TY - T1的机制自身抗体在收购neurofascins脱髓鞘疾病(P1.434) JF -神经学乔-神经学六世- 90 - 15补充SP - P1.434盟克里斯蒂娜帕特森AU -伊丽莎白Burnor盟Amit酒吧或非盟-首页埃里克·兰开斯特Y1 - 2018/04/10 UR - //www.ez-admanager.com/content/90/15_Supplement/P1.434.abstract N2 -目的:确定neurofascins自身抗体的致病机制。背景:自身抗体对细胞粘附分子(摄像头)表示有髓鞘的轴突和髓鞘细胞与神经系统疾病虽然这些自身抗体的机械基础知之甚少。Neurofascin155 (NF155)与Contactin / Caspr paranode,这个协会是必要的锚定轴突髓鞘。自身抗体NF155定义获得脱髓鞘神经病变患者的一个子集,这些抗体通常IgG4子类。IgG4,与其他免疫球蛋白亚型,经历让每个抗体bi-specific hemi-antibody交换。这使得交联和内化目标抗原的不可能和有利于抑制交互与其他摄像头作为一个潜在的机制。以前Contactin自身抗体(也IgG4)被证明破坏与NF155 Contactin / Caspr之间的交互。设计/方法:NF155存在自身抗体和免疫球蛋白测定子类被证实利用细胞筛选试验。固相绑定试验被用来确认绑定NF155 Contactin体外和探索的能力NF155自身抗体破坏这种交互。结果:我们发现4例获得脱髓鞘神经病变包含NF155自身抗体。所有4例包含IgG4 NF155自身抗体。IgG4主要亚型在3 4例。 These autoantibodies had variable effects on the interaction of NF155 with Contactin. While one case effectively inhibited this interaction, 3 of the 4 cases did not.Conclusions: More work needs to be done to investigate our hypothesis that IgG4 autoantibodies to NF155 disrupt its interaction with other CAMs. Future work will focus on the effect of NF155 autoantibodies on the NF155/Contactin/Caspr complex. These antibodies may interfere with the ability of NF155 to interact with Contactin/Caspr in a manner similar to IgG4 Contactin autoantibodies.Study Supported by: supported by NIH/NINDS R25 NS065745supported by a grant from Grifols IncDisclosure: Dr. Patterson has nothing to disclose. Dr. Burnor has nothing to disclose. Dr. Bar-Or has nothing to disclose. Dr. Lancaster has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Grifols Inc. ER -