The neurobiology and immunology of CASPR2-associated neurological disorders

IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY
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引用次数: 0

Abstract

CASPR2-associated neurological disorders encompass a wide clinical spectrum broadly divided into overlapping three autoimmune syndromes: CASPR2 limbic encephalitis, Morvan syndrome, and Isaacs syndrome. CASPR2 is a neuronal protein expressed at different sites in the central and peripheral nervous system and has a variety of roles and functions regarding neuronal excitability, synaptic plasticity, and homeostasis of inhibitory networks, most of which are only partially understood. CASPR2 antibodies have various pathogenic effects including internalization of CASPR2, disruption of protein-protein interactions, and, possibly, complement activation. Their pathogenic effect is well demonstrated in the limbic encephalitis phenotype, but the role of pathogenic antibodies in the development of other clinical manifestations is less clear. CASPR2 limbic encephalitis also differ from the other CASPR2-associated disorders in regard to HLA allele and paraneoplastic associations, suggesting it has immunological mechanisms distinct from the other clinical forms. Future studies are needed to better understand how the immunological alterations lead to the different phenotypes associated with CASPR2 antibodies.
CASPR2 相关神经系统疾病的神经生物学和免疫学。
CASPR2 相关神经系统疾病的临床范围很广,大致可分为三种重叠的自身免疫综合征:CASPR2 边缘脑炎、莫尔万综合征和艾萨克斯综合征。CASPR2 是一种在中枢神经系统和周围神经系统不同部位表达的神经元蛋白,在神经元兴奋性、突触可塑性和抑制网络的平衡方面具有多种作用和功能,其中大部分作用和功能目前还只是部分了解。CASPR2 抗体有多种致病作用,包括 CASPR2 的内化、蛋白-蛋白相互作用的破坏以及补体激活。它们的致病作用在边缘性脑炎表型中得到了很好的证实,但致病抗体在其他临床表现中的作用却不太清楚。CASPR2边缘脑炎在HLA等位基因和副肿瘤关联方面也不同于其他CASPR2相关疾病,这表明它的免疫机制不同于其他临床形式。未来的研究需要更好地了解免疫学改变是如何导致与 CASPR2 抗体相关的不同表型的。
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来源期刊
Revue neurologique
Revue neurologique 医学-临床神经学
CiteScore
4.80
自引率
0.00%
发文量
598
审稿时长
55 days
期刊介绍: The first issue of the Revue Neurologique, featuring an original article by Jean-Martin Charcot, was published on February 28th, 1893. Six years later, the French Society of Neurology (SFN) adopted this journal as its official publication in the year of its foundation, 1899. The Revue Neurologique was published throughout the 20th century without interruption and is indexed in all international databases (including Current Contents, Pubmed, Scopus). Ten annual issues provide original peer-reviewed clinical and research articles, and review articles giving up-to-date insights in all areas of neurology. The Revue Neurologique also publishes guidelines and recommendations. The Revue Neurologique publishes original articles, brief reports, general reviews, editorials, and letters to the editor as well as correspondence concerning articles previously published in the journal in the correspondence column.
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