马凡综合征胸主动脉病变的分子机制研究进展

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马凡综合征胸主动脉病变的分子机制研究进展
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作者:
                        高秋月1,2高秋月
首都医科大学基础医学院生理学与病理生理学系, ;心血管重塑相关疾病教育部重点实验室 代谢紊乱相关心血管疾病北京市重点实验室,北京市 100069 [专家简介] 于宝琪,博士,副教授,硕士研究生导师。主要从事血管干细胞及血管重塑性相关疾病的机制研究,发表SCI论文30余篇,其中以第一作者及通信作者含共同在Circulation等心血管领域杂志上发表SCI论文6篇。已获2项国家自然科学面上基金,并作为课题骨干参与国家重点研发计划青年科学家项目已获北京市委组织部优秀人才青年拔尖个人项目、北京市教委市属高校高水平教师队伍建设青年拔尖人才培育计划、北京市海外高层次人才青年项目等多项人才项目,授权1项发明专利。现任北京医学会血栓与止血分会青年委员会委员、《中国动脉硬化杂志》编委会青年编委、北京生理科学会理事会理事、中国生物物理学会代谢生物学分会青年委员、Global Translational Medicine编委。
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赵一铭1,2赵一铭
首都医科大学基础医学院生理学与病理生理学系, ;心血管重塑相关疾病教育部重点实验室 代谢紊乱相关心血管疾病北京市重点实验室,北京市 100069 [专家简介] 于宝琪,博士,副教授,硕士研究生导师。主要从事血管干细胞及血管重塑性相关疾病的机制研究,发表SCI论文30余篇,其中以第一作者及通信作者含共同在Circulation等心血管领域杂志上发表SCI论文6篇。已获2项国家自然科学面上基金,并作为课题骨干参与国家重点研发计划青年科学家项目已获北京市委组织部优秀人才青年拔尖个人项目、北京市教委市属高校高水平教师队伍建设青年拔尖人才培育计划、北京市海外高层次人才青年项目等多项人才项目,授权1项发明专利。现任北京医学会血栓与止血分会青年委员会委员、《中国动脉硬化杂志》编委会青年编委、北京生理科学会理事会理事、中国生物物理学会代谢生物学分会青年委员、Global Translational Medicine编委。
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于宝琪1,2于宝琪
首都医科大学基础医学院生理学与病理生理学系, ;心血管重塑相关疾病教育部重点实验室 代谢紊乱相关心血管疾病北京市重点实验室,北京市 100069 [专家简介] 于宝琪,博士,副教授,硕士研究生导师。主要从事血管干细胞及血管重塑性相关疾病的机制研究,发表SCI论文30余篇,其中以第一作者及通信作者含共同在Circulation等心血管领域杂志上发表SCI论文6篇。已获2项国家自然科学面上基金,并作为课题骨干参与国家重点研发计划青年科学家项目已获北京市委组织部优秀人才青年拔尖个人项目、北京市教委市属高校高水平教师队伍建设青年拔尖人才培育计划、北京市海外高层次人才青年项目等多项人才项目,授权1项发明专利。现任北京医学会血栓与止血分会青年委员会委员、《中国动脉硬化杂志》编委会青年编委、北京生理科学会理事会理事、中国生物物理学会代谢生物学分会青年委员、Global Translational Medicine编委。
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作者单位:(1.首都医科大学基础医学院生理学与病理生理学系, ;2.心血管重塑相关疾病教育部重点实验室 代谢紊乱相关心血管疾病北京市重点实验室,北京市 100069) [专家简介] 于宝琪,博士,副教授,硕士研究生导师。主要从事血管干细胞及血管重塑性相关疾病的机制研究,发表SCI论文30余篇,其中以第一作者及通信作者(含共同)在Circulation等心血管领域杂志上发表SCI论文6篇。已获2项国家自然科学面上基金,并作为课题骨干参与国家重点研发计划青年科学家项目;已获北京市委组织部优秀人才(青年拔尖个人项目)、北京市教委市属高校高水平教师队伍建设(青年拔尖人才培育计划)、北京市海外高层次人才(青年项目)等多项人才项目,授权1项发明专利。现任北京医学会血栓与止血分会青年委员会委员、《中国动脉硬化杂志》编委会青年编委、北京生理科学会理事会理事、中国生物物理学会代谢生物学分会青年委员、Global Translational Medicine编委。
作者简介:高秋月,硕士研究生,研究方向为干细胞与主动脉瘤,E-mail:gaoqiuyue@mail.ccmu.edu.cn。
通讯作者:
基金项目:国家自然科学基金资助项目(32271231和81870186)

Advances in molecular mechanisms of thoracic aorta disease in Marfan syndrome

Author:

GAO Qiuyue ^{^1,2}
GAO Qiuyue
Department of Physiology and Pathophysiology, School of Basic Medicine, Capital Medical University, Beijing 100069, China;The Key Laboratory of Cardiovascular Remodeling-related Diseases, Ministry of Education & Beijing Key Laboratory of Metabolic Disorder Related Cardiovascular Disease, Beijing 100069, China
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ZHAO Yiming ^{^1,2}
ZHAO Yiming
Department of Physiology and Pathophysiology, School of Basic Medicine, Capital Medical University, Beijing 100069, China;The Key Laboratory of Cardiovascular Remodeling-related Diseases, Ministry of Education & Beijing Key Laboratory of Metabolic Disorder Related Cardiovascular Disease, Beijing 100069, China
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YU Baoqi ^{^1,2}
YU Baoqi
Department of Physiology and Pathophysiology, School of Basic Medicine, Capital Medical University, Beijing 100069, China;The Key Laboratory of Cardiovascular Remodeling-related Diseases, Ministry of Education & Beijing Key Laboratory of Metabolic Disorder Related Cardiovascular Disease, Beijing 100069, China
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Affiliation:

1.Department of Physiology and Pathophysiology, School of Basic Medicine, Capital Medical University, Beijing 100069, China;2.The Key Laboratory of Cardiovascular Remodeling-related Diseases, Ministry of Education & Beijing Key Laboratory of Metabolic Disorder Related Cardiovascular Disease, Beijing 100069, China)

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摘要:

胸主动脉瘤/夹层是马凡综合征(MFS)患者主要的死亡原因。尽管转化生长因子β(TGF-β)通路的异常激活被认为是MFS胸主动脉瘤的核心发病机制,但近些年来的研究逐渐揭示了其他信号通路在MFS中的作用。本文将从经典TGF-β以及Notch、一氧化氮(NO)等相关信号通路、表观遗传学及药物基因治疗等多方面最新研究的成果综述MFS的分子机制,为MFS的预防和治疗提供新的思路。

关键词:马凡综合征;胸主动脉瘤;胸主动脉夹层;转化生长因子β;基质金属蛋白酶

Abstract:

Aortic aneurysm/dissection is the primary cause of mortality in patients with Marfan syndrome (MFS).Though aberrant activation of the transforming growth factor-β(TGF-β) pathway has been considered the central pathogenic mechanism for MFS aortic aneurysms, recent research has gradually revealed the involvement of other signaling pathways in MFS. This review summarizes the latest researches on the molecular mechanisms of MFS, including classical TGF-β and related signaling pathways such as Notch and nitric oxide(NO), as well as epigenetics and gene therapy, which provide new insights for the prevention and treatment of MFS.

Key words:Marfan syndrome; thoracic aortic aneurysm; thoracic aortic dissection; transforming growth factor-β; matrix metalloproteinase

参考文献

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高秋月,赵一铭,于宝琪.马凡综合征胸主动脉病变的分子机制研究进展[J].中国动脉硬化杂志,2024,32(8):645~653.

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收稿日期:2024-04-04
最后修改日期:2024-05-21
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在线发布日期: 2024-08-21

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