简体中文

AC-17-胞浆节段型

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描述

分裂间期细胞胞浆边缘呈现增强的节状荧光

AC-17型抗体的血清判定存在不确定性。对比两种血清在三种HEp-2品牌结果如下，血清#1在品牌#2上显著强于品牌#1；血清#2在品牌#3上清晰可见而品牌#1完全阴性，并提示品牌#1对AC-17检出灵敏度偏低；；血清#2在品牌#3上的形态偶似AC-23；，品牌#1能稳定检出AC-23，故可排除干扰；综上所诉：AC-17的检出能力部分依赖于HEp-2品牌，应审慎选材并辅以确证试验。

Enhanced decoration of short segments of stress fibers with periodic dense bodies. 

There is uncertainty in the characterization of sera with the AC-17 pattern. The composite figure shows the staining of two human sera (#1 and #2) each with two different HEp-2 brands. For serum #1, the distinctive staining illustrating AC-17 is much more prominent shown in brand #2 compared to brand #1. Similarly, serum #2 shows much more distinctive AC-17 staining in brand #3 while staining is not visible in brand #1. This limited data suggest that HEp-2 brand #1 is not quite sensitive for the detection of AC-17 as compared to brand #2 and #3. [One may question whether the Serum #2 staining with HEp-2 brand #3 is similar to some features of the cytoplasmic rods/rings staining (AC-23); however, this can be ruled out readily as HEp-2 brand #1 is known to show consistent AC-23 staining.] In summary, the ability to detect AC-17 may depend in part on staining on certain HEp-2 brands.
抗原相关性 α-辅肌动蛋白，黏着斑蛋白 alpha-actinin, vinculin

  • 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
  • ▶在常规诊断实验室环境中非常少见
    ▶试剂目前尚未商品化
    ▶Found very infrequently in a routine diagnostic laboratory setting.
    ▶Specific immunoassays for these autoantibodies are currently not commercially available.
  • 二级信息
    Second level information


  • 抗α-辅肌动蛋白的自身抗体：
    ▶在系统性红斑狼疮和狼疮性肾炎中，有交叉反应的抗a-肌动蛋白和抗dsDNA抗体具有致病性 [1]。
    ▶据报道，部分抗细胞膜抗体谱是狼疮性肾炎患者的特征;也是无肾脏受累的狼疮肾炎患者区分的一种生物标志物[2, 3]。
    ▶据报道，在相当高比例(-40%)的1型自身免疫性肝炎患者中检出该抗体，并且与更严重的疾病、临床和组织病理的疾病活动性、治疗反应的预测性以及和抗ssDNA抗体的双阳性相关联[4-6]。
    抗黏着斑蛋白的自身抗体。
    ▶据报道，在31例慢性炎性脱髓鞘神经病患者中有2例检出该抗体[7]。
    ▶据报道，系统性硬化症与间质肺病及胃肠受累中相关该抗体[8-10]。
    ▶据报道，该抗体在足细胞病和特发性肾病综合征中有致病潜力，可监测病情[11]。
    ▶据报道，在肠易激综合征中，vinculin和CdtB抗体为该抗体标志物[12-14]。
    注:大多数报道描述了与特异性抗原直接结合的自身抗体(即，抗原特异性免疫检测法)，实际上并未显示与AC-17核型的相关性。


    Autoantibodies to α-actinin:
    ▶In systemic lupus erythematosus and lupus nephritis cross-reactive α-actinin and dsDNA autoantibodies have been reported as pathogenic [1].
    ▶Reported as part of the cell membrane antibody spectrum that characterizes patients with lupus nephritis; also reported as a biomarker that differentiated patients with lupus nephritis from those without renal involvement [2, 3].
    ▶Reported with relatively high prevalence (~40%) in patients with autoimmune hepatitis type 1 and associated with more severe disease, clinical and histological disease activity, predictor of therapeutic response, and double positivity with ssDNA antibodies [4-6].
    Autoantibodies to vinculin:
    ▶Reported in 2 of 31 patients with chronic inflammatory demyelinating neuropathy [7].
    ▶Reported in systemic sclerosis where they are associated with interstitial lung disease and gastrointestinal involvement [8-10].
    ▶Reported in patients with podocytopathies and idiopathic nephrotic syndrome to have pathogenic potential; may be useful in monitoring disease activity [11].
    ▶Vinculin and Cytolethal Distending Toxin B (CdtB) antibodies are biomarkers in irritable bowel syndrome [12-14].
    Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e. solid phase antigen-specific immunoassays) and rarely demonstrate correlations with the AC-17 pattern.
  • 参考文献
  • 1.Zhao Z, Weinstein E, Tuzova M, Davidson A, Mundel P, Marambio P, Putterman C. Cross-reactivity of human lupus anti-DNA antibodies with alpha-actinin and nephritogenic potential. Arthritis Rheum. 2005;52:522-30.
    2.Seret G, Canas F, Pougnet-Di Costanzo L, Hanrotel-Saliou C, Jousse-Joulin S, Le Meur Y, Saraux A, Valeri A, et al. Anti-alpha-actinin antibodies are part of the anti-cell membrane antibody spectrum that characterize patients with lupus nephritis. J Autoimmun. 2015;61:54-61.
    3.Zhang WH, Pan HF, Zhao XF, Ye DQ, Li XP, Xu JH. Anti-alpha-actinin antibodies in relation to new-onset systemic lupus erythematosus and lupus nephritis. Mol Biol Rep. 2010;37:1341-5.
    4.Gueguen P, Dalekos G, Nousbaum JB, Zachou K, Putterman C, Youinou P, Renaudineau Y. Double reactivity against actin and alpha-actinin defines a severe form of autoimmune hepatitis type 1. J Clin Immunol. 2006;26:495-505.
    5.Renaudineau Y, Dalekos GN, Gueguen P, Zachou K, Youinou P. Anti-alpha-actinin antibodies cross-react with anti-ssDNA antibodies in active autoimmune hepatitis. Clin Rev Allergy Immunol. 2008;34:321-5.
    6.Zachou K, Oikonomou K, Renaudineau Y, Chauveau A, Gatselis N, Youinou P, Dalekos GN. Anti-alpha actinin antibodies as new predictors of response to treatment in autoimmune hepatitis type 1. Aliment Pharmacol Ther. 2012;35:116-25.
    7.Beppu M, Sawai S, Satoh M, Mori M, Kazami T, Misawa S, Shibuya K, Ishibashi M, et al. Autoantibodies against vinculin in patients with chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 2015;287:9-15.
    8.Ibrahim NH, Fawzy IM, Gouda TM, El Sayed RAH, Morsi MH, Sabry ASM, Hashaad NI. Anti-vinculin antibodies as a novel biomarker in Egyptian patients with systemic sclerosis. Clin Rheumatol. 2022;41:3401-9.
    9.Adler BL, McMahan Z. Anti-vinculin autoantibodies in systemic sclerosis: a step toward a novel biomarker? Clin Rheumatol. 2021;40:809-11.
    10.Herran M, Adler BL, Perin J, Morales W, Pimentel M, McMahan ZH. Antivinculin Antibodies in Systemic Sclerosis: Associations With Slow Gastric Transit and Extraintestinal Clinical Phenotype. Arthritis Care Res (Hoboken). 2023;75:2166-73.
    11.Meng H, Wang D, Zheng C, Zhou C, Mao X, Gu J, Qiao X, Liu F, et al. Autoantibodies Targeting Vinculin Reveal Novel Insight into the Mechanisms of Autoimmune Podocytopathies. Research (Wash D C). 2025;8:0722.
    12.Rezaie A, Park SC, Morales W, Marsh E, Lembo A, Kim JH, Weitsman S, Chua KS, et al. Assessment of Anti-vinculin and Anti-cytolethal Distending Toxin B Antibodies in Subtypes of Irritable Bowel Syndrome. Dig Dis Sci. 2017;62:1480-5.
    13.Vasapolli R, Schulz C, Schweden M, Casen C, Kirubakaran GT, Kirste KH, Macke L, Link A, et al. Gut microbiota profiles and the role of anti-CdtB and anti-vinculin antibodies in patients with functional gastrointestinal disorders (FGID). Eur J Clin Invest. 2021;51:e13666.
    14.Zaki MES, Elhammady D, Foda Salama M, Abdelsalam M, Osman AOB. Study of Antibodies to Cytolethal Distending Toxin B (CdtB) and Antibodies to Vinculin in Patients with Irritable Bowel Syndrome. F1000Res. 2021;10:303.

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