Antinuclear antibodies specific for primary biliary cirrhosis.
The serological hallmark of primary biliary cirrhosis (PBC) is the presence of antimitochondrial antibodies. However, antinuclear antibodies (ANA) are also detectable in approximately 50% of subjects with PBC. Most clinical laboratories use indirect immunofluorescence microscopy to detect ANA and two labeling patterns that predominate in PBC are 'punctate nuclear rim' and 'multiple nuclear dots.' Work over the past several years has shown that antibodies giving these patterns most often recognize nuclear pore membrane protein gp210 and nuclear body protein sp100, respectively. These ANA are highly specific for PBC and detected in approximately 25% of patients. Less frequently, ANA apparently unique to PBC recognize other proteins of the nuclear envelope and nuclear bodies. While antibodies against gp210, sp100 and some other nuclear proteins are very specific to PBC and may therefore be useful diagnostic markers, their connection to pathogenesis remains to be elucidated.
Worman
Howard J
HJ
Department of Medicine, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, 10th Floor, Room 508, New York, NY 10032, USA.
Courvalin
Jean Claude
JC
D034961:Antigens, Nuclear / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D002467:Cell Nucleus / Q000276:immunology*; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000276:immunology*; D008562:Membrane Glycoproteins / Q000276:immunology*; D028861:Nuclear Pore Complex Proteins; D009687:Nuclear Proteins / Q000276:immunology*
D034961:Antigens, Nuclear; D001323:Autoantibodies; D001324:Autoantigens; D008562:Membrane Glycoproteins; C063865:NUP210 protein, human; D028861:Nuclear Pore Complex Proteins; D009687:Nuclear Proteins; C066642:SP100 protein, human