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- Title
FIC1-mediated stimulation of FXR activity is decreased with PFIC1 mutations in HepG2 cells.
- Authors
Koh, Saori; Takada, Tappei; Kukuu, Ikuya; Suzuki, Hiroshi
- Abstract
Progressive familial intrahepatic cholestasis type 1 (PFIC1) is a specific form of genetic cholestasis caused by functional defects in FIC1/ATP8B1. Although the way FIC1 deficiency leads to PFIC1 remains unclear, some reports suggest that the loss of FIC1 function results in decreased activity of the farnesoid X receptor (FXR) in PFIC1 patients. In this study, in order to elucidate the molecular mechanism of the pathogenesis of PFIC1, we constructed an experimental system for the evaluation of FIC1-mediated stimulatory effects on FXR activity.
- Publication
Journal of gastroenterology, 2009, Vol 44, Issue 6, p592
- ISSN
0944-1174
- Publication type
Journal Article
- DOI
10.1007/s00535-009-0041-y