ICAP
AC-7 - 核少点型
  简体中文 English
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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Online since 19 May 2015