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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/28811612/coenzyme-q10-improves-lipid-metabolism-and-ameliorates-obesity-by-regulating-camkii-mediated-pde4-inhibition
#1
Zhe Xu, Jia Huo, Xin Ding, Mu Yang, Lin Li, Jian Dai, Kazunori Hosoe, Hiroshi Kubo, Masayuki Mori, Keiichi Higuchi, Jinko Sawashita
Our recent studies revealed that supplementation with the reduced form of coenzyme Q10 (CoQ10H2) inhibits oxidative stress and slows the process of aging in senescence-accelerated mice. CoQ10H2 inhibits adipocyte differentiation and regulates lipid metabolism. In the present study, we show that dietary supplementation with CoQ10H2 significantly reduced white adipose tissue content and improved the function of brown adipose tissue by regulating expression of lipid metabolism-related factors in KKAy mice, a model of obesity and type 2 diabetes...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28809727/models-of-non-alcoholic-fatty-liver-disease-and-potential-translational-value-the-effects-of-3-5-l-diiodothyronine
#2
Elena Grasselli, Laura Canesi, Piero Portincasa, Adriana Voci, Laura Vergani, Ilaria Demori
Non-alcoholic fatty liver disease (NAFLD) is the most common liver disorder in industrialized countries and is associated with increased risk of cardiovascular, hepatic and metabolic diseases. Molecular mechanisms on the root of the disrupted lipid homeostasis in NAFLD and potential therapeutic strategies can benefit of in vivo and in vitro experimental models of fatty liver. Here, we describe the high fat diet (HFD)-fed rat in vivo model, and two in vitro models, the primary cultured rat fatty hepatocytes or the FaO rat hepatoma fatty cells, mimicking human NAFLD...
August 8, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28808839/individuals-with-autism-have-higher-8-iso-pgf2%C3%AE-levels-than-controls-but-no-correlation-with-quantitative-assay-of-paraoxonase-1-serum-levels
#3
Bianca Pop, Alexandru-Ștefan Niculae, Tudor Lucian Pop, Andreea Liana Răchișan
Autism spectrum disorder (ASD) represents a very large set of neurodevelopmental issues with diverse clinical outcomes. Various hypotheses have been put forth for the etiology of autism spectrum disorder, including issues pertaining to oxidative stress. In this study, we conducted measurements of serum 8-Iso-Prostaglanding F2 α (8-iso-PGF2α, which is the results of non-enzimatically mediated polyunsaturated fatty acid oxidation) in a population of individuals with autism and a control group of age and sex matched controls...
August 14, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28801073/newborn-screening-for-carnitine-palmitoyltransferase-ii-deficiency-using-c16-c18-1-c2-evaluation-of-additional-indices-for-adequate-sensitivity-and-lower-false-positivity
#4
Go Tajima, Keiichi Hara, Miyuki Tsumura, Reiko Kagawa, Satoshi Okada, Nobuo Sakura, Shinsuke Maruyama, Atsuko Noguchi, Tomonari Awaya, Mika Ishige, Nobuyuki Ishige, Ikuma Musha, Sayaka Ajihara, Akira Ohtake, Etsuo Naito, Yusuke Hamada, Tomotaka Kono, Tomoko Asada, Hideo Sasai, Toshiyuki Fukao, Ryoji Fujiki, Osamu Ohara, Ryosuke Bo, Kenji Yamada, Hironori Kobayashi, Yuki Hasegawa, Seiji Yamaguchi, Masaki Takayanagi, Ikue Hata, Yosuke Shigematsu, Masao Kobayashi
BACKGROUND: Carnitine palmitoyltransferase (CPT) II deficiency is one of the most common forms of mitochondrial fatty acid oxidation disorder (FAOD). However, newborn screening (NBS) for this potentially fatal disease has not been established partly because reliable indices are not available. METHODS: We diagnosed CPT II deficiency in a 7-month-old boy presenting with hypoglycemic encephalopathy, which apparently had been missed in the NBS using C16 and C18:1 concentrations as indices...
July 31, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28792522/biochemical-characterization-and-anti-inflammatory-properties-of-an-isothiocyanate-enriched-moringa-moringa-oleifera-seed-extract
#5
Asha Jaja-Chimedza, Brittany L Graf, Charlotte Simmler, Youjin Kim, Peter Kuhn, Guido F Pauli, Ilya Raskin
Moringa oleifera Lam. is a tropical plant, used for centuries as food and traditional medicine. The aim of this study was to develop, validate and biochemically characterize an isothiocyanate-enriched moringa seed extract (MSE), and to compare the anti-inflammatory effects of MSE-containing moringa isothiocyanate-1 (MIC-1) with a curcuminoid-enriched turmeric extract (CTE), and a material further enriched in its primary phytochemical, curcumin (curcumin-enriched material; CEM). MSE was prepared by incubating ground moringa seeds with water to allow myrosinase-catalyzed enzymatic formation of bioactive MIC-1, the predominant isothiocyanate in moringa seeds...
2017: PloS One
https://www.readbyqxmd.com/read/28790228/association-between-serum-uric-acid-and-non-alcoholic-fatty-liver-disease-a-meta-analysis
#6
Guntur Darmawan, Laniyati Hamijoyo, Irsan Hasan
BACKGROUND: non-alcoholic fatty liver disease (NAFLD) is known to be associated with some metabolic disorders. Recent studies suggested the role of uric acid in NAFLD through oxidative stress and inflammatory process. This study is aimed to evaluate the association between serum uric acid and NAFLD. METHODS: a systematic literature review was conducted using Pubmed and Cochrane library. The quality of all studies was assessed using the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE)...
April 2017: Acta Medica Indonesiana
https://www.readbyqxmd.com/read/28789919/identification-of-long-and-very-long-chain-fatty-acids-plasmalogen-c16-0-and-phytanic-acid-as-new-lipid-biomarkers-in-tunisian-coronary-artery-disease-patients
#7
Samia Hadj Ahmed, Nadia Koubaa, Wafa Kharroubi, Amira Zarrouk, Amira Mnari, Fethi Batbout, Habib Gamra, Sonia Hammami, Gérard Lizard, Mohamed Hammami
Long and very long chain fatty acids (LCFAs and VLCFAs) may play an active role in coronary artery diseases (CAD) etiology. Our aim was to evaluate the associations between LCPUFAs (C20:4n-6; C20:5n-3 and C22:6n-3) and VLCSFAs (C22:0, C24:0; and C26:0), as well as markers of peroxisomal integrity evaluated by phytanic acid and plasmalogen-C16:0 (PL-C16:0) in addition to the markers of lipid peroxidation (malondialdehyde [MDA] and conjugated dienes [CD]) and inflammation (high sensitivity C-reactive protein [hs-CRP]) with vascular severity evaluated by Gensini score in order to determine their possible effects on CAD in Tunisian population...
August 5, 2017: Prostaglandins & Other Lipid Mediators
https://www.readbyqxmd.com/read/28777310/anti-atherosclerotic-action-of-agmatine-in-apoe-knockout-mice
#8
Anna Wiśniewska, Rafał Olszanecki, Justyna Totoń-Żurańska, Katarzyna Kuś, Aneta Stachowicz, Maciej Suski, Anna Gębska, Mariusz Gajda, Jacek Jawień, Ryszard Korbut
Atherosclerosis is an inflammatory disease in which dysfunction of mitochondria play an important role, and disorders of lipid management intensify this process. Agmatine, an endogenous polyamine formed by decarboxylation of arginine, exerts a protective effect on mitochondria and modulates fatty acid metabolism. We investigated the effect of exogenous agmatine on the development of atherosclerosis and changes in lipid profile in apolipoprotein E knockout (apoE-/-) mice. Agmatine caused an approximate 40% decrease of atherosclerotic lesions, as estimated by en face and cross-section methods with an influence on macrophage but not on smooth muscle content in the plaques...
August 4, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28770317/mitochondrial-h-atp-synthase-in-human-skeletal-muscle-contribution-to-dyslipidaemia-and-insulin-resistance
#9
Laura Formentini, Alexander J Ryan, Manuel Gálvez-Santisteban, Leslie Carter, Pam Taub, John D Lapek, David J Gonzalez, Francisco Villarreal, Theodore P Ciaraldi, José M Cuezva, Robert R Henry
AIMS/HYPOTHESIS: Mitochondria are important regulators of the metabolic phenotype in type 2 diabetes. A key factor in mitochondrial physiology is the H(+)-ATP synthase. The expression and activity of its physiological inhibitor, ATPase inhibitory factor 1 (IF1), controls tissue homeostasis, metabolic reprogramming and signalling. We aimed to characterise the putative role of IF1 in mediating skeletal muscle metabolism in obesity and diabetes. METHODS: We examined the 'mitochondrial signature' of obesity and type 2 diabetes in a cohort of 100 metabolically characterised human skeletal muscle biopsy samples...
August 2, 2017: Diabetologia
https://www.readbyqxmd.com/read/28753542/mri-in-medium-chain-acyl-coenzyme-a-dehydrogenase-deficiency-neuroimaging-during-the-first-month
#10
Lorenzo Figà Talamanca, Luca Pasquini, Antonio Napolitano, Daniela Longo
BACKGROUND: Medium-chain acyl-coenzyme A dehydrogenase (MCAD) is the most common genetic disorder of fatty acid oxidation, which presents before the age of 2 with the onset of acute hypoketotic hypoglycemia, and is typically precipitated by stress. CASE PRESENTATION: We report serial brain magnetic resonance imaging (MRI) changes, including MR spectroscopy (MRS) and diffusion weighted imaging (DWI), in a patient with a classical MCAD presentation, compared with five healthy controls...
August 28, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28752050/nrg4-promotes-fuel-oxidation-and-a-healthy-adipokine-profile-to-ameliorate-diet-induced-metabolic-disorders
#11
Zhimin Chen, Guo-Xiao Wang, Sara L Ma, Dae Young Jung, Hyekyung Ha, Tariq Altamimi, Xu-Yun Zhao, Liang Guo, Peng Zhang, Chun-Rui Hu, Ji-Xin Cheng, Gary D Lopaschuk, Jason K Kim, Jiandie D Lin
OBJECTIVE: Brown and white adipose tissue exerts pleiotropic effects on systemic energy metabolism in part by releasing endocrine factors. Neuregulin 4 (Nrg4) was recently identified as a brown fat-enriched secreted factor that ameliorates diet-induced metabolic disorders, including insulin resistance and hepatic steatosis. However, the physiological mechanisms through which Nrg4 regulates energy balance and glucose and lipid metabolism remain incompletely understood. The aims of the current study were: i) to investigate the regulation of adipose Nrg4 expression during obesity and the physiological signals involved, ii) to elucidate the mechanisms underlying Nrg4 regulation of energy balance and glucose and lipid metabolism, and iii) to explore whether Nrg4 regulates adipose tissue secretome gene expression and adipokine secretion...
August 2017: Molecular Metabolism
https://www.readbyqxmd.com/read/28751722/hepatocyte-specific-timp3-expression-prevents-diet-dependent-fatty-liver-disease-and-hepatocellular-carcinoma
#12
Viviana Casagrande, Alessandro Mauriello, Simone Bischetti, Maria Mavilio, Massimo Federici, Rossella Menghini
Non-alcoholic fatty liver disease (NAFLD) encompasses a broad spectrum of conditions, ranging from non-progressive bland steatosis to hepatocarcinoma. Tissue inhibitor of metalloproteinase 3 (Timp3) has a role in the pathogenesis of fatty liver disease associated with obesity and is silenced during metabolic disorders and liver cancer. We generated an hepatocyte-specific TIMP3 'gain-of-function' mouse model under the control of the Albumin promoter (AlbT3) and investigated its effects during high-fat diet (HFD)...
July 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28748224/utility-of-genetic-testing-for-confirmation-of-abnormal-newborn-screening-in-disorders-of-long-chain-fatty-acids-a-missed-case-of-carnitine-palmitoyltransferase-1a-cpt1a-deficiency
#13
Leah Dowsett, Lauren Lulis, Can Ficicioglu, Sanmati Cuddapah
An 18-month-old male was evaluated after presenting with disproportionately elevated liver transaminases in the setting of acute gastroenteritis. He had marked hepatomegaly on physical exam that was later confirmed with an abdominal ultrasound. Given this clinical picture, suspicion for a fatty acid oxidation disorder was raised. Further investigation revealed that his initial newborn screen was positive for carnitine palmitoyltransferase 1A (CPT1A) deficiency-a rare autosomal recessive disorder of long-chain fatty acid oxidation...
June 2017: International Journal of Neonatal Screening
https://www.readbyqxmd.com/read/28737815/isofraxidin-a-coumarin-component-improves-high-fat-diet-induced-hepatic-lipid-homeostasis-disorder-and-macrophage-inflammation-in-mice
#14
Jian Li, Xiaofei Li, Zhike Li, Lu Zhang, Yonggang Liu, Hong Ding, Shanye Yin
Isofraxidin (IF) is a coumarin compound produced in the functional foods Siberian ginseng and Apium graveolens. The first objective of this study was to investigate the protective effects and putative methods of IF in combating lipotoxicity in vitro and in vivo. Oleic acid was used to induce lipid turbulence in human hepatoma cells (HepG2). Alterations in triglyceride metabolism, inflammation and oxidative status were monitored. Results show that IF mainly reduced triglyceride accumulation, TNF-α release and ROS activation in metabolic disordered cells...
August 1, 2017: Food & Function
https://www.readbyqxmd.com/read/28736181/succinate-accumulation-impairs-cardiac-pyruvate-dehydrogenase-activity-through-grp91-dependent-and-independent-signaling-pathways-therapeutic-effects-of-ginsenoside-rb1
#15
Jia Li, Yi-Lin Yang, Lan-Zhu Li, Lei Zhang, Qun Liu, Kang Liu, Ping Li, Baolin Liu, Lian-Wen Qi
Altered mitochondrial oxidation increases vulnerability to cardiac ischemia/reperfusion (I/R) injury in metabolic disorders. However, the metabolic signaling responsible for the dysfunction remains partly unknown. We sought to test whether or not hypoxic succinate accumulation could inhibit pyruvate dehydrogenase (PDH) activity and subsequently aggravate I/R injury. Results showed that saturated fatty acid palmitate stimulation increased fatty acid oxidation and induced hypoxia in cardiomyocytes, leading to succinate accumulation...
July 20, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28727970/severe-fat-accumulation-in-multiple-organs-in-pediatric-autopsies-an-uncommon-but-significant-finding
#16
Jeannette C Bleeker, Gepke Visser, Frits A Wijburg, Sacha Ferdinandusse, Hans R Waterham, Peter G J Nikkels
Background The observation of fat accumulation in different organs at pediatric autopsy may help determine the cause of death. However, a comprehensive study on fat accumulation and related etiologies is still lacking. Aim To investigate the incidence of fat accumulation in different organs in pediatric autopsies and their relevance to the cause of death. Methods From February 2004 until March 2015, fat accumulation was assessed in 561 routinely performed pediatric autopsies in our center. Age at death ranged from 14 weeks of gestation for fetuses to 16 years...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28722145/dendrobium-nobile-lindl-alkaloids-regulate-metabolism-gene-expression-in-livers-of-mice
#17
Yun-Yan Xu, Ya-Sha Xu, Yuan Wang, Qin Wu, Yuan-Fu Lu, Jie Liu, Jing-Shan Shi
OBJECTIVES: In our previous studies, Dendrobium nobile Lindl. alkaloids (DNLA) has been shown to have glucose-lowering and antihyperlipidaemia effects in diabetic rats, in rats fed with high-fat diets, and in mice challenged with adrenaline. This study aimed to examine the effects of DNLA on the expression of glucose and lipid metabolism genes in livers of mice. METHODS: Mice were given DNLA at doses of 10-80 mg/kg, po for 8 days, and livers were removed for total RNA and protein isolation to perform real-time RT-PCR and Western blot analysis...
July 19, 2017: Journal of Pharmacy and Pharmacology
https://www.readbyqxmd.com/read/28720584/protein-kinase-n2-regulates-amp-kinase-signaling-and-insulin-responsiveness-of-glucose-metabolism-in-skeletal-muscle
#18
Maxwell A Ruby, Isabelle Riedl, Julie Massart, Marcus Åhlin, Juleen R Zierath
Insulin resistance is central to the development of type 2 diabetes and related metabolic disorders. As skeletal muscle is responsible for the majority of whole body insulin-stimulated glucose uptake, regulation of glucose metabolism in this tissue is of particular importance. While Rho GTPases and many of their affecters influence skeletal muscle metabolism, there is a paucity of information on the protein kinase N (PKN) family of serine/threonine protein kinases. We investigated the impact of PKN2 on insulin signaling and glucose metabolism in primary human skeletal muscle cells in vitro and mouse tibialis anterior muscle in vivo...
July 18, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28713383/the-toxicological-mechanisms-of-environmental-soot-black-carbon-and-carbon-black-focus-on-oxidative-stress-and-inflammatory-pathways
#19
REVIEW
Rituraj Niranjan, Ashwani Kumar Thakur
The environmental soot and carbon blacks (CBs) cause many diseases in humans, but their underlying mechanisms of toxicity are still poorly understood. Both are formed after the incomplete combustion of hydrocarbons but differ in their constituents and percent carbon contents. For the first time, "Sir Percival Pott" described soot as a carcinogen, which was subsequently confirmed by many others. The existing data suggest three main types of diseases due to soot and CB exposures: cancer, respiratory diseases, and cardiovascular dysfunctions...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28712316/disturbed-bovine-mitochondrial-lipid-metabolism-a-review
#20
J H van der Kolk, J J Gross, V Gerber, R M Bruckmaier
In mammals, excess energy is stored primarily as triglycerides, which are mobilized when energy demands arise and cannot be covered by feed intake. This review mainly focuses on the role of long chain fatty acids in disturbed energy metabolism of the bovine species. Long chain fatty acids regulate energy metabolism as ligands of peroxisome proliferator-activated receptors. β-Oxidation is the process by which fatty acid molecules are broken down in the mitochondria or peroxisomes to generate acetyl-CoA. Carnitine acts as a carrier of fatty acyl groups as long-chain acyl-CoA derivatives do not penetrate the mitochondrial inner membrane...
July 17, 2017: Veterinary Quarterly
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