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'carnitine' 'cardiac'

Jie Li, Brandon A Kemp, Nancy L Howell, James Massey, Krzysztof Mińczuk, Qiao Huang, Mahendra D Chordia, R Jack Roy, James T Patrie, Giovanni E Davogustto, Christopher M Kramer, Frederick H Epstein, Robert M Carey, Heinrich Taegtmeyer, Susanna R Keller, Bijoy K Kundu
Background Sustained pressure overload leads to changes in cardiac metabolism, function, and structure. Both time course and causal relationships between these changes are not fully understood. Therefore, we studied spontaneously hypertensive rats (SHR) during early hypertension development and compared them to control Wistar Kyoto rats. Methods and Results We serially evaluated myocardial glucose uptake rates (Ki) with dynamic 2-[18 F] fluoro-2-deoxy-D-glucose positron emission tomography, and ejection fraction and left ventricular mass to body weight ratios with cardiac magnetic resonance imaging in vivo, determined glucose uptake and oxidation rates in isolated perfused hearts, and analyzed metabolites, mammalian target of rapamycin activity and endoplasmic reticulum stress in dissected hearts...
February 19, 2019: Journal of the American Heart Association
Mohamed M Sayed-Ahmed, Badr I Alrufaiq, Ammar Alrikabi, Mashan L Abdullah, Mohamed M Hafez, Othman A Al-Shabanah
This study has been initiated to investigate whether sunitinib (SUN) alters the expression of key genes engaged in mitochondrial transport and oxidation of long chain fatty acids (LCFA), and if so, whether these alterations should be viewed as a mechanism of SUN-induced cardiotoxicity, and to explore the molecular mechanisms whereby carnitine supplementation could attenuate SUN-induced cardiotoxicity. Adult male Wister albino rats were assigned to one of the four treatment groups: Rats in group 1 received no treatment but free access to tap water for 28 days...
January 14, 2019: Cardiovascular Toxicology
Xia Zhang, Chun Liu, Congcong Liu, Yan Wang, Wenhua Zhang, Yanqiu Xing
BACKGROUND: This study aimed to investigate the effects of trimetazidine and l‑carnitine on heart aging and cardiac metabolism in the natural aging rats and explore the possible mechanism regarding the regulation of cardiac metabolic substrates. METHODS: A total of 28 young (2-month-old) and 28 aged (14-month-old) male Sprague-Dawley rats were randomly allocated to the following groups: young control (YC, n = 8), young trimetazidine (YT, n = 10), young l‑carnitine (YL, n = 10), aging control (AC, n = 8), aging trimetazidine (AT, n = 10), and aging l‑carnitine (AL, n = 10)...
January 8, 2019: Experimental Gerontology
Patrícia Janeiro, Rita Jotta, Ruben Ramos, Cristina Florindo, Fátima V Ventura, Laura Vilarinho, Isabel Tavares de Almeida, Ana Gaspar
Fatty acid β-oxidation (FAO) disorders have a wide variety of symptoms, not usually evident between episodes of acute decompensations. Cardiac involvement is frequent, and severe ventricular arrhythmias are suspected of causing sudden death. Expanded newborn screening (ENS) for these disorders, hopefully, contribute to prevent potentially acute life-threatening events. In order to characterize acute decompensations observed in FAO-deficient cases identified by ENS, a retrospective analysis was performed, covering a period of 9 years...
January 7, 2019: European Journal of Pediatrics
Gabor Voros, Joris Ector, Christophe Garweg, Walter Droogne, Johan Van Cleemput, Nele Peersman, Pieter Vermeersch, Stefan Janssens
BACKGROUND: Deranged energy metabolism contributes to the pathophysiology of heart failure (HF). Recent studies showed diminished free fatty acid (FFA) oxidation in experimental HF models with a shift towards oxidation of ketone bodies. However, conflicting clinical data on FFA metabolism and limited knowledge on ketone body metabolism in human HF mandate additional metabolic profiling studies. We, therefore, investigated cardiac uptake of FFAs and ketone bodies (β-hydroxybutyrate and acetoacetate) in patients with HF with reduced ejection fraction (HFrEF) or with aortic stenosis (AS)-induced left ventricular hypertrophy...
December 2018: Circulation. Heart Failure
Yoshihiro Miyagawa, Takuya Mori, Kei Goto, Isao Kawahara, Rina Fujiwara-Tani, Shingo Kishi, Takamitsu Sasaki, Kiyomu Fujii, Hitoshi Ohmori, Hiroki Kuniyasu
BACKGROUND: Oral intake of medium-chain fatty acids (MCFAs) reportedly suppresses the accumulation of visceral fat and has antitumor effects in tumor-bearing animals. MCFAs penetrate the mitochondrial membrane in a carnitine shuttle-independent manner and are metabolized more quickly than long-chain fatty acids. Based on these characteristics, MCFAs may have pronounced effects in mitochondria-rich tissues, such as the myocardium. We examined the effect of oral intake of MCFAs on the heart...
November 17, 2018: Lipids in Health and Disease
Oliver J Müller, Markus B Heckmann, Lin Ding, Kleopatra Rapti, Ashraf Y Rangrez, Thomas Gerken, Nicole Christiansen, Ulrike E E Rennefahrt, Henning Witt, Sandra González Maldonado, Philipp Ternes, Dominic M Schwab, Theresa Ruf, Susanne Hille, Anca Remes, Andreas Jungmann, Tanja M Weis, Julia Kreußer, Hermann-Josef Gröne, Johannes Backs, Philipp Schatz, Hugo A Katus, Norbert Frey
Aims: Heart failure is characterized by structural and metabolic cardiac remodelling. The aim of the present study is to expand our understanding of the complex metabolic alterations in the transition from pathological hypertrophy to heart failure and exploit the results from a translational perspective. Methods and results: Mice were subjected to transverse aortic constriction (TAC) or sham surgery and sacrificed 2, 4, or 6 weeks after the procedure. Samples from plasma, liver, skeletal muscle, and heart were collected and analysed using metabolomics...
November 9, 2018: Cardiovascular Research
Lucile Mercadal, Sophie Tezenas du Montcel, Michel B Chonchol, Alain Debure, Hélène Depreneuf, Aude Servais, Nader Bassilios, Ubald Assogba, Mahmoud Allouache, Dominique Prié
BACKGROUND: The use of L-carnitine has been proposed in haemodialysis (HD) when deficiency is present to improve anaemia resistant to erythropoietin stimulating agent, intradialytic hypotension or cardiac failure. We tested the effects of L-carnitine supplementation on parameters of chronic kidney disease-mineral bone disorder. METHODS: CARNIDIAL was a randomized, double-blinded trial having included 92 incident HD subjects for a 1-year period to receive L-carnitine versus placebo...
2018: American Journal of Nephrology
Rasha A Al-Eisa, Fawziah A Al-Salmi, Reham Z Hamza, Nahla S El-Shenawy
Aspartame (ASP) has been used as an alternative to sucrose for diabetics and obese people worldwide. Co-administration of L-carnitine (LC) with ASP has a protective effect against the liver and kidney toxicity induced of ASP. The goal of the investigation was to assess the enhancement of LC effect on the cardiac toxicity caused of ASP. The rats were divided into 6 groups: control with saline, LC (10 mg/kg), ASP (75 mg/kg), ASP (150 mg/kg), LC with 75 mg/kg of ASP, and LC with 150 mg/kg ASP. The antioxidants were determined by measuring the activities of myeloperoxidase, xanthine oxidase, superoxide dismutase, catalase, and glutathione peroxidase, and by assessing the levels of lipid peroxidation, total thiols, and glutathione...
2018: PloS One
Kenji Yamada, Takeshi Taketani
Mitochondrial fatty acid oxidation disorders (FAODs) are caused by defects in β-oxidation enzymes, including very long-chain acyl-CoA dehydrogenase (VLCAD), trifunctional protein (TFP), carnitine palmitoyltransferase-2 (CPT2), carnitine-acylcarnitine translocase (CACT) and others. During prolonged fasting, infection, or exercise, patients with FAODs present with hypoglycemia, rhabdomyolysis, cardiomyopathy, liver dysfunction, and occasionally sudden death. This article describes the diagnosis, newborn screening, and treatment of long-chain FAODs with a focus on VLCAD deficiency...
November 6, 2018: Journal of Human Genetics
Wanise M Souza Cruz, Sheila M S Guimarães, Gabrielle C Maciel, Ana Beatriz A Huguenin, Monicque E M DE Carvalho, Bárbara O Costa, Geisiane A DA Silva, Alexandre S Colafranceschi, Fernanda B Scalco, Marcia Ribeiro
Coronary artery bypass grafting reduces plasma L-carnitine and may impair the production of myocardial energy. L-carnitine supplementation may elevate plasma L-carnitine and increase cardiac mechanical efficiency. The objective of this study was to verify the recovery of preoperative plasma LC in patients with heart failure undergoing coronary artery bypass grafting supplemented with a daily oral dose of 50 mg / kg. Volunteers with ischemic heart failure who underwent surgery were randomized into a supplemented group (A-received 50 mg / kg L-carnitine) or placebo group (B) for 60 days...
July 2018: Anais da Academia Brasileira de Ciências
Stephen Tomlinson, John Atherton, Sandhir Prasad
A 24-year-old female with a diagnosis of primary carnitine deficiency, a rare inherited metabolic disorder predominantly described in the paediatric literature that causes cardiomyopathy, presented for evaluation after three months of nonadherence with prescribed carnitine therapy. Initial echocardiography demonstrated severe left ventricular dilation (104 ml/m2 ) (normal < 76 ml/m2 ) with moderate systolic dysfunction (ejection fraction 40%) and severe right ventricular dilation with mild systolic dysfunction...
2018: Case Reports in Cardiology
Stephanie J Valberg, Sudeep Perumbakkam, Erica C McKenzie, Carrie J Finno
Equine myofibrillar myopathy (MFM) causes exertional muscle pain and is characterized by myofibrillar disarray and ectopic desmin aggregates of unknown origin. To investigate the pathophysiology of MFM, we compared resting and 3 h post-exercise transcriptomes of gluteal muscle and the resting skeletal muscle proteome of MFM and control Arabian horses using RNA-sequencing and iTRAQ analyses. Three hours after exercise, 191 genes were identified as differentially expressed (DE) in MFM vs. control muscle with &gt; 1 log2 fold change [FC] in genes involved in sulfur compound/ cysteine metabolism such as cystathionine-beta-synthase [CBS, ↓4...
October 5, 2018: Physiological Genomics
Sheila Moreira da Silva Guimarães, Wanise Maria de Souza Cruz, Guilherme de Souza Weigert, Fernanda Bertão Scalco, Alexandre Siciliano Colafranceschi, Marcia Gonçalves Ribeiro, Gilson Teles Boaventura
The heart has an intense aerobic metabolism and is among the most metabolically active organs in the body. Its tissue stores fatty acid, the main energetic substrate, and requires high concentrations of plasma L-carnitine. This nutrient is essential in the transport of fatty acids to the mitochondria to generate energy and maintain the proper concentration of coenzyme A free. In decompensated chronic heart failure metabolic changes, associated with inflammation, alter the metabolism of L-carnitine and compromise cardiac energy metabolism...
September 26, 2018: Archives of Medical Research
Bei-Lei Liu, Mian Cheng, Shan Hu, Shun Wang, Le Wang, Zheng-Qing Hu, Cong-Xin Huang, Hong Jiang, Gang Wu
Background: Shensong Yangxin Capsule (SSYX), traditional Chinese medicine, has been used to treat arrhythmias, angina, cardiac remodeling, cardiac fibrosis, and so on, but its effect on cardiac energy metabolism is still not clear. The objective of this study was to investigate the effects of SSYX on myocardium energy metabolism in angiotensin (Ang) II-induced cardiac hypertrophy. Methods: We used 2 μl (10-6 mol/L) AngII to treat neonatal rat cardiomyocytes (NRCMs) for 48 h...
October 5, 2018: Chinese Medical Journal
Karen Lindhardt Madsen, Nicolai Preisler, Jan Rasmussen, Gitte Hedermann, Jess Have Olesen, Allan Meldgaard Lund, John Vissing
Context: Primary carnitine deficiency (PCD) is an inborn error of fatty acid metabolism. Patients with PCD are risk for sudden heart failure upon fasting or illness if they are not treated with daily l-carnitine. Objective: To investigate energy metabolism during exercise in patients with PCD with and without l-carnitine treatment. Design: Interventional study. Setting: Hospital exercise laboratories and department of cardiology...
December 1, 2018: Journal of Clinical Endocrinology and Metabolism
Guoliang Zhao, Haiying Zhang, Yi Wang, Xiaohui Gao, Huaqing Liu, Wei Liu
OBJECTIVE: To investigate the effects of levocarnitine on cardiac function, urinary albumin, high-sensitivity C-reactive protein (hs-CRP), brain natriuretic peptide (BNP) and troponin in patients with coronary heart disease and heart failure. METHODS: 246 patients with CHD-caused HF was selected and randomly divided into Group A and Group B. A fully automatic biochemical analyzer was used to measure the levels of ALB, Hs-CRP, BNP and troponin in two groups of patients, and the expression levels of LVDD and LVEF were detected by cardiac color ultrasonography...
September 6, 2018: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
Olli Kärkkäinen, Tomi Tuomainen, Ville Koistinen, Marjo Tuomainen, Jukka Leppänen, Tuomo Laitinen, Marko Lehtonen, Jaana Rysä, Seppo Auriola, Antti Poso, Pasi Tavi, Kati Hanhineva
Despite epidemiological evidence showing that diets rich in whole grains reduce the risk of chronic life-style related diseases, biological mechanisms for these positive effects are mostly unknown. Increased 5-aminovaleric acid betaine (5-AVAB) levels in plasma and metabolically active tissues such as heart have been associated with consumption of diets rich in whole grains. However, biological effects of 5-AVAB are poorly understood. We evaluated 5-AVAB concentrations in human and mouse heart tissue (3-22 µM and 38-78 µM, respectively) using mass spectrometry...
August 29, 2018: Scientific Reports
Mai Kaneko, Hirotaka Fukasawa, Kento Ishibuchi, Hiroki Niwa, Hideo Yasuda, Ryuichi Furuya
Patients on hemodialysis often have carnitine deficiency. We herein report a woman who experienced the dramatic improvement of cardiac dysfunction after intravenous L-carnitine administration. We also investigated the myocardial fatty acid metabolism using 123 I-labeled β-methyl-p-iodophenyl-pentadecanoic acid (BMIPP) single-photon emission computed tomography (SPECT) before and after L-carnitine therapy, and the impaired metabolism was ameliorated. Taken together, these findings indicate that L-carnitine therapy improved cardiac dysfunction via the amelioration of the abnormal myocardial fatty acid metabolism, at least in part...
August 24, 2018: Internal Medicine
Hong-Liang Kong, Ai-Jie Hou, Ning-Ning Liu, Bo-Han Chen, Sheng-Nan Dai, Hua-Ting Huang
Objectives: This study intended to investigate the effects of Ginsenoside-Rbl (Gs-Rbl) on fatty acid β-oxidation (FAO) in rat failing heart and to identify potential mechanisms of Gs-Rbl improving heart failure (HF) by FAO pathway dependent on AMP-activated protein kinase (AMPK). Materials and Methods: Rats with chronic HF, induced by adriamycin ( Adr ), were randomly grouped into 7 groups. Gs-Rb1, adenine 9-β-D-arabinofuranoside ( Ara A , specific AMPK inhibitor), and 5'-aminoimidazole-4-carboxamide riboside ( Aicar , specific AMPK activator) were administered to rats with HF, singly and/or combinedly...
July 2018: Iranian Journal of Basic Medical Sciences
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