Bettelli Estelle
Center for Neurologic Diseases, Brigham and Womens Hospital, Boston MA 02115, USA.
Ann N Y Acad Sci. 2007 Apr;1103:11-8. doi: 10.1196/annals.1394.021. Epub 2007 Mar 21.
Multiple sclerosis (MS) is a clinically and pathologically heterogeneous inflammatory/demyelinating disease of the central nervous system (CNS). Many patients first present with isolated optic neuritis. In some variants of MS, like Devic's disease or neuromyelitis optica (NMO), lesions are predominantly found in the optic nerves and spinal cord but not in the brain. The immunological bases of the different forms of MS are unknown. Here, we summarize our published findings on two mouse models: 2D2 myelin oligodendrocyte glycoprotein (MOG)-specific T cell receptor (TCR) transgenic mice, which develop spontaneous isolated optic neuritis, and 2D2 mice crossed with MOG-specific IgH knockin (TH) mice, which spontaneously develop a severe form of experimental autoimmune encephalomyelitis (EAE) with a selective distribution of meningeal and parenchymal inflammatory lesions in the spinal cord and optic nerves similar to that found in human Devic's disease.
多发性硬化症(MS)是一种临床和病理上异质性的中枢神经系统(CNS)炎症/脱髓鞘疾病。许多患者最初表现为孤立性视神经炎。在MS的某些变体中,如视神经脊髓炎(Devic病)或视神经脊髓炎(NMO),病变主要见于视神经和脊髓,而不见于脑。不同形式MS的免疫基础尚不清楚。在此,我们总结了我们在两种小鼠模型上发表的研究结果:2D2髓鞘少突胶质细胞糖蛋白(MOG)特异性T细胞受体(TCR)转基因小鼠,其会自发发生孤立性视神经炎;以及与MOG特异性IgH敲入(TH)小鼠杂交的2D2小鼠,其会自发发生严重形式的实验性自身免疫性脑脊髓炎(EAE),在脊髓和视神经中出现与人类Devic病相似的脑膜和实质炎症病变的选择性分布。