Levite Mia
Faculty of Medicine, School of Pharmacy, The Hebrew University, Jerusalem, Israel.
Institute of Gene Therapy, Hadassah Medical Center, 91120, Ein Karem, Jerusalem, Israel.
J Neural Transm (Vienna). 2017 Jul;124(7):775-798. doi: 10.1007/s00702-016-1661-z. Epub 2017 Feb 24.
Glutamate is the major excitatory neurotransmitter in the nervous system, where it induces multiple beneficial and essential effects. Yet, excess glutamate, evident in a kaleidoscope of acute and chronic pathologies, is absolutely catastrophic, since it induces excitotoxicity and massive loss of brain function. Both the beneficial and the detrimental effects of glutamate are mediated by a large family of glutamate receptors (GluRs): the ionotropic glutamate receptors (iGluRs) and the metabotropic glutamate receptors (mGluRs), expressed by most/all cells of the nervous system, and also by many non-neural cells in various peripheral organs and tissues. T cells express on their cell surface several types of functional GluRs, and so do few other immune cells. Furthermore, glutamate by itself activates resting normal human T cells, and induces/elevates key T cell functions, among them: T cell adhesion, chemotactic migration, cytokine secretion, gene expression and more. Glutamate has also potent effects on antigen/mitogen/cytokine-activated T cells. Furthermore, T cells can even produce and release glutamate, and affect other cells and themselves via their own glutamate. Multiple sclerosis (MS) and its animal model Experimental Autoimmune Encephalomyelitis (EAE) are mediated by autoimmune T cells. In MS and EAE, there are excess glutamate levels, and multiple abnormalities in glutamate degrading enzymes, glutamate transporters, glutamate receptors and glutamate signaling. Some GluR antagonists block EAE. Enhancer of mGluR4 protects from EAE via regulatory T cells (Tregs), while mGluR4 deficiency exacerbates EAE. The protective effect of mGluR4 on EAE calls for testing GluR4 enhancers in MS patients. Oral MS therapeutics, namely Fingolimod, dimethyl fumarate and their respective metabolites Fingolimod-phosphate and monomethyl fumarate, can protect neurons against acute glutamatergic excitotoxic damage. Furthermore, Fingolimod reduce glutamate-mediated intracortical excitability in relapsing-remitting MS. Glatiramer acetate -COPAXONE®, an immunomodulator drug for MS, reverses TNF-α-induced alterations of striatal glutamate-mediated excitatory postsynaptic currents in EAE-afflicted mice. With regard to T cells of MS patients: (1) The cell surface expression of a specific GluR: the AMPA GluR3 is elevated in T cells of MS patients during relapse and with active disease, (2) Glutamate and AMPA (a selective agonist for glutamate/AMPA iGluRs) augment chemotactic migration of T cells of MS patients, (3) Glutamate augments proliferation of T cells of MS patients in response to myelin-derived proteins: MBP and MOG, (4) T cells of MS patients respond abnormally to glutamate, (5) Significantly higher proliferation values in response to glutamate were found in MS patients assessed during relapse, and in those with gadolinium (Gd)+ enhancing lesions on MRI. Furthermore, glutamate released from autoreactive T cells induces excitotoxic cell death of neurons. Taken together, the evidences accumulated thus far indicate that abnormal glutamate levels and signaling in the nervous system, direct activation of T cells by glutamate, and glutamate release by T cells, can all contribute to MS. This may be true also to other neurological diseases. It is postulated herein that the detrimental activation of autoimmune T cells by glutamate in MS could lead to: (1) Cytotoxicity in the CNS: T cell-mediated killing of neurons and glia cells, which would subsequently increase the extracellular glutamate levels, and by doing so increase the excitotoxicity mediated by excess glutamate, (2) Release of proinflammatory cytokines, e.g., TNFα and IFNγ that increase neuroinflammation. Finally, if excess glutamate, abnormal neuronal signaling, glutamate-induced activation of T cells, and glutamate release by T cells are indeed all playing a key detrimental role in MS, then optional therapeutic tolls include GluR antagonists, although these may have various side effects. In addition, an especially attractive therapeutic strategy is the novel and entirely different therapeutic approach to minimize excess glutamate and excitotoxicity, titled: 'brain to blood glutamate scavenging', designed to lower excess glutamate levels in the CNS by 'pumping it out' from the brain to the blood. The glutamate scavanging is achieved by lowering glutamate levels in the blood by intravenous injection of the blood enzyme glutamate oxaloacetate transaminase (GOT). The glutamate-scavenging technology, which is still experimental, validated so far for other brain pathologies, but not tested on MS or EAE yet, may be beneficial for MS too, since it could decrease both the deleterious effects of excess glutamate on neural cells, and the activation of autoimmune T cells by glutamate in the brain. The topic of glutamate scavenging, and also its potential benefit for MS, are discussed towards the end of the review, and call for research in this direction.
谷氨酸是神经系统中主要的兴奋性神经递质,在神经系统中它会产生多种有益且必不可少的作用。然而,在一系列急性和慢性病症中明显存在的过量谷氨酸却是绝对灾难性的,因为它会引发兴奋性毒性并导致脑功能大量丧失。谷氨酸的有益和有害作用均由一大类谷氨酸受体(GluRs)介导:离子型谷氨酸受体(iGluRs)和代谢型谷氨酸受体(mGluRs),它们由神经系统的大多数/所有细胞表达,也由各种外周器官和组织中的许多非神经细胞表达。T细胞在其细胞表面表达几种功能性GluRs,其他一些免疫细胞也如此。此外,谷氨酸自身可激活静息的正常人T细胞,并诱导/增强关键的T细胞功能,其中包括:T细胞黏附、趋化性迁移、细胞因子分泌、基因表达等。谷氨酸对抗原/丝裂原/细胞因子激活的T细胞也有显著作用。此外,T细胞甚至可以产生和释放谷氨酸,并通过自身的谷氨酸影响其他细胞和自身。多发性硬化症(MS)及其动物模型实验性自身免疫性脑脊髓炎(EAE)由自身免疫性T细胞介导。在MS和EAE中,存在谷氨酸水平过高以及谷氨酸降解酶、谷氨酸转运体、谷氨酸受体和谷氨酸信号传导的多种异常情况。一些谷氨酸受体拮抗剂可阻断EAE。mGluR4增强剂通过调节性T细胞(Tregs)保护机体免受EAE侵害,而mGluR4缺乏则会加重EAE。mGluR4对EAE的保护作用促使人们在MS患者中测试GluR4增强剂。口服MS治疗药物,即芬戈莫德、富马酸二甲酯及其各自的代谢产物磷酸芬戈莫德和单甲基富马酸酯,可以保护神经元免受急性谷氨酸能兴奋性毒性损伤。此外,芬戈莫德可降低复发缓解型MS中谷氨酸介导的皮质内兴奋性。醋酸格拉替雷 - COPAXONE®,一种用于MS的免疫调节药物,可逆转肿瘤坏死因子-α诱导的EAE患病小鼠纹状体谷氨酸介导的兴奋性突触后电流的改变。关于MS患者的T细胞:(1)一种特定谷氨酸受体:AMPA GluR3在MS患者复发期间和疾病活动期的T细胞中细胞表面表达升高,(2)谷氨酸和AMPA(谷氨酸/AMPA离子型谷氨酸受体的选择性激动剂)增强MS患者T细胞的趋化性迁移,(3)谷氨酸增强MS患者T细胞对髓鞘来源蛋白:髓鞘碱性蛋白(MBP)和髓鞘少突胶质细胞糖蛋白(MOG)的增殖反应,(4)MS患者的T细胞对谷氨酸反应异常,(从复发期评估的MS患者以及MRI上有钆(Gd)+增强病灶的患者中发现,其对谷氨酸的增殖值显著更高。此外,自身反应性T细胞释放的谷氨酸会诱导神经元的兴奋性毒性细胞死亡。综上所述,迄今为止积累的证据表明,神经系统中异常的谷氨酸水平和信号传导、谷氨酸对T细胞的直接激活以及T细胞释放谷氨酸,都可能导致MS。其他神经疾病可能也是如此。本文推测,MS中谷氨酸对自身免疫性T细胞的有害激活可能导致:(1)中枢神经系统中的细胞毒性:T细胞介导的神经元和神经胶质细胞杀伤,这随后会增加细胞外谷氨酸水平,进而增加过量谷氨酸介导的兴奋性毒性,(2)促炎细胞因子的释放,例如肿瘤坏死因子-α和干扰素-γ,它们会增加神经炎症。最后,如果过量谷氨酸、异常的神经元信号传导、谷氨酸诱导的T细胞激活以及T细胞释放谷氨酸确实在MS中都起着关键的有害作用,那么可选的治疗手段包括谷氨酸受体拮抗剂,尽管这些可能有各种副作用。此外,一种特别有吸引力的治疗策略是一种全新且截然不同的治疗方法,旨在最大限度地减少过量谷氨酸和兴奋性毒性,即“脑-血谷氨酸清除”,旨在通过将其从大脑“泵出”到血液中来降低中枢神经系统中过量的谷氨酸水平。通过静脉注射血液酶谷氨酸草酰乙酸转氨酶(GOT)来降低血液中的谷氨酸水平,从而实现谷氨酸清除。这种谷氨酸清除技术仍处于实验阶段,目前已在其他脑部疾病中得到验证,但尚未在MS或EAE上进行测试,它可能对MS也有益,因为它既可以减少过量谷氨酸对神经细胞的有害影响,也可以减少大脑中谷氨酸对自身免疫性T细胞的激活。本文在综述结尾讨论了谷氨酸清除的主题及其对MS的潜在益处,并呼吁朝这个方向进行研究。