Role of cholangiocytes in primary biliary cirrhosis

Ana Lleo, Luca Maroni, Shannon Glaser, Gianfranco Alpini, Marco Marzioni

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by selective destruction of intrahepatic cholangiocytes. Mechanisms underlying the development and progression of the disease are still controversial and largely undefined. Evidence suggests that PBC results from an articulated immunologic response against an immunodominant mitochondrial autoantigen, the E2 component of the pyruvate dehydrogenase complex (PDC-E2); characteristics of the disease are also the presence of disease-specific antimitochondrial autoantibodies (AMAs) and autoreactive CD4 and CD8 T cells. Recent evidence suggests that cholangiocytes show specific immunobiological features that are responsible for the selective targeting of those cells by the immune system. The immune reaction in PBC selectively targets small sized, intrahepatic bile ducts; although a specific reason for that has not been defined yet, it has been established that the biliary epithelium displays a unique heterogeneity, for which the physiological and pathophysiological features of small and large cholangiocytes significantly differ. In this review article, the authors provide a critical overview of the current evidence on the role of cholangiocytes in the immune-mediated destruction of the biliary tree that characterizes PBC.

Original languageEnglish
Pages (from-to)273-284
Number of pages12
JournalSeminars in Liver Disease
Volume34
Issue number3
DOIs
Publication statusPublished - 2014

Fingerprint

Biliary Liver Cirrhosis
Dihydrolipoyllysine-Residue Acetyltransferase
Intrahepatic Bile Ducts
Autoantigens
Biliary Tract
Autoantibodies
Autoimmune Diseases
Disease Progression
Liver Diseases
Immune System
Epithelium
T-Lymphocytes

Keywords

  • apoptosis
  • cholangiocytes
  • intrahepatic bile ducts
  • mitochondrial antigens

ASJC Scopus subject areas

  • Hepatology
  • Medicine(all)

Cite this

Role of cholangiocytes in primary biliary cirrhosis. / Lleo, Ana; Maroni, Luca; Glaser, Shannon; Alpini, Gianfranco; Marzioni, Marco.

In: Seminars in Liver Disease, Vol. 34, No. 3, 2014, p. 273-284.

Research output: Contribution to journalArticle

Lleo, A, Maroni, L, Glaser, S, Alpini, G & Marzioni, M 2014, 'Role of cholangiocytes in primary biliary cirrhosis', Seminars in Liver Disease, vol. 34, no. 3, pp. 273-284. https://doi.org/10.1055/s-0034-1383727
Lleo, Ana ; Maroni, Luca ; Glaser, Shannon ; Alpini, Gianfranco ; Marzioni, Marco. / Role of cholangiocytes in primary biliary cirrhosis. In: Seminars in Liver Disease. 2014 ; Vol. 34, No. 3. pp. 273-284.
@article{f94690581b1141219a242763384e64e0,
title = "Role of cholangiocytes in primary biliary cirrhosis",
abstract = "Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by selective destruction of intrahepatic cholangiocytes. Mechanisms underlying the development and progression of the disease are still controversial and largely undefined. Evidence suggests that PBC results from an articulated immunologic response against an immunodominant mitochondrial autoantigen, the E2 component of the pyruvate dehydrogenase complex (PDC-E2); characteristics of the disease are also the presence of disease-specific antimitochondrial autoantibodies (AMAs) and autoreactive CD4 and CD8 T cells. Recent evidence suggests that cholangiocytes show specific immunobiological features that are responsible for the selective targeting of those cells by the immune system. The immune reaction in PBC selectively targets small sized, intrahepatic bile ducts; although a specific reason for that has not been defined yet, it has been established that the biliary epithelium displays a unique heterogeneity, for which the physiological and pathophysiological features of small and large cholangiocytes significantly differ. In this review article, the authors provide a critical overview of the current evidence on the role of cholangiocytes in the immune-mediated destruction of the biliary tree that characterizes PBC.",
keywords = "apoptosis, cholangiocytes, intrahepatic bile ducts, mitochondrial antigens",
author = "Ana Lleo and Luca Maroni and Shannon Glaser and Gianfranco Alpini and Marco Marzioni",
year = "2014",
doi = "10.1055/s-0034-1383727",
language = "English",
volume = "34",
pages = "273--284",
journal = "Seminars in Liver Disease",
issn = "0272-8087",
publisher = "Thieme Medical Publishers, Inc.",
number = "3",

}

TY - JOUR

T1 - Role of cholangiocytes in primary biliary cirrhosis

AU - Lleo, Ana

AU - Maroni, Luca

AU - Glaser, Shannon

AU - Alpini, Gianfranco

AU - Marzioni, Marco

PY - 2014

Y1 - 2014

N2 - Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by selective destruction of intrahepatic cholangiocytes. Mechanisms underlying the development and progression of the disease are still controversial and largely undefined. Evidence suggests that PBC results from an articulated immunologic response against an immunodominant mitochondrial autoantigen, the E2 component of the pyruvate dehydrogenase complex (PDC-E2); characteristics of the disease are also the presence of disease-specific antimitochondrial autoantibodies (AMAs) and autoreactive CD4 and CD8 T cells. Recent evidence suggests that cholangiocytes show specific immunobiological features that are responsible for the selective targeting of those cells by the immune system. The immune reaction in PBC selectively targets small sized, intrahepatic bile ducts; although a specific reason for that has not been defined yet, it has been established that the biliary epithelium displays a unique heterogeneity, for which the physiological and pathophysiological features of small and large cholangiocytes significantly differ. In this review article, the authors provide a critical overview of the current evidence on the role of cholangiocytes in the immune-mediated destruction of the biliary tree that characterizes PBC.

AB - Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by selective destruction of intrahepatic cholangiocytes. Mechanisms underlying the development and progression of the disease are still controversial and largely undefined. Evidence suggests that PBC results from an articulated immunologic response against an immunodominant mitochondrial autoantigen, the E2 component of the pyruvate dehydrogenase complex (PDC-E2); characteristics of the disease are also the presence of disease-specific antimitochondrial autoantibodies (AMAs) and autoreactive CD4 and CD8 T cells. Recent evidence suggests that cholangiocytes show specific immunobiological features that are responsible for the selective targeting of those cells by the immune system. The immune reaction in PBC selectively targets small sized, intrahepatic bile ducts; although a specific reason for that has not been defined yet, it has been established that the biliary epithelium displays a unique heterogeneity, for which the physiological and pathophysiological features of small and large cholangiocytes significantly differ. In this review article, the authors provide a critical overview of the current evidence on the role of cholangiocytes in the immune-mediated destruction of the biliary tree that characterizes PBC.

KW - apoptosis

KW - cholangiocytes

KW - intrahepatic bile ducts

KW - mitochondrial antigens

UR - http://www.scopus.com/inward/record.url?scp=84904993151&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84904993151&partnerID=8YFLogxK

U2 - 10.1055/s-0034-1383727

DO - 10.1055/s-0034-1383727

M3 - Article

C2 - 25057951

AN - SCOPUS:84904993151

VL - 34

SP - 273

EP - 284

JO - Seminars in Liver Disease

JF - Seminars in Liver Disease

SN - 0272-8087

IS - 3

ER -