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| Previous Nomenclature | Nuclear membrane pores |
| Description | Nuclear envelope reveals 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. |
| Antigen Association | nuclear pore complex proteins (i.e. gp210, p62, Tpr, LBR). |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ Found in patients with primary biliary cholangitis (PBC), as well as patients with other autoimmune liver diseases and systemic autoimmune rheumatic diseases [1, 2] ▶ If PBC is clinically suspected, it is recommended to perform a follow-up test for gp210 antibodies[3-7] ▶ Other antigens recognized include p62 nucleoporin, LBR, and Tpr; specific immunoassays for these autoantibodies are currently not commercially available [8-11] |
| Second level information |
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▶ p62 nucleoporin antibodies have been described in PBC and systemic lupus erythematosus (SLE)[8, 9] ▶ LBR antibodies have been described in PBC [10] ▶ Tpr antibodies have been described in PBC, autoimmune liver disease, SLE, systemic sclerosis, idiopathic inflammatory myopathies, and Sjögren’s disease [11, 12] Most reports describe autoantibodies directly binding to specific nuclear pore complex antigens in antigen‐specific immunoassays that do not clearly show correlations with the AC‐12 pattern |
| References |
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1. Miyachi K, Hankins RW, Matsushima H, Kikuchi F, Inomata T, Horigome T, Shibata M, Onozuka Y, 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 2. Cristoferi L, Gerussi A, Invernizzi P. Anti-gp210 and other anti-nuclear pore complex autoantibodies in primary biliary cholangitis: What we know and what we should know. Liver Int. 2021;41:432-5 3. Courvalin JC, Lassoued K, Bartnik E, Blobel G, Wozniak RW. 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 4. 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 5. Bandin O, Courvalin JC, Poupon R, Dubel L, Homberg JC, Johanet C. 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 6. Jaskowski TD, Nandakumar V, Novis CL, Palmer M, Tebo AE. Presence of anti-gp210 or anti-sp100 antibodies in AMA-positive patients may help support a diagnosis of primary biliary cholangitis. Clin Chim Acta. 2023;540:117219 7. Deng HM, Hu SL, Chen WX, Huang Y. [Meta-analysis of anti-GP210 antibody and anti-SP100 antibody detection for diagnosis of primary biliary cirrhosis]. Zhonghua Gan Zang Bing Za Zhi. 2016;24:62-8 8. Wesierska-Gadek J, Klima A, Komina O, Ranftler C, Invernizzi P, Penner E. 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 9. Kraemer DM, Tony HP. Nuclear Pore Protein p62 Autoantibodies in Systemic Lupus Erythematosus. Open Rheumatol J. 2010;4:24-7 10. Courvalin JC, Lassoued K, Worman HJ, Blobel G. 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 11. Ou Y, Enarson P, Rattner JB, Barr SG, Fritzler MJ. 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 12. Senecal JL, Isabelle C, Fritzler MJ, Targoff IN, Goldstein R, Gagne M, Raynauld JP, Joyal F, et al. An autoimmune myositis-overlap syndrome associated with autoantibodies to nuclear pore complexes: description and long-term follow-up of the anti-Nup syndrome. Medicine (Baltimore). 2014;93:383-94 |
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