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

被引：58
作者：
Rodrigues, Pedro M. [1 ]
Perugorria, Maria J. [1 ,2 ,3 ]
Santos-Laso, Alvaro [1 ]
Bujanda, Luis [1 ,2 ]
Beuers, Ulrich [4 ,5 ]
Banales, Jesus M. [1 ,2 ,3 ]
机构：
[1] Univ Basque Country UPV EHU, Dept Liver & Gastrointestinal Dis, Biodonostia Hlth Res Inst, Donostia Univ Hosp, San Sebastian, Spain
[2] Inst Salud Carlos III, CIBERehd, Nat Inst Study Liver & Gastrointestinal Dis, Madrid, Spain
[3] Basque Fdn Sci, Ikerbasque, Bilbao, Spain
[4] AMC, Tytgat Inst Liver & Intestinal Res, Amsterdam, Netherlands
[5] AMC, Amsterdam Gastroenterol & Metab, Dept Gastroenterol & Hepatol, Amsterdam, Netherlands
来源：
JOURNAL OF HEPATOLOGY | 2018年 / 69卷 / 06期
关键词：
PBC; Cholangiocytes; Biliary bicarbonate; AE2; miR-506; ANION-EXCHANGER; 2; PLACEBO-CONTROLLED TRIAL; GENOME-WIDE ASSOCIATION; LONG NONCODING RNAS; INTERCELLULAR-ADHESION MOLECULE-1; SOLUBLE ADENYLYL-CYCLASE; BLOOD MONONUCLEAR-CELLS; BILE-DUCT EPITHELIA; URSODEOXYCHOLIC ACID; DOUBLE-BLIND;
D O I：
10.1016/j.jhep.2018.08.020
中图分类号：
R57 [消化系及腹部疾病];
学科分类号：
100201 [内科学];
摘要：
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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页码：1371 / 1383
页数：13
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