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视神经脊髓炎(NMO)是主要累及视神经和脊髓的特发性炎性脱髓鞘和致坏死性疾病。在NMO患者血清中发现一种与水通道蛋白4(AQP4)结合的自身IgG抗体(NMO-IgG)。NMO-IgG并非在NMO患者血清中发现的唯一自身抗体,但与正常高表达AQP4的中枢神经系统的组织病理关系说明NMO-IgG可能具有致病性。就AQP4在NMO发病中的作用,AQP4的B细胞表位和发病的关系以及AQP4特异性B细胞和T细胞的启动进行综述。
Optic neuromyelitis (NMO) is an idiopathic inflammatory demyelinating and necrotizing disease that mainly affects the optic nerve and spinal cord. A self IgG antibody (NMO-IgG) bound to aquaporin 4 (AQP4) was found in the serum of NMO patients. NMO-IgG is not the only autoantibody found in the serum of NMO patients, but histopathological correlation with the central nervous system that normally overexpresses AQP4 suggests NMO-IgG may be pathogenic. The role of AQP4 in the pathogenesis of NMO, the relationship between B cell epitopes and pathogenesis of AQP4, and the initiation of AQP4-specific B cells and T cells were reviewed.