ArticlePediatric research2013
L-carnitine preserves endothelial function in a lamb model of increased pulmonary blood flow.
Article in Pediatric research, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers.
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Who cites it
30 citing papers in PubMed, 47 citations in OpenAlex.
- Effects of L-carnitine supplementation in patients with mild-to-moderate COVID-19 disease: a pilot study.Pharmacological reports : PR · 2022Trial
- Combined Metabolic Activators Accelerates Recovery in Mild-to-Moderate COVID-19.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2021Trial
- Identification of Diagnostic Biomarkers Causally Associated With Gut Microbiota and Pulmonary Arterial Hypertension.Pulmonary circulation · 2025Article
- Mechanotransductive stabilization of HIF-1α is inhibited by mitochondrial antioxidant therapy in the setting of pulmonary overcirculation.Scientific reports · 2025Article
- Alterations in Mitochondrial Function in Pulmonary Vascular Diseases.Antioxidants & redox signaling · 2025Article
- Innovative Dual Combination Cospray-Dried Rock Inhibitor/l-Carnitine Inhalable Dry Powder Aerosols.ACS bio & med chem Au · 2024Article
- The mitochondrial redistribution of ENOS is regulated by AKT1 and dimer status.Nitric oxide : biology and chemistry · 2024Article
- Mitochondrial hyperfusion induces metabolic remodeling in lung endothelial cells by modifying the activities of electron transport chain complexes I and III.Free radical biology & medicine · 2024Article
- Simvastatin restores pulmonary endothelial function in the setting of pulmonary over-circulation.Nitric oxide : biology and chemistry · 2024Article
- Novel Relationship between Mitofusin 2-Mediated Mitochondrial Hyperfusion, Metabolic Remodeling, and Glycolysis in Pulmonary Arterial Endothelial Cells.International journal of molecular sciences · 2023Article
- Metabolic reprogramming, oxidative stress, and pulmonary hypertension.Redox biology · 2023Review
- l-Carnitine therapy improves right heart dysfunction through Cpt1-dependent fatty acid oxidation.Pulmonary circulation · 2022Article
- Mitochondrial Metabolism, Redox, and Calcium Homeostasis in Pulmonary Arterial Hypertension.Biomedicines · 2022Review
- Computational simulation-derived hemodynamic and biomechanical properties of the pulmonary arterial tree early in the course of ventricular septal defects.Biomechanics and modeling in mechanobiology · 2021Article
- Arginine recycling in endothelial cells is regulated BY HSP90 and the ubiquitin proteasome system.Nitric oxide : biology and chemistry · 2021Article
- Metabolism in Pulmonary Hypertension.Annual review of physiology · 2021Review
- Comparison of l-Carnitine and l-Carnitine HCL salt for targeted lung treatment of pulmonary hypertension (PH) as inhalation aerosols: Design, comprehensive characterization, in vitro 2D/3D cell cultures, and in vivo MCT-Rat model of PH.Pulmonary pharmacology & therapeutics · 2020Article
- Metabolomics reveals metabolite changes of patients with pulmonary arterial hypertension in China.Journal of cellular and molecular medicine · 2020Article
- Biomechanical Forces and Oxidative Stress: Implications for Pulmonary Vascular Disease.Antioxidants & redox signaling · 2019Review
- Use of Electron Paramagnetic Resonance in Biological Samples at Ambient Temperature and 77 K.Journal of visualized experiments : JoVE · 2019Article
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 3 countries.
Funding
Abstract
backgroundIn our model of a congenital heart defect (CHD) with increased pulmonary blood flow (PBF; shunt), we have recently shown a disruption in carnitine homeostasis, associated with mitochondrial dysfunction and decreased endothelial nitric oxide synthase (eNOS)/heat shock protein (Hsp)90 interactions that contribute to eNOS uncoupling, increased superoxide levels, and decreased bioavailable nitric oxide (NO). Therefore, we undertook this study to test the hypothesis that L-carnitine therapy would maintain mitochondrial function and NO signaling.
methodsThirteen fetal lambs underwent in utero placement of an aortopulmonary graft. Immediately after delivery, lambs received daily treatment with oral L-carnitine or its vehicle.
resultsL-Carnitine-treated lambs had decreased levels of acylcarnitine and a reduced acylcarnitine:free carnitine ratio as compared with vehicle-treated shunt lambs. These changes correlated with increased carnitine acetyl transferase (CrAT) protein and enzyme activity and decreased levels of nitrated CrAT. The lactate:pyruvate ratio was also decreased in L-carnitine-treated lambs. Hsp70 protein levels were significantly decreased, and this correlated with increases in eNOS/Hsp90 interactions, NOS activity, and NOx levels, and a significant decrease in eNOS-derived superoxide. Furthermore, acetylcholine significantly decreased left pulmonary vascular resistance only in L-carnitine-treated lambs.
conclusionL-Carnitine therapy may improve the endothelial dysfunction noted in children with CHDs and has important clinical implications that warrant further investigation.
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