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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。例如抗gp210抗体。

 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.
抗原相关性 核孔复合蛋白（例如，gp210） nuclear pore complex proteins (i.e. gp210)

  • 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

    ►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;该抗原包含在疾病特异性免疫测定试剂中（例如，扩展的自免肝抗体谱）（62-64）

    ►其他被识别的抗原包括p62核孔蛋白、LBR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（65-68）

    *实用的炎症性肌病谱、系统性硬化症谱和自免肝抗体谱(扩展版)检测可能仅限于专业的临床实验室。


    ► 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.

  • 一级信息参考文献
    First level information references

  • 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.


    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.

  • 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（23-24）

    ►抗LBR抗体见于原发性胆汁性胆管炎（25）

    ►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、系统性硬化症和干燥综合征（26）

    注意：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；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。


    ► 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.

  • 二级信息参考文献
    Second level information references

  • 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.


    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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