常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病病理学及分子机制研究进展

常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病(CADASIL)是Notch3基因突变导致的遗传性脑小血管病.目前Notch3基因突变致CADASIL的分子机制尚不明确.本文综述CADASIL的病理改变、基因突变以及可能的相关分子机制,包括Notch3蛋白及其下游靶标的异常信号转导,细胞内质网应激和RhoA/Rho激酶活化,血管平滑肌细胞收缩异常及其自噬机制紊乱、增殖分化异常....

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Published in中国现代神经疾病杂志 Vol. 21; no. 10; pp. 828 - 833
Main Authors 孙瑗璟, 范玉华
Format Journal Article
LanguageChinese
Published 510080广州,中山大学附属第一医院神经科广东省重大神经疾病诊治研究重点实验室国家临床重点专科和国家重点学科 25.10.2021
Subjects
CADASIL;受体,Notch3;病理学;病理学,分子;综述
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ISSN1672-6731
DOI10.3969/j.issn.1672-6731.2021.10.002

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Abstract 常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病(CADASIL)是Notch3基因突变导致的遗传性脑小血管病.目前Notch3基因突变致CADASIL的分子机制尚不明确.本文综述CADASIL的病理改变、基因突变以及可能的相关分子机制,包括Notch3蛋白及其下游靶标的异常信号转导,细胞内质网应激和RhoA/Rho激酶活化,血管平滑肌细胞收缩异常及其自噬机制紊乱、增殖分化异常.
AbstractList 常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病(CADASIL)是Notch3基因突变导致的遗传性脑小血管病.目前Notch3基因突变致CADASIL的分子机制尚不明确.本文综述CADASIL的病理改变、基因突变以及可能的相关分子机制,包括Notch3蛋白及其下游靶标的异常信号转导,细胞内质网应激和RhoA/Rho激酶活化,血管平滑肌细胞收缩异常及其自噬机制紊乱、增殖分化异常.
Author 孙瑗璟
范玉华
AuthorAffiliation 510080广州,中山大学附属第一医院神经科广东省重大神经疾病诊治研究重点实验室国家临床重点专科和国家重点学科
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Author_FL SUN Yuan-jing
FAN Yu-hua
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Title 常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病病理学及分子机制研究进展
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