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.

周围神经系统 (PNS) 髓磷脂的发育涉及生长中的轴突和最终将轴突包裹起来的雪旺细胞 (SC) 祖细胞之间的一系列协调事件。髓鞘的进化是出于维持快速脉冲传播同时考虑身体空间限制的需要。然而，髓鞘化 SC 还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影响。有毒的侮辱。这些功能丧失和轴突变性开始之间的中间步骤尚不清楚，但这些过程的重要性提供了富有洞察力的线索。PNS 常见的脱髓鞘疾病包括遗传性神经病 1 型夏科-马里-图思病 (CMT1) 和遗传性压力性麻痹神经病 (HNPP) 以及炎症性疾病急性炎症性脱髓鞘性多发性神经病 (AIDP) 和慢性炎症性脱髓鞘性多发性神经病 (CIDP) ）。继发性轴突变性是脱髓鞘性神经病的常见特征，该过程通常与患者的临床缺陷和长期残疾相关。有丰富的电生理学和组织学证据表明 PNS 脱髓鞘疾病患者和啮齿动物模型中存在继发性轴突变性。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了解至关重要，这对于开发新的治疗策略至关重要。