Mechanisms regulating cerebral hypoperfusion in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy  

作　　者：Xi Yan Junkui Shang Runrun Wang Fengyu Wang Jiewen Zhang 

机构地区：[1]Department of Neurology,Henan Provincial People's Hospital,Zhengzhou University People's Hospital,Henan University People's Hospital,Zhengzhou,Henan 450003,China

出　　处：《The Journal of Biomedical Research》2022年第5期353-357,共5页生物医学研究杂志（英文版）

基　　金：This work was supported by National Natural Science Foundation of China(Grants No.81873727 and 82171196).

摘　　要：Cerebral small vessel disease (CSVD) is a leading cause of stroke and dementia. As the most common type of inherited CSVD, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by the NOTCH3 gene mutation which leads to Notch3 ectodomain deposition and extracellular matrix aggregation around the small vessels. It further causes smooth muscle cell degeneration and small vessel arteriopathy in the central nervous system. Compromised cerebral blood flow occurs in the early stage of CADASIL and is associated with white matter hyperintensity, the typical neuroimaging pathology of CADASIL. This suggests that cerebral hypoperfusion may play an important role in the pathogenesis of CADASIL. However, the mechanistic linkage between NOTCH3 mutation and cerebral hypoperfusion remains unknown. Therefore, in this mini-review, it examines the cellular and molecular mechanisms contributing to cerebral hypoperfusion in CADASIL.

关 键 词：cerebral hypoperfusion CADASIL NOTCH3 mural cell ASTROCYTE 

分 类 号：R743[医药卫生—神经病学与精神病学]