Department of Neurology, University of Wisconsin, Madison, Wisconsin.
Department of Ophthalmology, Rheinische Friedrich Wilhelms University, Bonn, Germany.
Pediatr Res. 2018 Jun;83(6):1200-1206. doi: 10.1038/pr.2018.45. Epub 2018 May 2.
BackgroundPelizaeus Merzbacher disease (PMD) is a dysmyelinating disorder of the central nervous system caused by impaired differentiation of oligodendrocytes. This study was prompted by findings that antimuscarinic compounds enhance oligodendrocyte differentiation and remyelination in vitro. One of these compounds, clemastine fumarate, is licensed for treatment of allergic conditions. We tested whether clemastine fumarate can promote myelination in two rodent PMD models, the myelin-deficient and the PLP transgenic rat.MethodsPups were treated with daily injections of clemastine (10-30 mg/kg/day) on postnatal days 1-21. Neurologic phenotypes and myelination patterns in the brain, optic nerves, and spinal cords were assessed using histological techniques.ResultsNo changes in neurological phenotype or survival were observed even at the highest dose of clemastine. Postmortem staining with Luxol fast blue and myelin basic protein immunohistochemistry revealed no evidence for improved myelination in the CNS of treated rats compared to vehicle-treated littermates. Populations of mature oligodendrocytes were unaffected by the treatment.ConclusionThese results demonstrate lack of therapeutic effect of clemastine in two rat PMD models. Both models have rapid disease progression consistent with the connatal form of the disease. Further studies are necessary to determine whether clemastine bears a therapeutic potential in milder forms of PMD.
Pelizaeus-Merzbacher 病(PMD)是一种中枢神经系统脱髓鞘疾病,由少突胶质细胞分化受损引起。本研究的起因是发现抗毒蕈碱化合物在体外增强少突胶质细胞分化和髓鞘再生。其中一种化合物富马酸氯马斯汀已获许可用于治疗过敏症。我们在两种啮齿动物 PMD 模型(髓鞘缺失和 PLP 转基因鼠)中测试了富马酸氯马斯汀是否可以促进髓鞘形成。
在产后第 1-21 天,每天给幼崽注射富马酸氯马斯汀(10-30mg/kg/天)。使用组织学技术评估大脑、视神经和脊髓中的神经表型和髓鞘形成模式。
即使在最高剂量的富马酸氯马斯汀下,也未观察到神经表型或存活的变化。与接受载体处理的同窝仔相比,死后用卢索快速蓝和髓鞘碱性蛋白免疫组织化学染色未发现中枢神经系统髓鞘形成得到改善的证据。成熟少突胶质细胞的数量不受治疗的影响。
这些结果表明富马酸氯马斯汀在两种 PMD 大鼠模型中均无治疗作用。这两种模型的疾病进展迅速,与先天性疾病一致。需要进一步研究以确定富马酸氯马斯汀在 PMD 较温和的形式中是否具有治疗潜力。