ArticleJournal of inherited metabolic disease2013
Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice.
Article in Journal of inherited metabolic disease, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 35 citations in OpenAlex.
- The Pathogenesis of Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.Biomolecules · 2025Review
- Assessment of cardiac and skeletal muscle metabolites usingNMR in biomedicine · 2025Article
- Tissue specific roles of fatty acid oxidation.Advances in biological regulation · 2025Review
- A review of fatty acid oxidation disorder mouse models.Molecular genetics and metabolism · 2024Review
- Metabolomic analysis of pig spleen reveals African swine fever virus infection increased acylcarnitine levels to facilitate viral replication.Journal of virology · 2023Article
- Long-Chain Acyl Carnitines Aggravate Polystyrene Nanoplastics-Induced Atherosclerosis by Upregulating MARCO.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2023Article
- The buzz in the field: the interaction between viruses, mosquitoes, and metabolism.Frontiers in cellular and infection microbiology · 2023Review
- Intestinal farnesoid X receptor signaling controls hepatic fatty acid oxidation.Biochimica et biophysica acta. Molecular and cell biology of lipids · 2022Article
- Murine deficiency of peroxisomal L-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis.Cellular and molecular life sciences : CMLS · 2021Article
- CardiacHeart failure reviews · 2021Review
- Dietary restriction in the long-chain acyl-CoA dehydrogenase knockout mouse.Molecular genetics and metabolism reports · 2021Article
- Enhancing Fatty Acids OxidationFrontiers in pharmacology · 2021Article
- Subclinical effects of long-chain fatty acid β-oxidation deficiency on the adult heart: A case-control magnetic resonance study.Journal of inherited metabolic disease · 2020Article
- Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Do not Improve with Carnitine Supplementation.Frontiers in pharmacology · 2020Article
- Dynamic remodeling of lipids coincides with dengue virus replication in the midgut of Aedes aegypti mosquitoes.PLoS pathogens · 2018Article
- The promiscuous enzyme medium-chain 3-keto-acyl-CoA thiolase triggers a vicious cycle in fatty-acid beta-oxidation.PLoS computational biology · 2017Article
- Diagnosis, Treatment, and Clinical Outcome of Patients with Mitochondrial Trifunctional Protein/Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency.JIMD reports · 2017Article
- Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience.Molecular genetics and metabolism · 2015Article
- Long-chain Acylcarnitines Reduce Lung Function by Inhibiting Pulmonary Surfactant.The Journal of biological chemistry · 2015Article
- DYNAmic Multi-coIl TEchnique (DYNAMITE) shimming of the rat brain at 11.7 T.NMR in biomedicine · 2014Article
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeElevation of long-chain acylcarnitine levels is a hallmark of long-chain mitochondrial β-oxidation (FAO) disorders, and can be accompanied by secondary carnitine deficiency. To restore free carnitine levels, and to increase myocardial export of long-chain fatty acyl-CoA esters, supplementation of L-carnitine in patients has been proposed. However, carnitine supplementation is controversial, because it may enhance the potentially lipotoxic buildup of long-chain acylcarnitines in the FAO-deficient heart. In this longitudinal study, we investigated the effects of carnitine supplementation in an animal model of long-chain FAO deficiency, the long-chain acyl-CoA dehydrogenase (LCAD) knockout (KO) mouse.
methodsCardiac size and function, and triglyceride (TG) levels were quantified using proton magnetic resonance imaging (MRI) and spectroscopy ((1)H-MRS) in LCAD KO and wild-type (WT) mice. Carnitine was supplemented orally for 4 weeks starting at 5 weeks of age. Non-supplemented animals served as controls. In vivo data were complemented with ex vivo biochemical assays.
resultsLCAD KO mice displayed cardiac hypertrophy and elevated levels of myocardial TG compared to WT mice. Carnitine supplementation lowered myocardial TG, normalizing myocardial TG levels in LCAD KO mice. Furthermore, carnitine supplementation did not affect cardiac performance and hypertrophy, or induce an accumulation of potentially toxic long-chain acylcarnitines in the LCAD KO heart.
conclusionThis study lends support to the proposed beneficial effect of carnitine supplementation alleviating toxicity by exporting acylcarnitines out of the FAO-deficient myocardium, rather than to the concern about a potentially detrimental effect of supplementation-induced production of lipotoxic long-chain acylcarnitines.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.