A farnesoid X receptor T296I variant disrupts ligand-induced FXR activation and thus bile acid transport in progressive familial intrahepatic cholestasis.
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| Title: | A farnesoid X receptor T296I variant disrupts ligand-induced FXR activation and thus bile acid transport in progressive familial intrahepatic cholestasis. |
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| Authors: | Behrendt A; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany., Bastianelli A; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany., Stindt J; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany., Pfister ED; Pediatric Gastroenterology and Hepatology, Department for Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany., Sgodda M; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany., Cantz T; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany., Hook S; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany., Gopalswamy M; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany., Grau K; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany., Brands S; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany., Dröge C; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany., Stalke A; Department of Human Genetics, Hannover Medical School, Hannover, Germany., Bonus M; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany., Franke S; Institute of Pathology, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany., Baumann U; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany., Keitel V; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany. Electronic address: verena.keitel-anselmino@med.ovgu.de., Gohlke H; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany; Institute of Bio- and Geosciences (IBG-4: Bioinformatics), Forschungszentrum Jülich GmbH, Jülich, Germany. Electronic address: gohlke@uni-duesseldorf.de. |
| Source: | The Journal of biological chemistry [J Biol Chem] 2025 Nov; Vol. 301 (11), pp. 110769. Date of Electronic Publication: 2025 Sep 29. |
| Publication Type: | Journal Article; Research Support, Non-U.S. Gov't |
| Journal Info: | Publisher: Elsevier Inc. on behalf of American Society for Biochemistry and Molecular Biology Country of Publication: United States NLM ID: 2985121R Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1083-351X (Electronic) Linking ISSN: 00219258 NLM ISO Abbreviation: J Biol Chem Subsets: MEDLINE |
| Database: | MEDLINE Ultimate |
| FullText | Text: Availability: 0 |
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| Header | DbId: mdl DbLabel: MEDLINE Ultimate An: 41033550 AccessLevel: 2 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: A farnesoid X receptor T296I variant disrupts ligand-induced FXR activation and thus bile acid transport in progressive familial intrahepatic cholestasis. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AU" term="%22Behrendt+A%22">Behrendt A</searchLink>; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Bastianelli+A%22">Bastianelli A</searchLink>; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany.<br /><searchLink fieldCode="AU" term="%22Stindt+J%22">Stindt J</searchLink>; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Pfister+ED%22">Pfister ED</searchLink>; Pediatric Gastroenterology and Hepatology, Department for Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany.<br /><searchLink fieldCode="AU" term="%22Sgodda+M%22">Sgodda M</searchLink>; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany.<br /><searchLink fieldCode="AU" term="%22Cantz+T%22">Cantz T</searchLink>; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany.<br /><searchLink fieldCode="AU" term="%22Hook+S%22">Hook S</searchLink>; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany.<br /><searchLink fieldCode="AU" term="%22Gopalswamy+M%22">Gopalswamy M</searchLink>; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Grau+K%22">Grau K</searchLink>; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Brands+S%22">Brands S</searchLink>; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Dröge+C%22">Dröge C</searchLink>; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Stalke+A%22">Stalke A</searchLink>; Department of Human Genetics, Hannover Medical School, Hannover, Germany.<br /><searchLink fieldCode="AU" term="%22Bonus+M%22">Bonus M</searchLink>; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany.<br /><searchLink fieldCode="AU" term="%22Franke+S%22">Franke S</searchLink>; Institute of Pathology, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany.<br /><searchLink fieldCode="AU" term="%22Baumann+U%22">Baumann U</searchLink>; Research Center for Translational Regenerative Medicine, Hannover Medical School, Hannover, Germany.<br /><searchLink fieldCode="AU" term="%22Keitel+V%22">Keitel V</searchLink>; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Magdeburg, Otto von Guericke University, Magdeburg, Germany; Medical Faculty, Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany. Electronic address: verena.keitel-anselmino@med.ovgu.de.<br /><searchLink fieldCode="AU" term="%22Gohlke+H%22">Gohlke H</searchLink>; Institute for Pharmaceutical and Medicinal Chemistry, Heinrich Heine University, Düsseldorf, Germany; Institute of Bio- and Geosciences (IBG-4: Bioinformatics), Forschungszentrum Jülich GmbH, Jülich, Germany. Electronic address: gohlke@uni-duesseldorf.de. – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%222985121R%22">The Journal of biological chemistry</searchLink> [J Biol Chem] 2025 Nov; Vol. 301 (11), pp. 110769. <i>Date of Electronic Publication: </i>2025 Sep 29. – Name: TypePub Label: Publication Type Group: TypPub Data: Journal Article; Research Support, Non-U.S. Gov't – Name: TitleSource Label: Journal Info Group: Src Data: <i>Publisher: </i><searchLink fieldCode="PB" term="%22Elsevier+Inc%2E+on+behalf+of+American+Society+for+Biochemistry+and+Molecular+Biology%22">Elsevier Inc. on behalf of American Society for Biochemistry and Molecular Biology </searchLink><i>Country of Publication: </i>United States <i>NLM ID: </i>2985121R <i>Publication Model: </i>Print-Electronic <i>Cited Medium: </i>Internet <i>ISSN: </i>1083-351X (Electronic) <i>Linking ISSN: </i><searchLink fieldCode="IS" term="%2200219258%22">00219258 </searchLink><i>NLM ISO Abbreviation: </i>J Biol Chem <i>Subsets: </i>MEDLINE |
| PLink | https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=mdl&AN=41033550 |
| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1016/j.jbc.2025.110769 Languages: – Code: eng Text: English PhysicalDescription: Pagination: StartPage: 110769 Titles: – TitleFull: A farnesoid X receptor T296I variant disrupts ligand-induced FXR activation and thus bile acid transport in progressive familial intrahepatic cholestasis. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Behrendt A – PersonEntity: Name: NameFull: Bastianelli A – PersonEntity: Name: NameFull: Stindt J – PersonEntity: Name: NameFull: Pfister ED – PersonEntity: Name: NameFull: Sgodda M – PersonEntity: Name: NameFull: Cantz T – PersonEntity: Name: NameFull: Hook S – PersonEntity: Name: NameFull: Gopalswamy M – PersonEntity: Name: NameFull: Grau K – PersonEntity: Name: NameFull: Brands S – PersonEntity: Name: NameFull: Dröge C – PersonEntity: Name: NameFull: Stalke A – PersonEntity: Name: NameFull: Bonus M – PersonEntity: Name: NameFull: Franke S – PersonEntity: Name: NameFull: Baumann U – PersonEntity: Name: NameFull: Keitel V – PersonEntity: Name: NameFull: Gohlke H IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 11 Text: 2025 Nov Type: published Y: 2025 Identifiers: – Type: issn-electronic Value: 1083-351X Numbering: – Type: volume Value: 301 – Type: issue Value: 11 Titles: – TitleFull: The Journal of biological chemistry Type: main |
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