Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study

J Autoimmun. 2003 May;20(3):247-54. doi: 10.1016/s0896-8411(03)00033-7.


Primary biliary cirrhosis (PBC) sera contain antibodies which recognize various nuclear envelope proteins of which antibody against gp210 has been proven to be diagnostic for disease. In contrast, the clinical significance of another nuclear envelope antibody, anti-p62 antibody has not been well investigated. In the present study, we have analyzed anti-nuclear envelope antibodies by indirect immunofluorescence and immunoblot using rat liver nuclear envelope proteins and wheat germ agglutinin-bound fraction. Test sera were obtained from 175 patients with PBC and from 120 controls. Anti-gp210, anti-lamina associated polypeptide 2, anti-lamin B receptor, and anti-p62 complex antibodies were detected with a frequency of 26% (46 of 175), 6% (11 of 175), 9% (16 of 175), and 13% (15 of 115), respectively. The confirmation of Scheuer's stage IV was made with a frequency of 27% (4 of 15) in PBC patients with anti-p62 complex antibody, in contrast to only 2% (2 of 100) in PBC patients without anti-p62 complex antibody. This difference was found to be statistically significant. The presence of anti-p62 complex antibody may be related with the progressive or advanced state of PBC.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies, Antinuclear / blood*
  • Case-Control Studies
  • DNA-Binding Proteins / immunology
  • Female
  • Fluorescent Antibody Technique, Indirect
  • Humans
  • Immunoblotting
  • In Vitro Techniques
  • Liver Cirrhosis, Biliary / immunology*
  • Liver Cirrhosis, Biliary / pathology
  • Male
  • Membrane Glycoproteins / immunology
  • Membrane Proteins / immunology
  • Middle Aged
  • Nuclear Envelope / immunology*
  • Nuclear Pore Complex Proteins
  • Nuclear Proteins / immunology
  • Rats
  • Receptors, Cytoplasmic and Nuclear / immunology


  • Antibodies, Antinuclear
  • DNA-Binding Proteins
  • Membrane Glycoproteins
  • Membrane Proteins
  • NUP210 protein, human
  • Nuclear Pore Complex Proteins
  • Nuclear Proteins
  • Receptors, Cytoplasmic and Nuclear
  • lamin B receptor
  • lamina-associated polypeptide 2
  • nuclear pore protein p62