Nuclear envelope protein autoantibodies/antilamin autoantibodies

Aroundthe mid-1980s, a number of nuclear envelope structures have been recognized as specific targets of antinuclear autoantibodies (ANA) producing a rim-like staining (anti-NE) at indirect immunofluorescence. In particular, peripheral pattern results from autoantibodies directed against proteins within three components of the nuclear envelope: the nuclear lamina (lamins A, B, and C), the nuclear pore complex (gp210, p62, and Tpr), and the inner nuclear membrane (LAP1, LAP2, and LBR). Although the fluorescent ANA test using HEp-2 cells is still the most widely used screening assay for anti-NE testing, new accurate immunochemical tests based on enzyme-linked immunosorbent (ELISA) and immunoblotting with purified antigens have been developed. Anti-NE is generally believed to be non-pathogenic but represent specific disease markers and is useful diagnostic tools. Immunoreactivities against components of the nuclear pore complex have been shown to be associated with primary biliary cirrhosis (PBC), whereas autoantibodies to the lamins are associated with various autoimmune diseases, such as autoimmune hepatitis, systemic lupus erythematosus, and antiphospholipid antibodies. In the last few years, data from different groups indicated that PBC-specific anti-NE correlate with disease severity and may, therefore, be a marker of poor prognosis. Much less is known, however, regarding the clinical and prognostic role of anti-NE in other autoimmune conditions.