简体中文

AC-18-胞浆散点型

同义词 GW小体（GW bodies）、P小体（processing bodies）、内涵体（endosomes）、溶酶体（lysosomes） GW bodies, processing bodies, endosomes, lysosomes
描述
胞质内可见散在分布的离散型点状荧光。
Discrete dots in the cytoplasm
抗原相关性 GW小体通常为间期细胞胞质内的球形结构，在S晚期/G2期细胞中数量增多，参与mRNA加工。电镜下，该结构呈高电子密度，无界膜包绕。相比之下，内涵体与溶酶体均为膜结合细胞器。内涵体参与吞噬作用及高尔基体蛋白质的输出，而溶酶体则含有能够分解蛋白质、核酸、碳水化合物及脂质的消化酶。 GW小体相关抗原：​ GW182、Su/Ago2、Ge-1/人类脱帽大亚基增强子（Hedls）[1,2]。 内涵体相关抗原：​ 早期内涵体抗原1（EEA-1）、胞质连接蛋白170（CLIP-170）、谷氨酸受体相互作用蛋白（GRIP）相关蛋白1（GRASP-1/GRIPAP）、溶血双磷脂酸-1 [1]。 溶酶体相关抗原：​ 自噬蛋白微管相关轻链3（LC3）[3]；溶酶体相关膜蛋白（LAMPs）抗体已有报道，尤以LAMP2为主，但尚无证据表明其可产生AC-18核型[4–6]。 上述自身抗体的特异性免疫检测方法目前尚无商业化产品。 注：​ 抗GW小体、内涵体及溶酶体自身抗体可能在HEp-2间接免疫荧光检测中呈现略有差异的染色模式[1,2]
GW bodies are generally spherical bodies in the cytoplasm of interphase cells with higher numbers in late S/G2 cells involved in mRNA processing. In electron microscopy the structures are electron dense and devoid of a limiting membrane. By comparison, endosomes and lysosomes are membrane bound organelles. Endosomes are involved in phagocytosis and export of proteins from the Golgi complex while lysosomes contain digestive enzymes capable of breaking down proteins, nucleic acids, carbohydrates, and lipids.
GW body associated antigens: GW182, Su/Ago2, Ge-1/human enhancer of decapping large subunit (Hedls) [1, 2].
▶ Endosome associated antigens: early endosome antigen 1 (EEA-1), cytoplasmic linker protein 170 (CLIP-170), glutamate receptor interacting protein (GRIP)-associated protein-1 (GRASP-1/GRIPAP), lysobisphosphatidic acid-1 [1].
▶ Lysosome associated antigen: autophagic protein microtubule-associated light-chain 3 (LC3) [3]; lysosome-associated membrane proteins (LAMPs) antibodies have been described particularly to LAMP2 but there is no evidence that they produce the AC-18 pattern [4-6].
▶ Specific immunoassays for these autoantibodies are currently not commercially available.
Notes: Autoantibodies to GW-bodies, endosomes, and lysosomes may yield slightly different HEp-2 IFA patterns [1, 2].

  • 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

  • ▶与AC-18核型相关的自身抗体已在系统性自身免疫性风湿病及多种其他疾病（包括肿瘤）中被报道[2,7]；然而，其在非选择性或特定疾病队列中的患病率尚未得到系统研究。


    ▶Autoantibodies associated with the AC-18 pattern have been reported in systemic autoimmune rheumatic diseases and in a variety of other diseases including cancers [2, 7]; their prevalence in unselected or specified disease cohorts has not been thoroughly studied.
  • 二级信息
    Second level information


  • 抗GW小体自身抗体：
    ▶一项纳入55份阳性血清的研究显示，最常见的临床表现包括神经系统症状（如共济失调、运动及感觉神经病变；占33%）、干燥综合征（SjS；占31%），其余病例涉及多种其他诊断，包括系统性红斑狼疮（SLE）、类风湿关节炎及原发性胆汁性胆管炎（PBC）[2]。
    ▶基于ALBIA及重组蛋白免疫沉淀法的分析表明，自身抗体主要靶向Ge-1/Hedls（58%）、GW182（40%）及Su/Ago2（16%）[2,8,9]。
    抗内涵体成分自身抗体：
    ▶抗EEA1自身抗体可见于多种疾病，但约40%的患者伴有神经系统疾病[1]。
    ▶抗CLIP-170自身抗体曾在4例患者中报道，所患疾病各不相同，包括首例SLE合并特发性炎症性肌病患者；其余3例分别为局限性皮肤型系统性硬化症、胶质母细胞瘤及特发性胸腔积液[10]。
    ▶抗LBPA及抗GRASP-1自身抗体在非选择性或特定疾病队列中的研究尚不充分；抗GRASP-1自身抗体在17%的PBC血清中检出[11,12]。
    抗溶酶体成分自身抗体：
    ▶LC3自身抗体曾在1例SLE患者中报道[3]。
    ▶LAMP-2自身抗体已在抗中性粒细胞胞浆抗体相关性血管病变中报道[4–6]。
    注： 多数关于直接结合特定内涵体及溶酶体抗原的自身抗体的报道，并未显示其与AC-18核型之间存在直接相关性。


    Autoantibodies 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 systemic lupus erythematosus (SLE), rheumatoid arthritis, and primary biliary cholangitis (PBC) [2].
    ▶Analysis by ALBIA and immunoprecipitation of recombinant proteins indicated that autoantibodies were directed against Ge-1/Hedls (58%), GW182 (40%), and Su/Ago2 (16%) [2, 8, 9].
    Autoantibodies to endosomal components:
    ▶Autoantibodies to EEA1 were seen in a variety of conditions, but ~40% of the patients had a neurological disease [1].
    ▶Autoantibodies to CLIP-170 were reported in 4 patients with different diseases including the prototype patient with SLE and idiopathic inflammatory myopathies; the remaining 3 patients had limited cutaneous systemic sclerosis, glioblastoma, and idiopathic pleural effusion [10].
    ▶Autoantibodies to both LBPA and GRASP-1 have not been studied thoroughly in unselected or specified disease cohorts; autoantibodies to GRASP-1 were detected in 17% of PBC sera [11, 12].
    Autoantibodies to lysosomal component:
    ▶LC3 autoantibodies were reported in a case of SLE [3].
    ▶LAMP-2 autoantibodies were reported in anti-neutrophil cytoplasmic antibodies-related vasculopathies [4-6].
    Notes: Most reports describing autoantibodies directly binding to specific endosomal and lysosomal antigens do not show correlations with the AC-18 pattern as such.
  • 参考文献


  • 1.Stinton LM, Eystathioy T, Selak S, Chan EKL, Fritzler MJ. 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.
    2.Bhanji RA, Eystathioy T, Chan EKL, Bloch DB, Fritzler MJ. Clinical and serological features of patients with autoantibodies to GW/P bodies. Clin Immunol. 2007;125:247-56.
    3.Maellaro E, Terzuoli L, Bacarelli MR, Del Bello B, Bizzaro N, Porcelli B. Autoantibodies against the autophagic protein microtubule-associated light-chain 3 (LC3): Immunocharacterization of an atypical ANA pattern. Autoimmunity. 2020;53:245-52.
    4.Kain R, Tadema H, McKinney EF, Benharkou A, Brandes R, Peschel A, Hubert V, Feenstra T, et al. High prevalence of autoantibodies to hLAMP-2 in anti-neutrophil cytoplasmic antibody-associated vasculitis. J Am Soc Nephrol. 2012;23:556-66.
    5.Akbaba TH, Toor KK, Mann SK, Gibson KM, Alfaro GA, Balci-Peynircioglu B, Cabral DA, Morishita KA, et al. Anti-LAMP-2 Antibody Seropositivity in Children with Primary Systemic Vasculitis Affecting Medium- and Large-Sized Vessels. Int J Mol Sci. 2024;25.
    6.Gibson KM, Kain R, Luqmani RA, Ross CJ, Cabral DA, Brown KL. Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis. Front Immunol. 2020;11:624758.
    7.Hsiao CY, Su LJ, Yu KH, Chan TM. Clinical significance of cytoplasmic discrete dots (AC-18) patterns in HEp-2 cell indirect Immunofluorescence: insights from a Taiwanese multicenter study. Clin Chim Acta. 2025;578:120521.
    8.Yu JH, Yang WH, Gulick T, Bloch KD, Bloch DB. Ge-1 is a central component of the mammalian cytoplasmic mRNA processing body. RNA. 2005;11:1795-802.
    9.Bloch DB, Yu JH, Yang WH, Graeme-Cook F, Lindor KD, Viswanathan A, Bloch KD, Nakajima A. The cytoplasmic dot staining pattern is detected in a subgroup of patients with primary biliary cirrhosis. J Rheumatol. 2005;32:477-83.
    10.Griffith KJ, Ryan JP, Senécal JL, Fritzler MJ. The cytoplasmic linker protein CLIP-170 is a human autoantigen. Clin Exp Immunol. 2002;127:533-8.
    11.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.
    12.Stinton LM, Swain M, Myers RP, Shaheen AA, Fritzler MJ. Autoantibodies to GW bodies and other autoantigens in primary biliary cirrhosis. Clin Exp Immunol. 2011;163:147-56.


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