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| Previous Nomenclature | Nuclear membrane pores |
| Description | Nuclear envelope reveals a punctate staining in interphase cells, with accentuation of fluorescence at the points where adjacent cells touch each other. No staining of the metaphase and anaphase chromatin plates. e.g. anti-gp210. |
| Antigen Association | nuclear pore complex proteins (i.e. gp210) |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ Found in patients with PBC, as well as patients with other autoimmune liver diseases and SARD (61) ▶ If PBC is clinically suspected, it is recommended to perform a follow-up test for anti-gp210 antibodies; the antigen is included in disease specific immunoassays (i.e., extended liver profile*) (62–64) ▶ Other antigens recognized include p62 nucleoporin, LBR, and Tpr; specific immunoassays for these autoantibodies are currently not commercially available (65-68) *Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories. |
| First level information references |
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61. Miyachi K, Hankins RW, Matsushima H, et al. Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study. J Autoimmun 2003;20:247-54. 62. Courvalin JC, Lassoued K, Bartnik E, et al. The 210-kD nuclear envelope polypeptide recognized by human autoantibodies in primary biliary cirrhosis is the major glycoprotein of the nuclear pore. J Clin Invest 1990;86:279-85. 63. Nickowitz RE, Worman HJ. Autoantibodies from patients with primary biliary cirrhosis recognize a restricted region within the cytoplasmic tail of nuclear pore membrane glycoprotein gp210. J Exp Med 1993;178:2237-42. 64. Bandin O, Courvalin JC, Poupon R, et al. Specificity and sensitivity of gp210 autoantibodies detected using an enzyme-linked immunosorbent assay and a synthetic polypeptide in the diagnosis of primary biliary cirrhosis. Hepatology 1996;23:1020-4. 65. Wesierska-Gadek J, Klima A, Komina O, et al. Characterization of autoantibodies against components of the nuclear pore complexes: high frequency of anti-p62 nucleoporin antibodies. Ann N Y Acad Sci 2007;1109:519-30. 66. Kraemer DM, Tony HP. Nuclear pore protein p62 autoantibodies in systemic lupus erythematosus. Open Rheumatol J 2010;4:24-7. 67. Courvalin JC, Lassoued K, Worman HJ, et al. Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis. J Exp Med 1990;172:961-7. 68. Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein TPR is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379-87. |
| Second level information |
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▶ Anti‐p62 nucleoporin antibodies have been described in PBC and SLE (23, 24) ▶ Anti‐LBR antibodies have been described in PBC (25) ▶ Anti‐Tpr antibodies have been described in PBC, autoimmune liver disease, SLE, SSc and SjS (26) Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e., antigen‐specific immunoassays) and do not actually show clear correlations with the AC‐12 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available. |
| Second level information references |
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23. Wesierska-Gadek J, Klima A, Komina O, et al. Characterization of Autoantibodies against Components of the Nuclear Pore Complexes: High Frequency of Anti-p62 Nucleoporin Antibodies. Ann NY Acad Sci 2007;1109:519-30. 24. Kraemer DM, Tony H-P. Nuclear Pore Protein p62 Autoantibodies in Systemic Lupus Erythematosus. Open Rheumatol J 2010;4:24-7. 25. Courvalin J-C, Lassoued K, Worman HJ, et al. Identification and Characterization of Autoantibodies Against the Nuclear Envelope Lamin B Receptor from Patients with Primary Biliary Cirrhosis. J Exp Med 1990;172:961-7. 26. Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein Tpr is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379-87. |
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