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

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 lin...

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Veröffentlicht in:	Journal of hepatology 2018-12, Vol.69 (6), p.1371-1383
Hauptverfasser:	Rodrigues, Pedro M., Perugorria, Maria J., Santos-Laso, Alvaro, Bujanda, Luis, Beuers, Ulrich, Banales, Jesus M.
Format:	Artikel
Sprache:	eng
Schlagworte:	AE2 Autoantibodies Bile ducts Biliary bicarbonate Biomarkers - metabolism Chloride-Bicarbonate Antiporters - metabolism Cholangiocytes Cholangitis Cholestasis Cholestasis - metabolism Dihydrolipoyllysine-Residue Acetyltransferase - immunology DNA Methylation Environment Environmental factors Epigenesis, Genetic Epigenetics Genetic diversity Genetic Predisposition to Disease Humans Immune response Liver Cirrhosis, Biliary - genetics Liver Cirrhosis, Biliary - immunology Liver Cirrhosis, Biliary - metabolism Liver diseases MicroRNAs - genetics MicroRNAs - metabolism miR-506 miRNA Pathogenesis PBC Polymorphism, Single Nucleotide RNA, Long Noncoding - genetics RNA, Long Noncoding - immunology
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Beschreibung
Zusammenfassung:	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.
ISSN:	0168-8278 1600-0641
DOI:	10.1016/j.jhep.2018.08.020