异常的长链脂肪酸代谢与不断发展的系统性硬化症相关的肺动脉高血压有关
Julie C Coursen1, Tijana Tuhy2, Mario Naranjo3
1Division of Hospital Medicine, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, United States.
概括
发生肺动脉高血压的全身性硬化症患者表现出明显的长链脂肪酸 (LCFA) 代谢. 这些脂质体特征可以在诊断前几年和轻度疾病中预测PAH的发展.
科学领域:
- 生物化学 生物化学
- 代谢学 代谢学 代谢学
- 肺部病理学 肺部病理学
背景情况:
- 系统性硬化症 (SSc) 是一种具有显著发病率和死亡率的结合组织疾病.
- 肺动脉高血压 (PAH) 是SSc的严重并发症,需要早期检测和生物标志物.
- 在SSc-PAH患者中,差异性新陈代谢仍未得到充分研究.
研究的目的:
- 在SSc患者中识别不同的血清代谢物,这些患者患有PAH,而不是那些不患PAH的患者.
- 研究这些代谢物作为SSc.中PAH早期生物标志物的潜力.
- 描述脂质代谢在SSc-PAH病变发生中的作用.
主要方法:
- 嵌套病例控制研究设计,使用SSc患者的血清样本进行PAH和没有PAH.
- 使用液态染色体质谱学 (LC-MS) 进行代谢物概况分析.
- 在独立的SSc队列中验证和对PAH发展的预测价值的评估.
主要成果:
- 长链脂肪酸 (LCFA) 的代谢在SSc-PAH患者中被持续改变.
- LCFA代谢物,特别是乙胆和酸衍生物,有区别的SSc-PAH.
- 在临床诊断和确定轻度疾病前2年,这些脂质体标预测了PAH.
结论:
- 异常的脂质代谢,特别是失调的LCFA代谢,是SSc-PAH的标志.
- 脂质组特征可以作为SSc患者PAH的预测生物标志物.
- 了解这些代谢变化可能为SSc-PAH提供新的治疗点.
关键词:
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