脑室周围白质软化症再髓鞘化的研究进展

Progress in Periventricular Leukomalacia and Remyelination of Axons

脑室周围白质软化症(Periventricular leukomalacia,PVL)是一种早产儿脱髓鞘疾病,该病与神经发育关系密切,随着疾病的进一步发展,易引发脑瘫和认知障碍等问题。患儿以大脑损伤及脑室周围白质损害为主要特征,其中成髓鞘前的少突胶质细胞是最重要的靶细胞。就发病机制而言,脑室周围白质软化症与小胶质细胞活化、缺血缺氧损伤、兴奋性氨基酸、感染及炎症反应和大脑白质成髓鞘前少突胶质细胞发育易损性等有关。脑室周围白质软化症脱髓鞘损伤后,中枢神经系统通常会通过转录因子改变等髓鞘化相关机制来促进其再生。其中鞘磷脂基因调节因子可促使成髓鞘前的少突胶质细胞向少突胶质细胞分化,该因子是髓鞘化的关键因子,也受其他转录因子调控。

Periventricular leukomalacia （Periventricular leukomalacia,PVL） is a demyelinating disease in preterm children,the disease is closely related with nerve development,with the further development of the disease,easily lead to cerebral palsy and cognitive impairment and other issues. Children with brain injury and periventricular white matter damage as the main feature,which oligodendrocytes into myelin is the most important pre-target cells. The pathogenesis is concerned,periventricular leukomalacia and microglial activation,hypoxic-ischemic injury,excitatory amino acids,infection and inflammation and white matter into a front myelin oligodendrocyte development and other relevant vulnerability . After periventricular leukomalacia demyelinating damage,central nervous system myelination mechanisms usual y by transcription factors and changes to promote its regeneration. Sphingomyelin gene regulatory factors which can contribute to oligodendrocyte myelin oligodendrocyte prior to cell differentiation and myelination of the factors are key factors,but also by other transcription factor regulation.