Jiang Chunxia, Hu Yunan, Zhang Feng, Qiu Mengsheng, Zhao Xiaofeng
Institute of Developmental and Regenerative Biology, Zhejiang Key Laboratory of Organ Development and Regeneration, College of Life and Environmental Sciences, Hangzhou Normal University, Hangzhou, 310036, China.
Quzhou Affiliated Hospital of Wenzhou Medical University, Quzhou People's Hospital, Quzhou, 324000, China.
Neurosci Bull. 2025 Jul 17. doi: 10.1007/s12264-025-01452-x.
Although oligodendrocytes (OLs) are known to form the myelin sheath around neuronal axons for the saltatory conduction of action potentials, recent studies have suggested that OLs also modulate neuronal function and plasticity. In the present study, we found that OL maturation deficiency in Myrf-CKO mice caused spontaneous epileptogenesis and resulted in death. To further investigate the association between OL development and epilepsy, we examined the Adamts4 KO mouse line, which has a mild OL differentiation phenotype in the hippocampus. As a result, the differentiation defect in the mutant hippocampus reduced the expression of myelin-associated glycoprotein and lessened its inhibition of the dephosphorylation of phosphorylated tropomyosin-related kinase B, which is associated with retarded adolescent hippocampal mossy fiber development and higher susceptibility to epileptogenesis in adulthood. More importantly, enhancing differentiation by orally administered clemastine rescues the defective mossy fiber development in the early postnatal period and attenuates epilepsy susceptibility in adults. Together, these results strongly suggest that an OL differentiation defect in the hippocampus may contribute to susceptibility to epilepsy in adults.
尽管已知少突胶质细胞(OLs)会在神经元轴突周围形成髓鞘以实现动作电位的跳跃传导,但最近的研究表明,OLs 还能调节神经元功能和可塑性。在本研究中,我们发现 Myrf - CKO 小鼠中的 OL 成熟缺陷会导致自发性癫痫发作并最终死亡。为了进一步研究 OL 发育与癫痫之间的关联,我们检测了 Adamts4 KO 小鼠品系,该品系在海马体中具有轻度的 OL 分化表型。结果,突变体海马体中的分化缺陷降低了髓鞘相关糖蛋白的表达,并减弱了其对磷酸化原肌球蛋白相关激酶 B 去磷酸化的抑制作用,这与青少年海马苔藓纤维发育迟缓以及成年期癫痫易感性增加有关。更重要的是,通过口服氯马斯汀增强分化可挽救出生后早期有缺陷的苔藓纤维发育,并减轻成年期的癫痫易感性。总之,这些结果有力地表明,海马体中的 OL 分化缺陷可能导致成年期癫痫易感性增加。