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Updated: Sep 13, 2025

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过度的糖化驱动胸前大动脉动脉瘤的形成,通过综合应激反应

Antonio Rochano-Ortiz¹, Irene San Sebastián-Jaraba^1,2, Carmen Zamora^1,3

¹Laboratory of Vascular Pathology, Health Research Institute-Fundación Jiménez Díaz University Hospital, Universidad Autónoma de Madrid (IIS-FJD, UAM), 28040 Madrid, Spain.

European heart journal

|July 28, 2025

概括

六胺生物合成途径 (HBP) 和综合应激反应 (ISR) 驱动胸前大动脉动脉瘤和解剖 (TAAD). 针对这一HBP-ISR轴为遗传和非遗传TAAD提供了一个潜在的治疗策略.

科学领域:

心血管生物学心血管生物学
结合组织疾病结合组织疾病
代谢途径代谢途径

背景情况:

胸前大动脉动脉瘤和解剖 (TAAD) 涉及大动脉中枢退化,并与马凡综合征 (MFS) 相关.
血管光滑肌细胞代谢功能障碍是TAAD发病的一个关键因素,但手术修复仍然是主要的治疗方法.
在TAAD病理生理学中,胺生物合成途径 (HBP) 的作用需要进一步研究.

研究的目的:

研究赫索胺生物合成途径 (HBP) 在零星和遗传胸前大动脉动脉瘤和解剖 (TAAD) 的病理生理学中的作用.

主要方法:

在小鼠模型 (MFS,β-aminopropionitrile诱导的TAAD) 和人类TAAD样本中使用转录和代谢学方法分析HBP激活.
通过超声波成像监测大动脉扩张.
通过药理抑制HBP和综合应激反应 (ISR) 来评估治疗潜力.

主要成果:

在MFS和非遗传TAAD模型中,以及在人类MFS和零星TAAD患者样本中,HBP被上调.
增加的HBP活动加剧了大动脉扩张和中间退行,通过损害血管光滑肌肉细胞功能和激活ISR.
抑制HBP或ISR在MFS小鼠模型中改善了这些有害影响.

结论:

HBP-ISR轴是TAAD中中枢退化的一个关键驱动因素.
针对HBP-ISR途径为TAAD的遗传和非遗传形式提供了一个有希望的治疗途径.

关键词:

大动脉中枢退化赫索胺生物合成途径综合应激反应综合应激反应马芬综合征是什么意思马芬综合征胸前大动脉动脉瘤胸前大动脉动脉瘤

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