Autoantibodies to p75/LEDGF, a cell survival factor, found in patients with atypical retinal degeneration
Autoantibodies to p75/LEDGF, a cell survival factor, found in patients with atypical retinal degeneration
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DOI：
10.1016/j.jaut.2006.04.002
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发表时间：
2006-08-01
影响因子：
12.8
通讯作者：
Hooks, John J.
中科院分区：
文献类型：
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作者：
Chin, Marian S.;Caruso, Rafael C.;Hooks, John J.
我们已经确定了一组患者的非典型视网膜变性抗视网膜自身抗体在他们的血清中。该选择人群的特征在于与光感受器功能降低相关的进行性严重视力丧失、视网膜色素上皮的异常色素沉着和视网膜营养不良的阴性家族史。用来自这组患者的血清对小鼠视网膜组织进行的免疫组织化学分析显示出高滴度的抗视网膜抗体（320至1280）。在正常个体、葡萄膜炎或已知遗传性视网膜变性患者的血清中未检测到这些水平的抗视网膜反应。从具有来自非典型视网膜变性患者的血清的视网膜cDNA文库中鉴定的一种抗原是透镜上皮衍生生长因子（LEDGF）。蛋白质印迹分析显示，所有三名患者的血清都表现出对p75/LEDGF的反应性，p75/LEDGF是一种保护细胞免受氧化、热和紫外线损伤的存活因子。总之，我们发现了一组新的非副肿瘤性、非家族性视网膜变性患者，表现出对自身抗原p75/LEDGF的免疫反应性，迄今为止与这种疾病无关。最后，特异性抗视网膜抗体的鉴定可能在视网膜变性的诊断和管理中具有应用。爱思唯尔有限公司出版
We have identified a group of patients with an atypical retinal degeneration having anti-retinal autoantibodies in their sera. This select population is characterized by a progressively severe loss of vision associated with a decrease in photoreceptor function, abnormal pigmentation of the retinal pigment epithelium and a negative family history of retinal dystrophy. Immunohistochemical analysis on mouse retinal tissues with sera from this group of patients demonstrated high titers of anti-retinal antibodies (320 to 1280). Anti-retinal reactivity at these levels was not detected in sera from normal individuals, or from patients with uveitis or known genetic retinal degenerations. One antigen that was identified from a retina cDNA library with sera from a patient with atypical retinal degeneration was lens epithelium-derived growth factor (LEDGF). Western blot analysis revealed that sera from all three patients demonstrated reactivity to p75/LEDGF, a survival factor that protects cells from oxidative, thermal and UV damage. In conclusion, we have found a novel group of patients with a retinal degeneration of non-paraneoplastic, non-familial origin demonstrating immunoreactivity to an autoantigen, p75/LEDGF, heretofore not associated with this disease. Finally, identification of specific anti-retinal antibodies may have applications in the diagnosis and management of retinal degeneration. Published by Elsevier Ltd.