Department of Neurogenetics, Max Planck Institute of Experimental Medicine, Göttingen, Germany.
FEBS Lett. 2011 Jul 21;585(14):2205-11. doi: 10.1016/j.febslet.2011.05.032. Epub 2011 May 23.
Demyelinating diseases of the nervous system cause axon loss but the underlying mechanisms are not well understood. Here we show by confocal and electron microscopy that in myelin-forming glia peroxisomes are associated with myelin membranes. When peroxisome biogenesis is experimentally perturbed in Pex5 conditional mouse mutants, myelination by Schwann cells appears initially normal. However, in nerves of older mice paranodal loops become physically unstable and develop swellings filled with vesicles and electron-dense material. This novel model of a demyelinating neuropathy demonstrates that peroxisomes serve an important function in the peripheral myelin compartment, required for long-term axonal integrity.
神经系统脱髓鞘疾病会导致轴突丧失，但其中的潜在机制尚不清楚。在这里，我们通过共聚焦和电子显微镜观察到，在形成髓鞘的神经胶质细胞中，过氧化物酶体与髓鞘膜相关。当过氧化物酶体生物发生在 Pex5 条件性小鼠突变体中受到实验干扰时，施万细胞的髓鞘形成最初看起来正常。然而，在老年小鼠的神经中，连接结的旁区环路变得不稳定，并出现充满囊泡和电子致密物质的肿胀。这种新型脱髓鞘神经病模型表明，过氧化物体在周围髓鞘区具有重要功能，是维持轴突长期完整性所必需的。
