Primary biliary cholangitis: A tale of epigenetically-induced secretory failure?
Author(s): ,
Jesus M. Banales
Affiliations:
Ikerbasque, Basque Foundation for Science, Bilbao, Spain
Corresponding author. Address: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, Paseo del Dr. Begiristain s/n, E-20014 San Sebastian, Spain; Tel.: +34 943006067. This author was the
,
Ulrich Beuers
Affiliations:
Tytgat Institute for Liver and Intestinal Research and Department of Gastroenterology & Hepatology, Amsterdam Gastroenterology and Metabolism, AMC, Amsterdam, The Netherlands
,
Luis Bujanda
Affiliations:
National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, “Instituto de Salud Carlos III”), Spain
,
Alvaro Santos-Laso
Affiliations:
Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
,
Maria J. Perugorria
Affiliations:
Ikerbasque, Basque Foundation for Science, Bilbao, Spain
Pedro M. Rodrigues
Affiliations:
Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
EASL LiverTree™. Banales J. Dec 1, 2018; 256778
Dr. Jesus Banales
Dr. Jesus Banales

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Summary

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease associated with autoimmune-related 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.

Keyword(s)
PBC, Cholangiocytes, Biliary bicarbonate, AE2, miR-506
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