Birkner Katharina, Loos Julia, Gollan René, Steffen Falk, Wasser Beatrice, Ruck Tobias, Meuth Sven G, Zipp Frauke, Bittner Stefan
Department of Neurology, Focus Program Translational Neuroscience (FTN) and Immunotherapy (FZI), Rhine Main Neuroscience Network (rmn2), University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany.
Department of Neurology, University of Muenster, Muenster, Germany.
Front Neurol. 2019 Mar 12;10:205. doi: 10.3389/fneur.2019.00205. eCollection 2019.
Multiple sclerosis (MS) is a chronic autoimmune disease of the central nervous system (CNS) leading to CNS inflammation and neurodegeneration. Current anti-inflammatory drugs have only limited efficacy on progressive neurodegenerative processes underlining the need to understand immune-mediated neuronal injury. Cell adhesion molecules play an important role for immune cell migration over the blood-brain barrier whereas their role in mediating potentially harmful contacts between invading immune cells and neurons is incompletely understood. Here, we assess the role of the CNS-specific neuronal adhesion molecule ICAM-5 using experimental autoimmune encephalomyelitis (EAE), an animal model of MS. ICAM-5 knockout mice show a more severe EAE disease course in the chronic phase indicating a neuroprotective function of ICAM-5 in progressive neurodegeneration. In agreement with the predominant CNS-specific function of ICAM-5, lymphocyte function-associated antigen 1 (LFA-1)/ICAM-1 contact between antigen-presenting cells and T helper (Th)17 cells in EAE is not affected by ICAM-5. Strikingly, intrathecal application of the shed soluble form, sICAM-5, ameliorates EAE disease symptoms and thus might serve locally as an endogenous neuronal defense mechanism which is activated upon neuroinflammation in the CNS. In humans, cerebrospinal fluid from patients suffering from progressive forms of MS shows decreased sICAM-5 levels, suggesting a lack of this endogenous protective pathway in these patient groups. Overall, our study points toward a novel role of ICAM-5 in CNS autoinflammation in progressive EAE/MS.
多发性硬化症(MS)是一种中枢神经系统(CNS)的慢性自身免疫性疾病,可导致中枢神经系统炎症和神经退行性变。目前的抗炎药物对进行性神经退行性过程的疗效有限,这突出了理解免疫介导的神经元损伤的必要性。细胞粘附分子在免疫细胞跨越血脑屏障的迁移中起重要作用,而它们在介导侵入性免疫细胞与神经元之间潜在有害接触中的作用尚未完全明确。在此,我们使用实验性自身免疫性脑脊髓炎(EAE)(一种MS的动物模型)来评估中枢神经系统特异性神经元粘附分子ICAM-5的作用。ICAM-5基因敲除小鼠在慢性期表现出更严重的EAE病程,表明ICAM-5在进行性神经退行性变中具有神经保护功能。与ICAM-5主要的中枢神经系统特异性功能一致,EAE中抗原呈递细胞与辅助性T(Th)17细胞之间的淋巴细胞功能相关抗原1(LFA-1)/ICAM-1接触不受ICAM-5影响。引人注目的是,鞘内注射可溶性形式的sICAM-5可改善EAE疾病症状,因此可能在局部作为一种内源性神经元防御机制,在中枢神经系统神经炎症时被激活。在人类中,患有进行性MS的患者脑脊液中sICAM-5水平降低,表明这些患者群体缺乏这种内源性保护途径。总体而言,我们的研究表明ICAM-5在进行性EAE/MS的中枢神经系统自身炎症中具有新作用。