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'carnitine' 'cardiac'

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. PCD patients risk 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...
September 13, 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
Yan-Zhu Pan, Chun-Lan Song, Yan-Jun Guo, Ling-Ling Wang, Ya-Jie Cui, Yi-Fan Ren
OBJECTIVE: To observe the effects of L-carnitine treatment on serum levels of brain natriuretic peptide (BNP) and N-terminal pro-BNP (NT-proBNP) and cardiac function in children with heart dysfunction and severe hand-foot-mouth disease (HFMD). METHODS: A total of 120 children with severe HFMD were enrolled and randomly and equally divided into routine treatment group and L-carnitine treatment group. Thirty healthy children served as the control group. HFMD patients were given anti-fever and antiviral treatment as the basic treatment, while the patients in the L-carnitine treatment group were given L-carnitine as an adjuvant treatment to the basic treatment...
August 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Etedal Abbas Huwait
BACKGROUND: L-carnitine and Vitamin E have antioxidant properties. This study aimed to assess the effectiveness of L-carnitine, Vitamin E and the combination of them in protection against isoproterenol (ISO)-induced biochemical and histopathological changes in rat heart. MATERIAL AND METHODS: Fifty male Wistar rats assigned to 5 groups; control, ISO-treated group (100 mg/kg), ISO+vitamin E-treated group (100 IU/kg), ISO+L-carnitine (100 mg/kg) and ISO+vitamin E+L-carnitine treated group...
August 14, 2018: Folia Morphologica (Warsz)
Keiko Ishigaki, Ikuko Kato, Terumi Murakami, Takatoshi Sato, Minobu Shichiji, Kumiko Ishiguro, Kiyonobu Ishizuka, Makoto Funatsuka, Kayoko Saito, Makiko Osawa, Satoru Nagata
BACKGROUND: The leading cause of death in patients with Fukuyama congenital muscular dystrophy (FCMD) is congestive heart failure or respiratory dysfunction, which is same as that in Duchenne muscular dystrophy (DMD). Recent studies reported that renal dysfunction is a common complication and an increasing cause of death in advanced DMD. It can be attributable to circulatory instability or inappropriate use of drugs for treating cardiac dysfunction. METHODS: We retrospectively evaluated renal function in 38 genetically diagnosed patients with FCMD (range, 1...
August 1, 2018: Brain & Development
Suprovath Kumar Sarker, Md Tarikul Islam, Golam Sarower Bhuyan, Nusrat Sultana, Mst Noorjahan Begum, Mohammad Al Mahmud-Un-Nabi, Md Abdulla Al Noman Howladar, Tashmim Farhana Dipta, A K M Muraduzzaman, Syeda Kashfi Qadri, Tahmina Shirin, Salma Sadiya, Manzoor Hussain, Waqar Ahmed Khan, Sharif Akhteruzzaman, Syed Saleheen Qadri, Firdausi Qadri, Kaiissar Mannoor
Patients with beta-thalassemia major (BTM) suffer from fatigue, poor physical fitness, muscle weakness, lethargy, and cardiac complications which are related to an energy crisis. Carnitine and acylcarnitine derivatives play important roles in fatty acid oxidation, and deregulation of carnitine and acylcarnitine metabolism may lead to an energy crisis. The present study aimed to investigate carnitine and acylcarnitine metabolites to gain an insight into the pathophysiology of BTM. Dried blood spots of 45 patients with BTM and 96 age-matched healthy controls were analyzed for free carnitine and 24 acylcarnitines by using liquid chromatography-tandem mass spectrometry (LC-MS/MS)...
July 2018: Journal of Advanced Research
Terumi Higuchi
BACKGROUND: Carnitine deficiency is a common condition in hemodialysis patients. There have been numerous reports on the efficacy of levocarnitine therapy in hemodialysis patients, including different views. Reported effects of levocarnitine are: (1) improvement of renal anemia, (2) improvement of cardiac functions, (3) effects on muscle spasm and asthenia, (4) anti-atherogenic effects, (5) anti-oxidant and anti-inflammatory effects, and (6) inhibitory effects on dialysis hypotension...
2018: Contributions to Nephrology
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