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Article

Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity.

Gastroenterology - 1 Mar 2004

Chen Frank, Ananthanarayanan M, Emre Sukru, Neimark Ezequiel, Bull Laura N, Knisely A S, Strautnieks Sandra S, Thompson Richard J, Magid Margret S, Gordon Ronald, Balasubramanian N, Suchy Frederick J, Shneider Benjamin L

Abstract excerpt

BACKGROUND & AIMS: The mechanisms by which mutations in the familial intrahepatic cholestasis-1 gene cause Byler's disease (progressive familial intrahepatic cholestasis type 1) are unknown. METHODS: Interactions among the apical sodium-dependent bile acid transporter, the farnesoid X receptor (FXR), and familial intrahepatic cholestasis-1 were studied in the ileum of children with progressive familial...

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PMID: 14988830
DOI: 10.1053/j.gastro.2003.12.013

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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