简体中文

AC-7-核少点型

同义词 1至6个核点，螺旋小体，卡哈尔体 1 to 6 nuclear dots, coiled body, Cajal bodies.
描述
每细胞核内有1到6个离散的小点 ,且染色质团块未被染色。这些小点也被称为卡哈尔小体，螺旋小体、核宝石小体或者卷曲小体双生小体。
One to 6 nuclear discrete speckles/cell. The chromatin mass is not stained. These speckles (dots) are known as Cajal bodies, coiled bodies and nuclear gems or ‘gemini of coiled bodies’.
抗原相关性 抗p80螺旋蛋白，生存运动神经元（SMN）复合物 （包括SMN蛋白及其他被称为gemin的蛋白质）。 p80 coilin, Survival Motor Neuron (SMN) complex (including SMN protein and other proteins known as gemins).

  • 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[1, 2]

    ▶抗原主要分布于核点中，这些核点含有 p80-螺旋蛋白（抗p80-coilin抗体）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 [3, 4]




    ▶The AC-7 pattern has low positive predictive value for any disease [1, 2]

    ▶Antigens primarily localized in the dots include p80-coilin and proteins of the SMN complex; specific immunoassays for these autoantibodies are currently not commercially available [3, 4]

  • 二级信息
    Second level information
  • ▶ p80-螺旋蛋白（抗p80-coilin抗体）在系统性红斑狼疮、硬皮病、干燥综合征中偶见 [1-3]
    ▶ 抗SMN抗体（不伴抗snRNP）可见于特发性炎性肌病或硬皮病-肌炎重叠综合征患者[4, 5]
    ▶ p80-螺旋蛋白（抗p80-coilin抗体）与抗DFS70可同时检出，提示AC-7核型也可出现在非自身免疫性风湿病人群中。
    ▶ p80-螺旋蛋白（抗p80-coilin抗体）和抗SMN的特异性可通过免疫印迹、重组蛋白固相免疫分析或免疫沉淀确认；但多数报道中的自身抗体检测与AC-7核型并无明确对应关系。

    ▶p80‐coilin antibodies may rarely occur in systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren’s disease SjD [1-3]

    ▶Isolated (without anti‐snRNPs) anti-SMN is reported in patients with idiopathic inflammatory myopathies (IIM) or SSc‐IIM overlap syndrome [4, 5]
    ▶The detection of both anti-p80-coilin (AC-7) and anti-DFS70 (AC-2) was reported in Japan [6] and it is also the experience in some reference laboratories in Europe. The implication is that AC-7 thus can be found in people without SARD as reported [6]
    ▶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 [3, 4, 7]
    Most reports describe autoantibodies directly binding antigen‐specific immunoassays and do not actually show clear correlations with the AC‐7 pattern



  • 参考文献
  • 1.Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun. 1999;13:225-32
    2.Fujimoto M, Kikuchi K, Tamaki T, Yazawa N, Kubo M, Ihn H, Sato S, Soma Y, et al. Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol. 1997;137:916-20
    3.Andrade LE, Chan EKL, Raska I, Peebles CL, Roos G, Tan EM. 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
    4.Satoh M, Chan JY, Ross SJ, Ceribelli A, Cavazzana I, Franceschini F, Li Y, Reeves WH, 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
    5.Landon-Cardinal O, Baril-Dionne A, Hoa S, Meyer A, Leclair V, Bourre-Tessier J, Mansour AM, Zarka F, et al. Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies. RMD Open. 2020;6
    6.Goto N, Sugiura K, Ogawa Y, Watanabe A, Onouchi H, Tomita Y, Muro Y. Anti-p80 coilin autoantibodies react with a conserved epitope and are associated with anti-DFS70/LEDGF autoantibodies. J Autoimmun. 2006;26:42-51
    7.Amlani A, Hazlewood GS, Hamilton L, Satoh M, Fritzler MJ. Autoantibodies to the survival of motor neuron complex in a patient with necrotizing autoimmune myopathy. Rheumatology (Oxford). 2018;57:199-200

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