lynette Moore,Jeremy Raftos,Henrik Arnell,Björn Fischler,Antal Nemeth,Nikos Papadogiannakis,Joanna Cielecka-Kuszyk,Irena Jankowska,Joanna Pawłowska,Hector Melin-Aldana,Karan M. Emerick,Peter F. Whitington,Giorgina Mieli-Vergani,Richard J. Thompson +25 more
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TLDR
PFIC associated with BSEP deficiency represents a previously unrecognized risk for HCC in young children and correlates well with demonstrable mutation in ABCB11.About:
This article is published in Hepatology.The article was published on 2006-08-01. It has received 329 citations till now. The article focuses on the topics: Progressive familial intrahepatic cholestasis & Neonatal hepatitis.read more
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EASL Clinical Practice Guidelines: management of cholestatic liver diseases
Ulrich Beuers,Kirsten Muri Boberg,Roger W. Chapman,Olivier Chazouillères,Pietro Invernizzi,David Jones,Frank Lammert,Albert Parés,Michael Trauner +8 more
TL;DR: The clinical care for patients with cholestatic liver diseases has advanced considerably during recent decades thanks to growing insight into pathophysiological mechanisms and remarkable methodological and technical developments in diagnostic procedures as well as therapeutic and preventive approaches.
Journal ArticleDOI
Bile acid-microbiota crosstalk in gastrointestinal inflammation and carcinogenesis.
TL;DR: The mechanistic links between bile acids and gastrointestinal carcinogenesis in CRC and HCC are discussed, which involve two major bile acid-sensing receptors, farnesoid X receptor (FXR) and G protein-coupled bile Acid receptor 1 (TGR5).
Journal ArticleDOI
From NASH to HCC: current concepts and future challenges.
Quentin M. Anstee,Quentin M. Anstee,Helen L. Reeves,Helen L. Reeves,Elena Kotsiliti,Olivier Govaere,Mathias Heikenwalder +6 more
TL;DR: This Review discusses NAFLD-associated HCC, including its epidemiology, key features that promote hepatocarcinogenesis and the management of HCC in patients with obesity and associated metabolic comorbidities, and the challenges and future directions of research.
Journal ArticleDOI
Xenobiotic, Bile Acid, and Cholesterol Transporters: Function and Regulation
TL;DR: A comprehensive overview of transporters of the solute carrier family (SLC) is provided with regard to tissue distribution, subcellular localization, and substrate preferences.
Journal ArticleDOI
Bile acid transporters
TL;DR: Current understanding of the physiological role and regulation of these bile acid transporters is reviewed, including the recently indentified heteromeric organic solute transporter, OSTα-OSTβ.
References
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Journal ArticleDOI
A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis
S S Strautnieks,Laura N. Bull,A.S. Knisely,S A Kocoshis,Niklas Dahl,H Arnell,Etienne Sokal,Karin Dahan,Sarah J. Childs,Ling,M. S. Tanner,Amir F. Kagalwalla,Antal Nemeth,Joanna Pawłowska,Amie Baker,Giorgina Mieli-Vergani,Nelson B. Freimer,R M Gardiner,Richard J. Thompson +18 more
TL;DR: Data provide evidence that SPGP is the human bile salt export pump (BSEP), and the product of the orthologous rat gene has been shown to be an effective bile acid transporter in vitro.
Journal ArticleDOI
A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis.
Laura N. Bull,van Eijk Mj,Ludmila Pawlikowska,Joseph DeYoung,J. A. Juijn,Liao M,Leo W.J. Klomp,Noureddine Lomri,Rolf M. F. Berger,Bruce F. Scharschmidt,Bruce F. Scharschmidt,A.S. Knisely,Roderick H. J. Houwen,Nelson B. Freimer +13 more
TL;DR: This gene, called FIC1, is the first identified human member of a recently described subfamily of P-type ATPases; ATP-dependent aminophospholipid transport is the previously described function of members of this subfamily.
Journal ArticleDOI
Bile acids as carcinogens in human gastrointestinal cancers.
Harris Bernstein,Carol Bernstein,Claire M. Payne,Katerina Dvorakova,Harinder S. Garewal,Harinder S. Garewal +5 more
TL;DR: The much larger number of cell generations in the colonic epithelia of humans compared to rodents may allow time for induction and selection of mutations leading to cancer in humans, although not in rodents.
Journal ArticleDOI
Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis.
Plm Jansen,S S Strautnieks,Emmanuel Jacquemin,Michelle Hadchouel,Etienne Sokal,Gjej Hooiveld,JH Koning,A. de Jager-Krikken,Folkert Kuipers,Frans Stellaard,Cma Bijleveld,Annette S. H. Gouw,van Harry Goor,Richard J. Thompson,Michael Müller +14 more
TL;DR: Investigation of expression of the bile salt export pump in liver samples from patients with a PFIC phenotype and correlated this with BSEP gene mutations shows a close correlation between BSEp gene mutations and canalicular B SEP expression.
Journal ArticleDOI
The human bile salt export pump: characterization of substrate specificity and identification of inhibitors.
J A Byrne,Sandra Strautnieks,Giorgina Mieli-Vergani,Christopher F. Higgins,Kenneth J. Linton,Richard J. Thompson +5 more
TL;DR: The human BSEP is a high-affinity bile salt transporter and is competitively inhibited by therapeutic drugs, a potentially significant mechanism for drug-induced cholestasis.
Related Papers (5)
Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families.
S S Strautnieks,J A Byrne,Ludmila Pawlikowska,Dita Cebecauerova,Anne Rayner,Laura Dutton,Yvonne Meier,Anthony Antoniou,Bruno Stieger,Henrik Arnell,Figen Özçay,Hussa F. Al-Hussaini,Atif F. Bassas,Henkjan J. Verkade,Björn Fischler,Antal Nemeth,Radana Kotalova,Benjamin L. Shneider,Joanna Cielecka-Kuszyk,Patricia McClean,Peter F. Whitington,Etienne Sokal,Milan Jirsa,Sami Wali,Irena Jankowska,Joanna Pawłowska,Giorgina Mieli-Vergani,A.S. Knisely,Laura N. Bull,Richard J. Thompson +29 more