Autoantibodies against integral membrane proteins of the nuclear envelope in patients with primary biliary cirrhosis

Gastroenterology. 1994 Jan;106(1):193-9. doi: 10.1016/s0016-5085(94)95333-3.


Background/aims: Autoantibodies against nuclear membrane proteins have been identified in patients with primary biliary cirrhosis (PBC). The aim of the present study was to determine the incidence of these autoantibodies in patients with PBC and examine their significance.

Methods: An assay using recombinant polypeptides was designed to unequivocally detect autoantibodies against gp210 and the lamin B receptor, integral proteins of the nuclear membranes.

Results: Autoantibodies against gp210 were detected in 15 of 159 patients with PBC and 0 of 46 controls. Autoantibodies against lamin B receptor were detected in 2 patients with PBC and 0 controls. The presence of these autoantibodies had a sensitivity of 11% and specificity of 100% for the diagnosis of PBC. Autoantibodies against gp210 were present in 4 of 19 (21%) patients with PBC who did not have detectable antimitochondrial antibodies. Patients with PBC and gp210 autoantibodies had a higher incidence of associated arthritis.

Conclusions: Autoantibodies against gp210 and the lamin B receptor are present in approximately 10% of patients with PBC. These autoantibodies are highly specific for the diagnosis of PBC and may be useful in diagnosing individuals without antimitochondrial antibodies and in identifying a subgroup of patients with an increased incidence of associated arthritis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies / immunology*
  • Fluorescent Antibody Technique
  • Humans
  • Liver Cirrhosis, Biliary / diagnosis
  • Liver Cirrhosis, Biliary / immunology*
  • Liver Cirrhosis, Biliary / physiopathology
  • Membrane Glycoproteins / immunology
  • Membrane Proteins / immunology*
  • Nuclear Envelope / metabolism*
  • Nuclear Pore Complex Proteins
  • Nuclear Proteins / immunology
  • Receptors, Cytoplasmic and Nuclear / immunology
  • Recombinant Proteins
  • Sensitivity and Specificity


  • Autoantibodies
  • Membrane Glycoproteins
  • Membrane Proteins
  • NUP210 protein, human
  • Nuclear Pore Complex Proteins
  • Nuclear Proteins
  • Receptors, Cytoplasmic and Nuclear
  • Recombinant Proteins
  • lamin B receptor