The Evolving Story of Autoantibodies in Pemphigus Vulgaris: Development of the "Super Compensation Hypothesis".
The Evolving Story of Autoantibodies in Pemphigus Vulgaris: Development of the "Super Compensation Hypothesis".
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DOI：
10.3389/fmed.2018.00218
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发表时间：
2018
影响因子：
3.9
通讯作者：
Sajda T
中科院分区：
文献类型：
--
作者：
Sinha AA;Sajda T
新兴数据和创新技术正在重塑我们对寻常天疱疮（PV）自身免疫反应的范围和特异性的理解，这是一种典型的体液介导的自身免疫性皮肤起泡疾病。精子研究确定桥粒蛋白桥粒芯糖蛋白3和1（Dsg 3和Dsg 1），钙粘蛋白家族蛋白，其功能是维持细胞粘附，作为致病性自身抗体的主要靶标。因此，PV的发病机制主要被认为是抗Dsg自身抗体单独作用的结果。然而，越来越多的数据表明，抗Dsg autoAb本身不能充分解释PV中观察到的细胞-细胞粘附的丧失，也不能解释PV患者中表现出的疾病异质性，这促使人们认为其他autoAb特异性可能导致疾病。为了研究非Dsg autoAb在PV中的作用，越来越多的研究试图表征PV autoAb的其他靶标。最近出现的蛋白质微阵列技术，它允许快速，高灵敏度和多重评估的autoAb特异性，促进了全面分类的范围和特异性的autoAb反应在PV。除了简单地追踪抗Dsg autoAb之外，PV中自身免疫应答的这种详细解构提供了关于疾病机制和增强的疾病分类的宝贵的新见解，其可以直接转化为用于免疫学和临床管理的上级工具，以及新型疾病特异性治疗的开发。
Emerging data and innovative technologies are re-shaping our understanding of the scope and specificity of the autoimmune response in Pemphigus vulgaris (PV), a prototypical humorally mediated autoimmune skin blistering disorder. Seminal studies identified the desmosomal proteins Desmoglein 3 and 1 (Dsg3 and Dsg1), cadherin family proteins which function to maintain cell adhesion, as the primary targets of pathogenic autoAbs. Consequently, pathogenesis in PV has primarily considered to be the result of anti-Dsg autoAbs alone. However, accumulating data suggesting that anti-Dsg autoAbs by themselves cannot adequately explain the loss of cell-cell adhesion seen in PV, nor account for the disease heterogeneity exhibited across PV patients has spurred the notion that additional autoAb specificities may contribute to disease. To investigate the role of non-Dsg autoAbs in PV, an increasing number of studies have attempted to characterize additional targets of PV autoAbs. The recent advent of protein microarray technology, which allows for the rapid, highly sensitive, and multiplexed assessment of autoAb specificity has facilitated the comprehensive classification of the scope and specificity of the autoAb response in PV. Such detailed deconstruction of the autoimmune response in PV, beyond simply tracking anti-Dsg autoAbs, has provided invaluable new insights concerning disease mechanisms and enhanced disease classification which could directly translate into superior tools for prognostics and clinical management, as well as the development of novel, disease specific treatments.
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影响因子：
10.3
作者：
Baroni, A;Buommino, E;Ruocco, V
通讯作者：
Ruocco, V
影响因子：
12.8
作者：
Abulafia-Lapid, R;Gillis, D;Cohen, IR
通讯作者：
Cohen, IR
影响因子：
--
作者：
Belloni-Fortina A;Faggion D;Pigozzi B;Peserico A;Bordignon M;Baldo V;Alaibac M
通讯作者：
Alaibac M
影响因子：
6.5
作者：
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通讯作者：
Stanley, JR
影响因子：
6.5
作者：
AMAGAI, M;ISHII, K;NISHIKAWA, T
通讯作者：
NISHIKAWA, T