4.8 Review

Primary biliary cholangitis: A tale of epigenetically-induced secretory failure?

Journal

JOURNAL OF HEPATOLOGY
Volume 69, Issue 6, Pages 1371-1383

Publisher

ELSEVIER SCIENCE BV
DOI: 10.1016/j.jhep.2018.08.020

Keywords

PBC; Cholangiocytes; Biliary bicarbonate; AE2; miR-506

Funding

  1. Spanish Ministry of Economy and Competitiveness [PI12/00380, PI15/01132, PI18/01075, PI14/00399, PI17/00022, RYC-2015-17755]
  2. Fondo Europeo de Desarrollo Regional (FEDER)
  3. ISCIII CIBERehd, Spain
  4. BIOEF (Basque Foundation for Innovation and Health Research: EiTB Maratoia) [BIO15/CA/016/BD]
  5. Diputacion Foral de Gipuzkoa [DFG15/010, DFG16/004]
  6. Department of Health of the Basque Country [2015111100, 2017111010]
  7. Fundacion Cientifica de la Asociacion Espanola Contra el Cancer (AECC Scientific Foundation)
  8. Basque Government [PRE_2015_1_0126]

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Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease associated with auto-immunerelated destruction of small to medium size intrahepatic bile ducts. The aetiology of PBC is unknown and its pathogenesis remains obscure. Both genetic variants and environmental factors have been linked to increased PBC susceptibility, with other alterations known to cooperate in disease pathobiology. Increasing evidence indicates the presence of epigenetic abnormalities in PBC, particularly alterations of cholangiocellular microRNAs (miRNAs or miRs). This review highlights and discusses the most relevant epigenetic alterations found in patients with PBC, focusing on the role of miR-506 in the promotion of cholestasis and immune activation. (C) 2018 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

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