神经科学
少突胶质细胞
亨廷顿病
髓鞘
白质
亨廷顿蛋白
发病机制
疾病
生物
多发性硬化
病理
医学
中枢神经系统
免疫学
磁共振成像
放射科
作者
Costanza Ferrari Bardile,Carola I. Radulescu,Mahmoud A. Pouladi
标识
DOI:10.1016/j.molmed.2023.07.010
摘要
Oligodendrocytes (OLGs), highly specialized glial cells that wrap axons with myelin sheaths, are critical for brain development and function. There is new recognition of the role of OLGs in the pathogenesis of neurodegenerative diseases (NDDs), including Huntington’s disease (HD), a prototypic NDD caused by a polyglutamine tract expansion in huntingtin (HTT), which results in gain- and loss-of-function effects. Clinically, HD is characterized by a constellation of motor, cognitive, and psychiatric disturbances. White matter (WM) structures, representing myelin-rich regions of the brain, are profoundly affected in HD, and recent findings reveal oligodendroglia dysfunction as an early pathological event. Here, we focus on mechanisms that underlie oligodendroglial deficits and dysmyelination in the progression of the disease, highlighting the pathogenic contributions of mutant HTT (mHTT). We also discuss potential therapeutic implications involving these molecular pathways.
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