Advances in molecular mechanisms of thoracic aorta disease in Marfan syndrome

VernacularTitle:马凡综合征胸主动脉病变的分子机制研究进展
Author: Qiuyue GAO ^{1
,

2} ; Yiming ZHAO ; Baoqi YU
Author Information

1. 首都医科大学基础医学院生理学与病理生理学系,北京市 100069
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
Keywords: Marfan syndrome; thoracic aortic aneurysm; thoracic aortic dissection; transforming growth factor-β; matrix metalloproteinase
From: Chinese Journal of Arteriosclerosis 2024;32(8):645-653
CountryChina
Language:Chinese
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.