Primary biliary cirrhosis and the nuclear pore complex

Carolina Duarte-Rey, Dimitrios Bogdanos, Chen Yen Yang, Krista Roberts, Patrick S Leung, Juan Manuel Anaya, Howard J. Worman, M. Eric Gershwin

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

Experimental models of autoimmune diseases have led to the conclusion that an immune response to nuclear antigens is a sentinel marker for loss of tolerance and potential tissue damage. Various proteins are targets of antinuclear antibodies in a variety of autoimmune diseases, ranging from systemic rheumatologic disorders to diseases affecting specific organs such as the liver. Autoantibodies against specific nuclear constituents have also been used as probes to understand the structure and the function of the targeted components and their relevance to disease pathogenesis. Approximately a quarter of patients with primary biliary cirrhosis (PBC) have antibodies targeting proteins of the nuclear pore complex (NPC), a multi-protein structure that mediates molecular transport across the nuclear envelope. Autoantibodies against the integral membrane glycoprotein gp210 and nucleoporin p62 appear to be highly specific for PBC, an autoimmune disease characterized by progressive destruction of intrahepatic biliary epithelial cells. This review discusses the diagnostic and clinical relevance of anti-NPC antibodies in PBC and the possibility that this autoimmune response may arise as a result of molecular mimicry.

Original languageEnglish (US)
Pages (from-to)898-902
Number of pages5
JournalAutoimmunity Reviews
Volume11
Issue number12
DOIs
StatePublished - Oct 2012

Keywords

  • Autoantibody
  • Autoantigen
  • Autoimmunity
  • Nuclear envelope
  • Nuclear pore complex
  • Nucleoporin
  • Primary biliary cirrhosis

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

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