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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Isotypes and antigenic profiles of pemphigus foliaceus and pemphigus vulgaris autoantibodies.
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Isotypes and antigenic profiles of pemphigus foliaceus and pemphigus vulgaris autoantibodies.

机译：天疱疮天疱疮和寻常型天疱疮自身抗体的同种型和抗原谱。

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摘要

In this study we systematically characterized isotype profiles and antigenic and tissue specificity of antidesmoglein autoantibodies from patients with pemphigus foliaceus (PF) and pemphigus vulgaris (PV) using enzyme-linked immunoabsorbent assays (ELISA), indirect immunofluorescence (IIF) staining, and immunoblotting (IB). In PF, we found that IgG1 antidesmoglein-1 (Dsg1) reacts with a linear epitope(s) on the ectodomain of Dsg1, while its IgG4 counterpart recognizes a conformational epitope(s). These two subclasses of anti-Dsg1 are both capable of recognizing tissues from monkey esophagus and adult human skin, but IgG1 is not able to react with mouse skin, which may explain why this isotype of anti-Dsg1 failed to induce PF-like lesions in the passive transfer animal model. In mucosal PV patients, we found that both IgG1 and IgG4 only recognized monkey esophagus tissue by IIF, except in one patient, indicating that these antibodies react with a unique conformational epitope(s) that is present in mucosal but not skin tissue. In generalized PV, IgG1 anti-Dsg3 autoantibodies appeared to recognize a linear epitope(s) on the Dsg3 ectodomain. In contrast, IgG4 anti-Dsg3 antibodies recognized both linear and conformational epitopes on the Dsg3 molecule. Interestingly, the IgG1 anti-Dsg3 antibodies failed to react with human and mouse skin tissues, suggesting that this subclass of autoantibodies may not play an essential role in the development of PV suprabasilar lesions. In summary, we conclude that this study further elucidates the pathological mechanisms of PF and PV autoantibodies by revealing their distinct isotype and antigenic profiles. This information may help us to better understand the autoimmune mechanisms underlying the development of pemphigus.
机译：在这项研究中，我们通过酶联免疫吸附测定（ELISA），间接免疫荧光（IIF）染色和免疫印迹（系统分析）鉴定了叶天疱疮（PF）和寻常型天疱疮（PV）患者的抗桥粒体自身抗体的同种型谱以及抗原和组织特异性IB）。在PF中，我们发现IgG1抗桥粒芯蛋白1（Dsg1）与Dsg1胞外域上的线性表位反应，而其IgG4对应物识别构象表位。抗Dsg1的这两个亚类都能够识别来自猴子食道和成年人类皮肤的组织，但IgG1无法与小鼠皮肤反应，这可能解释了为什么这种抗Dsg1的同种型未能在小鼠体内诱导PF样病变。被动转移动物模型。在黏膜PV患者中，我们发现IgG1和IgG4都只能通过IIF识别猴食道组织，只有一名患者除外，这表明这些抗体与黏膜中存在的独特构象表位反应，而皮肤组织中没有。在广义PV中，IgG1抗Dsg3自身抗体似乎可以识别Dsg3胞外域上的线性表位。相反，IgG4抗Dsg3抗体识别Dsg3分子上的线性和构象表位。有趣的是，IgG1抗Dsg3抗体无法与人和小鼠的皮肤组织反应，这表明这种自身抗体亚类可能在PV上基底膜病变的发展中不发挥重要作用。总之，我们得出的结论是，这项研究通过揭示PF和PV自身抗体的独特同种型和抗原谱，进一步阐明了它们的病理机制。这些信息可以帮助我们更好地了解天疱疮发生的自身免疫机制。

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