Diversity in autoimmunity against retinal, neuronal, and axonal antigens in acquired neuro-retinopathy
Diversity in autoimmunity against retinal, neuronal, and axonal antigens in acquired neuro-retinopathy
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DOI：
10.1007/s12348-011-0028-8
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发表时间：
2011-01-01
影响因子：
2.9
通讯作者：
Iannaccone, Alessandro
中科院分区：
文献类型：
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作者：
Adamus, Grazyna;Brown, Lori;Iannaccone, Alessandro
目的自身免疫性视网膜病和视神经病是视网膜和视神经的复杂疾病，其中患者产生针对视网膜和视神经蛋白的自身抗体（AAbs）。自身免疫可能对视网膜和视神经退行性病变的转归有重要影响，但其发病机制尚未完全阐明。为了更好地了解这些可疑的自身免疫性视觉障碍的致病性的AAbs的作用，我们专注于独特的AAbs特异性相关的综合征，以确定其抗原的目标在视神经和retina.Methods血清样本从患者，其视觉障碍是潜在的自身免疫性的性质，包括患者与癌症可能的副肿瘤综合征。免疫印迹和免疫荧光检测抗人视神经和视网膜抗原的特异性自身抗体。结果在209例抗视神经自身抗体检测中，55%显示特异性神经元自身抗体。抗视神经自身抗体的库通常不同于抗视网膜抗体。这些抗体的主要抗原靶标可分为四组。对参与能量产生的经典糖酵解酶具有特异性的自身抗体（a和.烯醇化酶、甘油醛3-磷酸脱氢酶）也与视网膜抗原反应。针对神经元特异性髓鞘蛋白（MBP，MOG），水通道蛋白4和塌陷反应介导蛋白5的自身抗体与视神经抗原反应。他们表现出免疫染色的轴突和髓鞘在视神经确定由双重immunofluorescence.Conclusion我们确定了新的神经元自身抗原，以前不知道与获得性自身免疫性视网膜病变和视神经病变。了解使这种综合征永久存在的完整自身抗体库是增加我们对自身免疫过程的理解以促进更好的诊断、预后和治疗的重要首要要求。
Purpose Autoimmune retinopathies and optic neuropathies are complex disorders of the retina and the optic nerve, in which patients develop autoantibodies (AAbs) against retinal and optic nerve proteins. Autoimmunity might significantly influence the outcome of retinal and optic nerve degenerative process but the pathogenic process is not fully elucidated. To better understand the role of AAbs in pathogenicity of these suspected autoimmune visual disorders, we focused on unique AAbs specificities associated with the syndrome to identify their antigenic targets in the optic nerve and retina.Methods Serum samples were obtained from patients, whose visual disorders were potentially autoimmune in nature, including patients with cancer with possible paraneoplastic syndrome. Autoantibodies were tested against human optic nerve and retinal antigens for specificity by Western blotting and immunofluorescence.Results Out of 209 tested for anti-optic nerve autoantibodies, 55% showed specific neuronal autoantibodies. The repertoire of anti-optic nerve autoantibodies often differed from anti-retinal antibodies. The major antigenic targets for these antibodies could be divided into four groups. Autoantibodies specific to classical glycolytic enzymes involved in energy production (a and. enolases, glyceraldehyde 3-phosphate dehydrogenase) also reacted with retinal antigens. Autoantibodies targeted neuronal-specific myelin proteins (MBP, MOG), aquaporin 4, and collapsing response mediator protein 5 reacted with optic nerve antigens. They showed immunostaining of axons and myelin in the optic nerve as determined by double immunofluorescence.Conclusion We identified novel neuronal autoantigens not previously known to be associated with acquired autoimmune retinopathy and optic neuropathy. Knowledge of the full autoantibody repertoire perpetuating this syndrome is an important first requirement in increasing our understanding of the autoimmune process to facilitate better diagnosis, prognosis, and treatment.