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The immunopathology of human biliary cell epithelium

Seminars in Immunopathology volume 31, Article number: 323 (2009) Cite this article

Abstract

Bile ducts lined with biliary epithelial cells, or cholangiocytes, are the main components of the biliary system in liver. Cholangiocytes participate in the production and transport of bile substances, as well as participate in immune responses. Cholangiocytes protect against pathogens by expressing toll-like receptors and anti-microbial peptides; act as antigen-presenting cells by expressing human leukocyte antigen molecules and costimulatory molecules; recruit leukocytes to the target site by expressing adhesion molecules, cytokines, and chemokines; and induce apoptosis of leukocytes to limit the immune responses. Several cholangiopathies result from dysfunctions of the biliary system. They can broadly be divided into autoimmune, genetic, infectious, drug, and ischemic-injury-induced categories. The pathogenesis of many of these cholangiopathies is unclear and treatment is limited. Further understanding of the complexity of the biliary system is critical for medical advancements in this field.

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Fig. 1

Abbreviations

CFTR:

cystic fibrosis transmembrane regulator

DR4, DR5:

death receptors 4 and 5

GVHD:

graft-vs-host disease

hBD-1:

human β-defensin 1

HLAs:

human leukocyte antigen

IFN-γ:

interferon γ

IRAK-M:

interleukin-1 receptor-associated kinase M

LPS:

lipopolysaccharide

PBC:

primary biliary cirrhosis

PSC:

primary sclerosing cholangitis

SC:

secretory component

SIgA:

secretory IgA

TLRs:

toll-like receptors

TNF-α:

tumor necrosis factor-α

TRAIL:

TNF-related apoptosis-inducing ligand

UCDA:

ursodeoxycholic acid

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Acknowledgments

This work was partly supported by a grant from the National Science Council, Taiwan (NSC97-2320-B-002-001-).

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Affiliations

  1. Department of Clinical Laboratory Sciences and Medical Biotechnology, College of Medicine, National Taiwan University, Taipei, Taiwan

    Ya-Hui Chuang

  2. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 E. Health Sciences Drive, Suite 6510, Davis, 95616, CA, USA

    Ruth Y. Lan & M. Eric Gershwin

Authors
  1. Ya-Hui Chuang
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  2. Ruth Y. Lan
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  3. M. Eric Gershwin
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Corresponding author

Correspondence to M. Eric Gershwin.

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YH Chuang and RY Lan contributed equally to this work.

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Chuang, YH., Lan, R.Y. & Eric Gershwin, M. The immunopathology of human biliary cell epithelium. Semin Immunopathol 31, 323 (2009). https://doi.org/10.1007/s00281-009-0172-5

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Keywords

  • Biliary epithelium
  • Cholangiocytes
  • Cholangiopathy
  • Immune response