Amino Acid and Phospholipid Metabolism as an Indicator of Inflammation and Subtle Cardiomyopathy in Patients with Marfan Syndrome

被引：6

作者：

Bartenbach, Lisa ^{[1
]}

Karall, Thomas ^{[1
]}

Koch, Jakob ^{[2
]}

Keller, Markus Andreas ^{[2
]}

Oberacher, Herbert ^{[3
,4
]}

Scholl-Buergi, Sabine ^{[5
]}

Karall, Daniela ^{[5
]}

Oemer, Gregor ^{[2
]}

Baumgartner, Daniela ^{[6
]}

Meinel, Katharina ^{[6
]}

Aly, Safwat ^{[7
]}

Odri-Komazec, Irena ^{[1
]}

Geiger, Ralf ^{[1
]}

Michel, Miriam ^{[1
]}

机构：

[1] Med Univ Innsbruck, Dept Child & Adolescent Hlth, Div Pediat Cardiol Pulmonol Allergol & Cyst Fibro, A-6020 Innsbruck, Austria

[2] Med Univ Innsbruck, Inst Human Genet, A-6020 Innsbruck, Austria

[3] Med Univ Innsbruck, Inst Legal Med, A-6020 Innsbruck, Austria

[4] Med Univ Innsbruck, Core Facil Metabol, A-6020 Innsbruck, Austria

[5] Med Univ Innsbruck, Dept Child & Adolescent Hlth, Div Pediat Inherited Metab Disorders 1, A-6020 Innsbruck, Austria

[6] Med Univ Graz, Clin Div Pediat Cardiol, Dept Pediat, A-8010 Graz, Austria

[7] Hosp Sick Children, Labatt Family Heart Ctr, Div Pediat Cardiol, Toronto, ON M5G 1X8, Canada

来源：

METABOLITES | 2021年 / 11卷 / 12期

关键词：

cardiomyopathy; Marfan syndrome; metabolomics; taurine; LEFT-VENTRICULAR FUNCTION; OXIDATIVE STRESS; MOUSE MODEL; ASYMMETRIC DIMETHYLARGININE; DIASTOLIC FUNCTION; ADULTS; ECHOCARDIOGRAPHY; DYSFUNCTION; DEFICIENT; CHILDREN;

D O I：

10.3390/metabo11120805

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Patients with Marfan syndrome (MFS) have an increased risk of aortic aneurysm formation, dissection and development of a subtle cardiomyopathy. We analyzed amino acid and lipid metabolic pathways in MFS patients, seeking biomarker patterns as potential monitoring tools of cardiovascular risk with deterioration of myocardial function. We assessed myocardial function in 24 adult MFS patients and compared traditional laboratory values and mass spectrometry-based amino acid, phospholipid and acylcarnitine metabolomes in patients with those in healthy controls. Analytes for which values differed between patients and controls were subjected to regression analysis. A high proportion of patients had signs of impaired diastolic function and elevated serum levels of NT-proBNP. Patients had lower serum levels of taurine, histidine and PCaeC42:3 than controls. The evidence of diastolic dysfunction, aortic root dimensions and history of aortic root surgery correlated with NT-proBNP and taurine levels. Alterations in serum levels of metabolism derived analytes link MFS pathophysiology with inflammation, oxidative stress and incipient cardiomyopathy.

引用

页数：16

共 49 条

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