Research Center of Neurology, Moscow, 125367, Russia.
Biochemistry (Mosc). 2023 Apr;88(4):551-563. doi: 10.1134/S0006297923040107.
Demyelinating diseases of the central nervous system are caused by an autoimmune attack on the myelin sheath surrounding axons. Myelin structural proteins become antigenic, leading to the development of myelin lesions. The use of highly specialized laboratory diagnostic techniques for identification of specific antibodies directed against myelin components can significantly improve diagnostic approaches. Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) currently includes demyelinating syndromes with known antigens. Based on the demonstrated pathogenic role of human IgG against MOG, MOGAD was classified as a distinct nosological entity. However, generation of multiple MOG isoforms by alternative splicing hinders antigen detection even with the most advanced immunofluorescence techniques. On the other hand, MOG conformational changes ensure the structural integrity of other myelin proteins and maintain human-specific mechanisms of immune autotolerance.
中枢神经系统脱髓鞘疾病是由针对轴突周围髓鞘的自身免疫攻击引起的。髓鞘结构蛋白成为抗原，导致髓鞘损伤的发生。使用高度专业化的实验室诊断技术来鉴定针对髓鞘成分的特定抗体可以显著改善诊断方法。髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）目前包括具有已知抗原的脱髓鞘综合征。基于针对 MOG 的人 IgG 的致病性作用，MOGAD 被归类为一个独特的疾病实体。然而，通过选择性剪接产生的多种 MOG 异构体，即使使用最先进的免疫荧光技术，也会妨碍抗原检测。另一方面，MOG 的构象变化确保了其他髓鞘蛋白的结构完整性，并维持了人类特有的免疫自身耐受机制。
