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Nuclear receptor FXR,bile acids and liver damage: Introducing the progressive familial intrahepatic cholestasis with FXR mutations
Authors:Marica Cariello  Elena Piccinin  Oihane Garcia-Irigoyen  Carlo Sabbà  Antonio Moschetta
Affiliation:1. Department of Interdisciplinary Medicine, “Aldo Moro” University of Bari, 70124 Bari, Italy;2. INBB, National Institute for Biostructures and Biosystems, 00136 Rome, Italy;3. National Cancer Center, IRCCS Istituto Oncologico “Giovanni Paolo II”, 70124 Bari, Italy
Abstract:
Keywords:NR  nuclear receptor  FXR  farnesoid X receptor  RXR  retinoid X receptor  PXR  pregnane X receptor  VDR  Vitamin D receptor  TGR5  G protein–coupled bile acid receptor  Lrh1  liver receptor homolog 1  Mdr  multidrug resistance protein  Abcb4  ATP Binding Cassette Subfamily B Member 4  Bsep  bile salt export pump  Abcb11  ATP Binding Cassette Subfamily B Member 11  BACS  BA CoA synthase  BAAT  SHP  small heterodimer partner  CYP7A1  cytochrome P450 7A1  MRP2  multidrug related protein 2  DBD  DNA binding domain  LBD  ligand binding domain  BA  bile acids  CA  cholic acid  CDCA  chenodeoxycholic acid  OCA  obeticholic acid  UDCA  ursodeoxycholic acid  HCC  hepatocellular carcinoma  IBD  inflammatory bowel disease  PFIC  progressive familial intrahepatic cholestasis  ICP  cholestasis of pregnancy  DIC  drug-induced cholestasis  BRIC  benign recurrent intrahepatic cholestasis  FGFR4  fibroblast growth factor receptor 4  FGF15/19  fibroblast growth factor 15/19  TNFα  tumor necrosis factor α  BAK1  BCL-2 antagonist killer 1  FADD  Fas-associated death domain  BCL2  B cell lymphoma gene-2  NF-κB  nuclear factor-κB  HSC  hepatic stellate cells  CTGF  connective tissue growth factor  TGF-β  transforming growth factor-β  PDGF  platelet-derived growth factor  NTCP  sodium (Na)-Taurocholate Cotransporter Protein  OSTα/β  organic solute transporter  OATP  organic anion transporting polypeptide  Nuclear receptors  Bile acids  Farnesoid X receptor (FXR)  Fibroblast growth factor 19 (FGF19)  Cholestasis
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