烟雾病易感因素研究进展  

Advance in predisposing factors for moyamoya disease

作　　者：郭鹏宇 张豪杰 张增雨 李怡萌 范亚杰 齐金博 卢壮壮 邱艳艳 杨静怡 邢红霞[2] 李超堃[1] Guo Pengyu;Zhang Haojie;Zhang Zengyu;Li Yimeng;Fan Yajie;Qi Jinbo;Lu Zhuangzhuang;Qiu Yanyan;Yang Jingyi;Xing Hongxia;Li Chaokun(Department of Physiology and Neurobiology,Xinxiang Medical University,Sino-UK Joint Laboratory for Brain Function and Injury of Henan Province,Xinxiang 453000,China)

机构地区：[1]新乡医学院生理学与神经生物学教研室河南省中英脑功能损伤国际联合实验室,453000 [2]新乡医学院第一附属医院河南省神经修复重点实验室

出　　处：《中国脑血管病杂志》2019年第2期101-108,共8页Chinese Journal of Cerebrovascular Diseases

基　　金：河南省自然科学基金资助项目(162300410218);河南省高等学校青年骨干教师资助计划(2015GGJS-132);河南省神经修复重点实验室开放课题(HNSJXF-2018-002)

摘　　要：烟雾病是一种进行性脑血管病,具有种族易感性、家族聚集性特征。烟雾血管表现出内膜增厚,中膜变薄,内弹力板迂曲,伴有淋巴细胞浸润等特征性病理表现,其病因及发病机制尚未完全阐明,目前认为该病与内皮细胞、平滑肌细胞、细胞外基质的异常代谢和炎性反应相关。作者从遗传因素、机体内环境因素、免疫因素3个层面,阐述内皮细胞易感基因环指蛋白213、平滑肌细胞易感基因、细胞外基质代谢相关基因、血管生成相关因子以及免疫易感基因在烟雾病的发生、发展中所起的作用。Moyamoya disease(MMD)is a progressive cerebrovascular disease with characteristics of ethnic susceptibility and familial aggregation.The pathological changes of stenosed vessels in MMD include intima thickening,tunica media thinning,irregular undulation of the internal elastic laminae and lymphocytic infiltration.The etiology and pathogenesis are not completely clarified.It is presently thought that this disease is related to endothelial cells,smooth muscle cells,abnormal metabolism of extracellular matrix and inflammatory responses.This review will discuss,from genetic,cellular environmental and immunological perspectives,the specific roles of several genes in the development of MMD,including endothelial cell susceptibility gene ring finger protein 213,smooth muscle cell susceptibility genes,extracellular matrix genes,angiogenesis factors and immune-related genes.

关 键 词：脑底异常血管网病 遗传学 细胞内环境 免疫 发病机制 综述 

分 类 号：R743[医药卫生—神经病学与精神病学]