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AC-7-核少点型

同义词 1至6个核点，螺旋小体，卡哈尓体 1 to 6 nuclear dots, coiled body, Cajal bodies
描述

出现清晰可数的点状荧光（大多有1-6个核点），也称为螺旋小体和卡哈尔体。例如抗p80螺旋蛋白。

Countable discrete speckles (1 to 6 nuclear dots/cell in most cells).

These are known as Cajal bodies or coiled bodies. e.g. anti-p80-coilin
抗原相关性 P80-螺旋蛋白，SMN p80-coilin, SMN

  • 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


  • ►AC-7核型对疾病的阳性预测值较低(40,41)。

    ►抗原主要分布于核点中，这些核点含有p80-螺旋蛋白与SMN复合体等。目前针对这些自身抗体的特异性免疫检测尚无商品化试剂(42,43)。



    ► The AC-7 pattern has low positive predictive value for any disease (40, 41)

    ► Antigens primarily localized in the dots include p80-coilin and SMN complex; specific immunoassays for these autoantibodies are currently not commercially available (42, 43)

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


  • 40. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun 1999;13:225–32.

    41. Fujimoto M, Kikuchi K, Tamaki T, et al. Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol 1997;137:916–20.

    42. Andrade LE, Chan EK, Raska I, et al. Human autoantibody to a novel protein of the nuclear coiled body: immunological characterization and cDNA cloning of p80-coilin. J Exp Med 1991;173:1407–19.

    43. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to survival of motor neuron complex in patients with polymyositis: immunoprecipitation of D, E, F, and G proteins without other components of small nuclear ribonucleoproteins. Arthritis Rheum 2011;63:1972–8.



    40. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun 1999;13:225–32.

    41. Fujimoto M, Kikuchi K, Tamaki T, et al. Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol 1997;137:916–20.

    42. Andrade LE, Chan EK, Raska I, et al. Human autoantibody to a novel protein of the nuclear coiled body: immunological characterization and cDNA cloning of p80-coilin. J Exp Med 1991;173:1407–19.

    43. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to survival of motor neuron complex in patients with polymyositis: immunoprecipitation of D, E, F, and G proteins without other components of small nuclear ribonucleoproteins. Arthritis Rheum 2011;63:1972–8.

  • 二级信息
    Second level information


  • ►抗p80-螺旋蛋白抗体可能罕见于系统性红斑狼疮、系统性硬化症（局限型），以及干燥综合征等(15-17)。

    ►有报道于自身免疫性肌病患者或系统性硬化症-自身免疫性肌病重叠综合征患者中分离到了抗SMN抗体，同时未分离到抗snRNP抗体(18)。

    ►特异性的抗p-80螺旋蛋白抗体与抗SMN复合体抗体可运用蛋白免疫印迹法、重组蛋白与免疫沉淀的固相免疫法进行检测确认(15,18,19)。

    注：多数研究报道的是直接结合特异性抗原的自身抗体（例如，通过抗原特异性免疫检测法检测），并且未报道这些抗原与AC-7明确相关。目前尚无针对这些自身抗体的商品化免疫检测试剂。


    ► Anti‐p80‐coilin antibodies may rarely occur in SLE, (localized linear) SSc, and SjS (15‐17)

    ► Isolated (without anti‐snRNPs) anti‐SMN antibodies are reported in patients with AIM or SSc‐AIM overlap syndrome (18)

    ► The specificity of antibodies to p80‐coilin and the SMN complex can be confirmed by Western blot, solid phase immunoassays using recombinant proteins and immunoprecipitation (15, 18, 19)

    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‐7 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


  • 15. Andrade LE, Chan EK, Raska I, et al. Human autoantibody to a novel protein of the nuclear coiled body: immunological characterization and cDNA cloning of p80‐coilin. J Exp Med 1991;173:1407‐19.

    16. Fujimoto M, Kikuchi K, Tamaki T, et al. Distribution of anti‐p80‐coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol 1997;137:916‐20.

    17. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti‐p80 coilin antibodies. J Autoimmun 1999;13:225‐32.

    18. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to survival of motor neuron (SMN) complex in patients with polymyositis – immunoprecipitation of D‐EF‐G without other components of small nuclear ribonucleoproteins. Arthritis Rheum 2011;63:1972‐8.

    19. Amlani A, Hazlewood GS, Hamilton L, et al. Autoantibodies to the survival of motor neuron complex in a patient with necrotizing autoimmune myopathy. Rheumatology 2018;57:199‐200.


    15. Andrade LE, Chan EK, Raska I, et al. Human autoantibody to a novel protein of the nuclear coiled body: immunological characterization and cDNA cloning of p80‐coilin. J Exp Med 1991;173:1407‐19.

    16. Fujimoto M, Kikuchi K, Tamaki T, et al. Distribution of anti‐p80‐coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol 1997;137:916‐20.

    17. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti‐p80 coilin antibodies. J Autoimmun 1999;13:225‐32.

    18. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to survival of motor neuron (SMN) complex in patients with polymyositis – immunoprecipitation of D‐EF‐G without other components of small nuclear ribonucleoproteins. Arthritis Rheum 2011;63:1972‐8.

    19. Amlani A, Hazlewood GS, Hamilton L, et al. Autoantibodies to the survival of motor neuron complex in a patient with necrotizing autoimmune myopathy. Rheumatology 2018;57:199‐200.


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