New evidence for secondary axonal degeneration in demyelinating neuropathies.
New evidence for secondary axonal degeneration in demyelinating neuropathies.
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DOI：
10.1016/j.neulet.2020.135595
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发表时间：
2021-01-23
影响因子：
2.5
通讯作者：
Höke A
中科院分区：
文献类型：
--
作者：
Moss KR;Bopp TS;Johnson AE;Höke A
周围神经系统（PNS）髓磷脂的发育涉及生长轴突和最终包裹它们的许旺细胞（SC）祖细胞之间的一系列协调事件。髓鞘已经进化出维持快速冲动传播的必要性，同时考虑到身体空间的限制。然而，髓鞘形成SC执行保持轴突完整性所需的其他关键功能，包括通过建立节点结构来减轻能量消耗，通过组织轴突细胞骨架网络来调节轴突口径，提供营养和潜在的代谢支持，可能提供遗传翻译材料并保护轴突免受毒性损伤。这些功能的丧失和轴突变性的开始之间的中间步骤是未知的，但这些过程的重要性提供了有见地的线索。PNS的流行性脱髓鞘疾病包括遗传性神经病Charcot-Marie-Tooth病，1型（CMT 1）和遗传性神经病伴易患压力性麻痹（HNPP）以及炎性疾病急性炎性脱髓鞘性多发性神经病（AIDP）和慢性炎性脱髓鞘性多发性神经病（CIDP）。继发性轴突变性是脱髓鞘神经病的常见特征，并且该过程通常与患者的临床缺陷和长期残疾相关。在PNS脱髓鞘疾病的患者和啮齿动物模型中有大量的继发性轴突变性的电生理和组织学证据。充分了解继发性轴突变性在这些疾病中的参与对于扩大我们对疾病发病机制和预后的认识是至关重要的，这对于开发新的治疗策略是至关重要的。
Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will eventually ensheath them. Myelin sheaths have evolved out of necessity to maintain rapid impulse propagation while accounting for body space constraints. However, myelinating SCs perform additional critical functions that are required to preserve axonal integrity including mitigating energy consumption by establishing the nodal architecture, regulating axon caliber by organizing axonal cytoskeleton networks, providing trophic and potentially metabolic support, possibly supplying genetic translation materials and protecting axons from toxic insults. The intermediate steps between the loss of these functions and the initiation of axon degeneration are unknown but the importance of these processes provides insightful clues. Prevalent demyelinating diseases of the PNS include inherited neuropathies Charcot-Marie-Tooth Disease, Type 1 (CMT1) and Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) and the inflammatory diseases Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Secondary axon degeneration is a common feature of demyelinating neuropathies and this process is often correlated with clinical deficits and long-lasting disability in patients. There is abundant electrophysiological and histological evidence for secondary axon degeneration in patients and rodent models of PNS demyelinating diseases. Fully understanding the involvement of secondary axon degeneration in these diseases is essential for expanding our knowledge of disease pathogenesis and prognosis, which will be essential for developing novel therapeutic strategies.
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