Bhat Roopa, Steinman Lawrence
Beckman Center for Molecular Medicine, B002, Stanford University, Stanford, CA 94305, USA.
Neuron. 2009 Oct 15;64(1):123-32. doi: 10.1016/j.neuron.2009.09.015.
The immune system has two major components, an innate arm and an adaptive arm. Certain autoimmune diseases of the brain represent examples of disorders where one of these constituents plays a major role. Some rare autoimmune diseases involve activation of the innate arm and include chronic infantile neurologic, cutaneous, articular (CINCA) syndrome. In contrast, adaptive immunity is prominent in multiple sclerosis, neuromyelitis optica, and the paraneoplastic syndromes where highly specific T cell responses and antibodies mediate these diseases. Studies of autoimmune brain disorders have aided in the elucidation of distinct neuronal roles played by key molecules already well known to immunologists (e.g., complement and components of the major histocompatibility complex). In parallel, molecules known to neurobiology and sensory physiology, including toll-like receptors, gamma amino butyric acid and the lens protein alpha B crystallin, have intriguing and distinct functions in the immune system, where they modulate autoimmunity directed to the brain.
免疫系统有两个主要组成部分，即先天性免疫分支和适应性免疫分支。某些脑部自身免疫性疾病是这些组成部分之一起主要作用的病症实例。一些罕见的自身免疫性疾病涉及先天性免疫分支的激活，包括慢性婴儿神经、皮肤、关节（CINCA）综合征。相比之下，适应性免疫在多发性硬化症、视神经脊髓炎和副肿瘤综合征中很突出，在这些疾病中，高度特异性的T细胞反应和抗体介导疾病。对自身免疫性脑部疾病的研究有助于阐明免疫学家已经熟知的关键分子（如补体和主要组织相容性复合体的成分）所发挥的不同神经元作用。与此同时，神经生物学和感觉生理学中已知的分子，包括Toll样受体、γ-氨基丁酸和晶状体蛋白αB晶体蛋白，在免疫系统中具有有趣且独特的功能，它们在免疫系统中调节针对大脑的自身免疫。
