朗维耶淋巴结在健康和疾病中的作用

IF 3.9 3区 医学 Q1 CLINICAL NEUROLOGY Journal of the Peripheral Nervous System Pub Date : 2023-06-05 DOI:10.1111/jns.12568
Yael Eshed-Eisenbach, Peter J. Brophy, Elior Peles
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引用次数: 0

摘要

沿着有髓鞘轴突的动作电位传播取决于髓鞘单位的几何形状和潜在轴突向特殊结构域的划分。后者包括兰维尔结(NOR)、位于结两侧的节旁结(PNJ)以及位于节间致密髓鞘下方的邻近的颈旁区。这些结构域中的每一个都包含一种独特的轴珠粘附分子(CAM)和细胞骨架支架蛋白组成,它们共同指导特定离子通道在淋巴结和颈旁轴上的位置。在过去的十年里,越来越清楚的是,针对其中一些轴突CAM的抗体会导致免疫介导的神经病变。在目前的综述中,我们详细介绍了NOR和相邻膜结构域的分子组成,描述了介导髓鞘单元轴突-神经胶质相互作用的不同CAM复合物的功能,并讨论了它们在外周神经病理中的参与和潜在机制。这一不断增长的病理组代表了一种新型的神经病理学,称为“多巴胺”或“副多巴胺”,其特征是独特的临床和分子特征,这些特征共同反映了分子组装和维持这一特殊膜结构域的机制。
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Nodes of Ranvier in health and disease

Action potential propagation along myelinated axons depends on the geometry of the myelin unit and the division of the underlying axon to specialized domains. The latter include the nodes of Ranvier (NOR), the paranodal junction (PNJ) flanking the nodes, and the adjacent juxtaparanodal region that is located below the compact myelin of the internode. Each of these domains contains a unique composition of axoglial adhesion molecules (CAMs) and cytoskeletal scaffolding proteins, which together direct the placement of specific ion channels at the nodal and juxtaparanodal axolemma. In the last decade it has become increasingly clear that antibodies to some of these axoglial CAMs cause immune-mediated neuropathies. In the current review we detail the molecular composition of the NOR and adjacent membrane domains, describe the function of different CAM complexes that mediate axon-glia interactions along the myelin unit, and discuss their involvement and the underlying mechanisms taking place in peripheral nerve pathologies. This growing group of pathologies represent a new type of neuropathies termed “nodopathies” or “paranodopathies” that are characterized by unique clinical and molecular features which together reflect the mechanisms underlying the molecular assembly and maintenance of this specialized membrane domain.

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来源期刊
CiteScore
6.10
自引率
7.90%
发文量
45
审稿时长
>12 weeks
期刊介绍: The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.
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