Autoimmunity of the peripheral and central nervous system is an important cause of disease and long-term neurological disability. Autoantibodies can target both intracellular and extracellular neuronal epitopes. Autoantibodies that target cell-surface epitopes infer pathogenicity through several distinct mechanisms, while patients often respond to immunotherapy. However, the underlying pathogenesis of these autoantibodies is yet to be fully understood. Human stem cell–based disease modeling, and the rise of induced pluripotent stem cell technology in particular, has revolutionized the fields of disease modeling and therapeutic screening for neurological disorders. These human disease models offer a unique platform in which to study autoimmunity of the nervous system. Here, we take an in-depth look at the possibilities that these models provide to study neuronal autoantibodies and their underlying pathogenesis.

周围和中枢神经系统的自身免疫是疾病和长期神经系统残疾的重要原因。自身抗体可以靶向细胞内和细胞外神经元表位。靶向细胞表面表位的自身抗体可通过几种不同的机制推断致病性，而患者通常会对免疫疗法产生反应。然而，这些自身抗体的潜在发病机理尚未完全了解。基于人类干细胞的疾病建模，尤其是诱导多能干细胞技术的兴起，彻底改变了疾病建模和神经疾病治疗筛选领域。这些人类疾病模型提供了一个独特的平台，用于研究神经系统的自身免疫性。这里，