Ellwardt Erik, Zipp Frauke
Focus Program Translational Neurosciences (FTN), Rhine Main Neuroscience Network (rmn2), Department of Neurology, University Medical Center of the Johannes-Gutenberg University Mainz, Langenbeckstr. 1, 55131 Mainz, Germany.
Focus Program Translational Neurosciences (FTN), Rhine Main Neuroscience Network (rmn2), Department of Neurology, University Medical Center of the Johannes-Gutenberg University Mainz, Langenbeckstr. 1, 55131 Mainz, Germany.
Exp Neurol. 2014 Dec;262 Pt A:8-17. doi: 10.1016/j.expneurol.2014.02.006. Epub 2014 Feb 14.
Multiple sclerosis (MS) is an inflammatory demyelinating autoimmune disorder of the central nervous system (CNS) and one of the leading causes of neurological deficits and disability in young adults in western countries. Current medical treatment mainly influences disease progression via immunomodulatory or immunosuppressive actions. Indeed, MS research has been foremost focused on inflammation in the CNS, but more recent evidence suggests that chronic disability in MS is caused by neurodegeneration. Imaging studies show an early involvement of neurodegeneration as brain atrophy and gray matter lesions can be observed at disease onset. Thus, neuroprotective treatment strategies and the elucidation of the molecular mechanisms underlying neurodegeneration in MS have attracted the attention of the scientific community. Experimental autoimmune encephalomyelitis (EAE; the most commonly used animal model for MS), novel in-vivo imaging techniques such as two-photon microscopy and recently discovered molecular changes have offered new insights into the pathogenesis of neuroinflammation as well as neurodegeneration in MS. This review focuses on the interaction between components of the immune system and the neuronal compartment, as well as describing the most important molecular mechanisms that lead to axonal and neuronal degeneration in MS and EAE.
多发性硬化症(MS)是一种中枢神经系统(CNS)的炎性脱髓鞘自身免疫性疾病,是西方国家年轻人神经功能缺损和残疾的主要原因之一。目前的医学治疗主要通过免疫调节或免疫抑制作用来影响疾病进展。事实上,MS研究一直主要集中在CNS的炎症上,但最近的证据表明,MS中的慢性残疾是由神经退行性变引起的。影像学研究表明,神经退行性变在疾病早期就已出现,因为在疾病发作时就可观察到脑萎缩和灰质病变。因此,神经保护治疗策略以及对MS中神经退行性变潜在分子机制的阐明已引起科学界的关注。实验性自身免疫性脑脊髓炎(EAE;MS最常用的动物模型)、双光子显微镜等新型体内成像技术以及最近发现的分子变化,为MS中神经炎症以及神经退行性变的发病机制提供了新的见解。本综述重点关注免疫系统各组成部分与神经元区室之间的相互作用,并描述导致MS和EAE中轴突和神经元变性最重要的分子机制。