简体中文

AC-12-点状核膜型

同义词 核膜孔 Nuclear membrane pores
描述
间期细胞核膜呈现颗粒状荧光，临近细胞相触部位的荧光增强。分裂中期和后期细胞的染色体阴性。
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.
抗原相关性 核孔复合蛋白(例如，gp210,p62, Tpr) Nuclear pore complex proteins (i.e. gp210, p62, Tpr).

  • 临床相关性

    一级信息

    关于临床相关性和缩写列表

    Clinical Relevance

    First level information

    About Clinical Relevance & List of Abbreviations
  • ▶发现于原发性胆汁性胆管炎患者，以及其他自身免疫性肝病和系统性自身免疫性风湿疾病患者 [1, 2].
    ▶如临床怀疑为原发性胆汁性胆管炎，建议随访检测抗gp210抗体[3-7].
    ▶其他被识别的抗原包括p62核孔蛋白和Tpr;目前还没有针对这些自身抗体的特异性免疫检测商品化试剂盒[8-10].
    ▶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 and Tpr; specific immunoassays for these autoantibodies are currently not commercially available [8-10].
  • 二级信息
    Second level information
  • ▶抗p62核孔蛋白抗体见于原发性胆汁性胆管炎和系统性红斑狼疮[8, 9].
    ▶抗Tpr抗体见于原发性胆汁性胆管炎、自身免疫性肝病、系统性红斑狼疮、系统性硬化症、特发性炎性肌病和干燥综合征[10,11].
    注释：AC-12 是抗核抗体（ANA）间接免疫荧光法中的一种标准荧光模式，特指点状核膜型。大多数报告都描述了直接结合特定抗原的自身抗体(例如，抗原特异性免疫检测)，实际上并没有显示出与AC-12核型的明确关联.
    ▶p62 nucleoporin antibodies have been described in PBC and systemic lupus erythematosus (SLE)[8, 9].
    ▶Tpr antibodies have been described in PBC, autoimmune liver diseases, SLE, systemic sclerosis, idiopathic inflammatory myopathies, and Sjögren’s disease [10,11].
    Notes: Several reports from basic science laboratories describe autoantibodies directly binding to specific nuclear pore complex antigens in antigen‐specific immunoassays do not clearly show correlations with the AC‐12 pattern.
  • 参考文献

  • 2026 Topic Review:
    Konstantinov KN, Konstantinov NK, de Melo Cruvinel W, Andrade LEC, Klotz W, Fritzler MJ. Autoantibodies to the nuclear envelope: Functional anatomy of the HEp-2 cell substrate. Autoimmun Rev. 2026:104154.
    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.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.
    11.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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