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AC-18-胞浆散点型

同义词 GW小体，处理小体，溶酶体* GW body, processing body, lysosome*
描述

GW小体在间期细胞的胞浆中有染色，常见于S/G2晚期细胞。例如抗GW182抗体，抗anti-Su/Ago2抗体。

 Staining of GW bodies in the cytoplasm of interphase cells with high numbers in late S/G2 cells. e.g. anti-GW182, anti-Su/Ago2.
抗原相关性 GW182, Su/Ago2, *没有分子证据支持该模式与溶酶体靶抗原相关 GW182, Su/Ago2, *no molecular evidence to support this pattern is associated with lysosomal targets

  • 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


  • ►具有AC-18模式的自身抗体已在不同的SARD和多种其他疾病中被报道；它们在非选择或特定疾病组群中的流行率尚未得到深入研究(84)

    ►所识别的抗原包括GW小体 (处理中的或P小体) 抗原 (Ge-1/Hedls、GW182和Su/Ago2) 和胞内体抗原 (EEA1、CLIP-170、GRASP-1和LBPA)；这些自身抗体的特异性免疫检测试剂目前尚未商品化。

    注：针对GW小体和胞内体的自身抗体的HEp-2 细胞间接免疫荧光核型可能会有微小差异(84, 85)



    ► Autoantibodies revealing the AC-18 pattern have been reported in distinct SARD and in a variety of other diseases; their prevalence in unselected or specified disease cohorts has not been thoroughly studied (84)

    ► Antigens recognized include GW-body (Processing or P body) antigens (Ge-1/Hedls, GW182, and Su/Ago2) and endosomal antigens (EEA1, CLIP-170, GRASP-1, and LBPA); specific immunoassays for these autoantibodies are currently not commercially available

    Notes: Autoantibodies to GW-bodies and endosomes may yield slightly different HEp-2 IIFA patterns (84, 85).

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


  • 84. Bhanji RA, Eystathioy T, Chan EK, et al. Clinical and serological features of patients with autoantibodies to GW/P bodies. Clin Immunol 2007;125:247–56.

    85. Stinton LM, Eystathioy T, Selak S, et al. Autoantibodies to protein transport and messenger RNA processing pathways: endosomes, lysosomes, Golgi complex, proteasomes, assemblyosomes, exosomes, and GW bodies. Clin Immunol 2004;110:30–44.



    84. Bhanji RA, Eystathioy T, Chan EK, et al. Clinical and serological features of patients with autoantibodies to GW/P bodies. Clin Immunol 2007;125:247–56.

    85. Stinton LM, Eystathioy T, Selak S, et al. Autoantibodies to protein transport and messenger RNA processing pathways: endosomes, lysosomes, Golgi complex, proteasomes, assemblyosomes, exosomes, and GW bodies. Clin Immunol 2004;110:30–44.

  • 二级信息
    Second level information


  • 抗GW小体的自身抗体：

    ►在一项包含55例阳性血清的研究中，最常见的临床表现为神经方面症状 (即共济失调、运动感觉性神经病；33％)、干燥综合征(31％)，其他诊断包括系统性红斑狼疮、类风湿性关节炎和原发性胆汁性胆管炎 (28)

    ►激光免疫磁珠分析和重组蛋白的免疫沉淀分析表明，自身抗体直接与Ge-1/Hedls (58％)，GW182 (40％)和Su/Ago2 (16%) 这些抗原结合(28)

    抗胞内体成分的自身抗体：

    ►EEA1的自身抗体可见于多种病症，但~40% 的患者有神经系统疾病 (29)

    ►据报道，CLIP-170的自身抗体在4例不同疾病患者中检出，包括1例系统性红斑狼疮和自身免疫性肌病的原发型患者；其余3例患者患局限性皮肤型系统性硬化症、胶质母细胞瘤和特发性胸膜炎 (30)

    ►LBPA和GRASP-1自身抗体在非选择或特定疾病组群中尚未得到深入研究；在17％的原发性胆汁性胆管炎患者血清中检出抗GRASP-1自身抗体 (31, 32)

    注：大多数报道仅描述了自身抗体与特异性胞内抗原结合，实际上并未显示与AC-18核型的相关性，与GW小体或胞内体抗原反应的自身抗体的特异性免疫检测试剂目前尚未商品化



    utoantibodies to GW bodies:

    ► The most common clinical presentations in a single study with 55 positive sera were neurological symptoms (i.e. ataxia, motor and sensory neuropathy; 33%), SjS (31%), and the remainder had a variety of other diagnoses including SLE, RA, and PBC (28)

    ► Analysis by ALBIA and immunoprecipitation of recombinant proteins indicated that autoantibodies were directed against Ge-1/Hedls (58%), GW182 (40%), and Su/Ago2 (16%) (28)

    Autoantibodies to endosomal components:

    ► Autoantibodies to EEA1 were seen in a variety of conditions, but ~40% of the patients had a neurological disease (29)

    ► Autoantibodies to CLIP-170 were reported in 4 patients with different diseases including the prototype patient with SLE and AIM; the remaining 3 patients had limited cutaneous SSc, glioblastoma, and idiopathic pleural effusion (30)

    ► Autoantibodies to both LBPA and GRASP-1 have not been studied thoroughly in unselected or specified disease cohorts; anti-GRASP-1 autoantibodies were detected in 17% of PBC sera (31, 32)

    Notes: Most reports describing autoantibodies directly binding to specific endosomal antigens do not show correlations with the AC-18 pattern as such; specific immunoassays for the autoantibodies reacting with antigens of GW bodies or endosomes are currently not commercially available.

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


  • 28. Bhanji RA, Eystathioy T, Chan EKL, et al. Clinical and serological features of patients with autoantibodies to GW/P bodies. Clin Immunol 2007;125:247-56.

    29. Stinton LM, Eystathioy T, Selak S, et al. Autoantibodies to protein transport and messenger RNA processing pathways: endosomes, lysosomes, Golgi complex, proteasomes, assemblyosomes, exosomes, and GW bodies. Clin Immunol 2004;110:30-44.

    30. Griffith KJ, Ryan JP, Senecal JL, et al. The cytoplasmic linker protein CLIP-170 is a human autoantigen. Clin Exp Immunol 2002;127:533-8.

    31. Stinton LM, Selak S, Fritzler MJ. Identification of GRASP-1 as a novel 97 kDa autoantigen localized to endosomes. Clin Immunol 2005;116:108-17.

    32. Stinton LM, Swain M, Myers RP, et al. Autoantibodies to GW bodies and other autoantigens in primary biliary cirrhosis. Clin Exp Immunol 2011;163:147-56.



    28. Bhanji RA, Eystathioy T, Chan EKL, et al. Clinical and serological features of patients with autoantibodies to GW/P bodies. Clin Immunol 2007;125:247-56.

    29. Stinton LM, Eystathioy T, Selak S, et al. Autoantibodies to protein transport and messenger RNA processing pathways: endosomes, lysosomes, Golgi complex, proteasomes, assemblyosomes, exosomes, and GW bodies. Clin Immunol 2004;110:30-44.

    30. Griffith KJ, Ryan JP, Senecal JL, et al. The cytoplasmic linker protein CLIP-170 is a human autoantigen. Clin Exp Immunol 2002;127:533-8.

    31. Stinton LM, Selak S, Fritzler MJ. Identification of GRASP-1 as a novel 97 kDa autoantigen localized to endosomes. Clin Immunol 2005;116:108-17.

    32. Stinton LM, Swain M, Myers RP, et al. Autoantibodies to GW bodies and other autoantigens in primary biliary cirrhosis. Clin Exp Immunol 2011;163:147-56.

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