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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/28324172/enzymes-involved-in-branched-chain-amino-acid-metabolism-in-humans
#1
REVIEW
María M Adeva-Andany, Laura López-Maside, Cristóbal Donapetry-García, Carlos Fernández-Fernández, Cristina Sixto-Leal
Branched-chain amino acids (leucine, isoleucine and valine) are structurally related to branched-chain fatty acids. Leucine is 2-amino-4-methyl-pentanoic acid, isoleucine is 2-amino-3-methyl-pentanoic acid, and valine is 2-amino-3-methyl-butanoic acid. Similar to fatty acid oxidation, leucine and isoleucine produce acetyl-coA. Additionally, leucine generates acetoacetate and isoleucine yields propionyl-coA. Valine oxidation produces propionyl-coA, which is converted into methylmalonyl-coA and succinyl-coA. Branched-chain aminotransferase catalyzes the first reaction in the catabolic pathway of branched-chain amino acids, a reversible transamination that converts branched-chain amino acids into branched-chain ketoacids...
March 21, 2017: Amino Acids
https://www.readbyqxmd.com/read/28322157/diabetes-related-neurological-implications-and-pharmacogenomics
#2
Rojas Carranza Camilo Andrés, Bustos Cruz Rosa Helena, Pino Pinzón Carmen Juliana, Ariza Marquez Yeimy Viviana, Gómez Bello Rosa Margarita, Cañadas Garre Marisa
Diabetes mellitus (DM) is the most commonly occurring cause of neuropathy around the world and is beginning to grow in countries where there is a risk of obesity. DM Type II, (T2DM) is a common age-related disease and is a major health concern, particularly in developed countries in Europe where the population is aging. T2DM is a chronic disease which is characterised by hyperglycemia, hyperinsulinemia and insulin resistance, together with the body's inability to use glucose as energy. Such metabolic disorder produces a chronic inflammatory state, as well as changes in lipid metabolism leading to hypertriglyceridemia, thereby producing chronic deterioration of the organs and premature morbidity and mortality...
March 17, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28314621/docosahexaenoic-acid-dha-an-essential-nutrient-and-a-nutraceutical-for-brain-health-and-diseases
#3
REVIEW
Grace Y Sun, Agnes Simonyi, Kevin L Fritsche, Dennis Y Chuang, Mark Hannink, Zezong Gu, C Michael Greenlief, Jeffrey K Yao, James C Lee, David Q Beversdorf
Docosahexaenoic acid (DHA), a polyunsaturated fatty acid (PUFA) enriched in phospholipids in the brain and retina, is known to play multi-functional roles in brain health and diseases. While arachidonic acid (AA) is released from membrane phospholipids by cytosolic phospholipase A2 (cPLA2), DHA is linked to action of the Ca(2+)-independent iPLA2. DHA undergoes enzymatic conversion by 15-lipoxygenase (Alox 15) to form oxylipins including resolvins and neuroprotectins, which are powerful lipid mediators. DHA can also undergo non-enzymatic conversion by reacting with oxygen free radicals (ROS), which cause the production of 4-hydoxyhexenal (4-HHE), an aldehyde derivative which can form adducts with DNA, proteins and lipids...
March 10, 2017: Prostaglandins, Leukotrienes, and Essential Fatty Acids
https://www.readbyqxmd.com/read/28292183/n-docosahexaenoyl-dopamine-an-endocannabinoid-like-conjugate-of-dopamine-and-the-n-3-fatty-acid-docosahexaenoic-acid-attenuates-lipopolysaccharide-induced-activation-of-microglia-and-macrophages-via-cox-2
#4
Ya Wang, Pierluigi Plastina, Jean-Paul Vincken, Renate Jansen, Michiel Balvers, Jean Paul Ten Klooster, Harry Gruppen, Renger Witkamp, Jocelijn Meijerink
Several studies indicate that the n-3 long-chain polyunsaturated fatty acid docosahexaenoic acid (DHA) contributes to an attenuated inflammatory status in the development of neurodegenerative disorders, such as Alzheimer's and Parkinson's disease. To explain these effects, different mechanisms are being proposed, including those involving endocannabinoids and related signaling molecules. Many of these compounds belong to the fatty acid amides, conjugates of fatty acids with biogenic amines. Conjugates of DHA with ethanolamine or serotonin have previously been shown to possess anti-inflammatory and potentially neuroprotective properties...
March 15, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/28283530/identification-of-enzymes-involved-in-oxidation-of-phenylbutyrate
#5
Neža Palir, Jos P N Ruiter, Ronald J A Wanders, Riekelt H Houtkooper
In recent years the short-chain fatty acid 4-phenylbutyrate (PB) has emerged as a promising drug for various clinical conditions. In fact, PB is FDA-approved for urea cycle disorders since 1996. PB is more potent and less toxic than its metabolite phenylacetate (PA), and not just a pro-drug for PA as it was initially assumed. The metabolic pathway of PB, however, has remained unclear. Therefore, we set out to identify the enzymes involved in the β-oxidation of PB. We used cells deficient in specific steps of fatty acid β-oxidation, and used ultra-high performance liquid chromatography (UHPLC) to measure to which enzymes are able to convert PB or its downstream products...
March 9, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28259037/altered-soluble-epoxide-hydrolase-derived-oxylipins-in-patients-with-seasonal-major-depression-an-exploratory-study
#6
Marie Hennebelle, Yurika Otoki, Jun Yang, Bruce D Hammock, Anthony J Levitt, Ameer Y Taha, Walter Swardfager
Many cytochrome p450-derived lipids promote resolution of inflammation, in contrast to their soluble epoxide hydrolase(sEH)-derived oxylipin breakdown products. Here we compare plasma oxylipins and precursor fatty acids between seasons in participants with major depressive disorder with seasonal pattern (MDD-s). Euthymic participants with a history of MDD-s recruited in summer-fall were followed-up in winter. At both visits, a structured clinical interview (DSM-5 criteria) and the Beck Depression Inventory II (BDI-II) were administered...
February 27, 2017: Psychiatry Research
https://www.readbyqxmd.com/read/28248536/cardiovascular-diseases-depression-disorders-and-potential-effects-of-omega-3-fatty-acids
#7
J Trebatická, A Dukát, Z Ďuračková, J Muchová
Cardiovascular disease (CVD) and depressive disorders (DD) are two of the most prevalent health problems in the world. Although CVD and depression have different origin, they share some common pathophysiological characteristics and risk factors, such as the increased production of proinflammatory cytokines, endothelial dysfunction, blood flow abnormalities, decreased glucose metabolism, elevated plasma homocysteine levels, oxidative stress and disorder in vitamin D metabolism. Current findings confirm the common underlying factors for both pathologies, which are related to dramatic dietary changes in the mid-19th century...
February 28, 2017: Physiological Research
https://www.readbyqxmd.com/read/28247148/very-long-chain-acyl-coa-dehydrogenase-vlcad-deficiency-studies-on-treatment-effects-and-long-term-outcomes-in-mouse-models
#8
REVIEW
Sara Tucci
Very-long-chain-acyl-CoA-dehydrogenase deficiency is the most common disorder of mitochondrial long-chain fatty acid (LCFA) oxidation, with an incidence of 1:50,000-1:100,000 in newborns. Catabolic situations contribute to the aggravation of symptoms and induce severe metabolic derangement. Treatment for VLCAD-deficiency includes avoidance of fasting and a long-chain fat-restricted and fat-modified diet in which LCFAs are fully or partially replaced by medium-chain triglycerides (MCT). The aim of this work was to investigate the outcome and the effects of long-term treatment in a mouse model of VLCAD-deficiency...
February 28, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28242381/antidiabetic-effect-of-sn158-through-ppar%C3%AE-%C3%AE-dual-activation-in-ob-ob-mice
#9
Yujung Jung, Yongkai Cao, Suresh Paudel, Goo Yoon, Seung Hoon Cheon, Gyu-Un Bae, Li Tai Jin, Yong Kee Kim, Su-Nam Kim
In this study, we aimed to demonstrate the antidiabetic potential of (E)-N-(4-(3-(5-bromo-4-hydroxy-2-methoxyphenyl)acryloyl) phenyl)-4-tert-butylbenzamide (SN158) through peroxisome proliferator-activated receptor (PPAR)-α/γ dual activation. SN158 interacted with both PPARα and PPARγ, and increased their transcriptional activities. Simultaneously, SN158 treatment led to an increase in adipogenic differentiation of 3T3-L1 preadipocytes and fatty acid oxidation in hepatocytes. In addition, glucose uptake in myotubes was significantly increased by SN158 treatment...
February 24, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28241817/berberine-improves-glucogenesis-and-lipid-metabolism-in-nonalcoholic-fatty-liver-disease
#10
Li Zhao, Zhen Cang, Honglin Sun, Xiaomin Nie, Ningjian Wang, Yingli Lu
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is considered a critical hepatic manifestation of metabolic syndrome. Berberine (BBR) exerts anti-hyperglycemic and anti-dyslipidemic effects and can also ameliorate NAFLD. Thus, BBR might exert its therapeutic effect on NAFLD by improving glucolipid metabolism. Here, we investigated the aspects and extent to which glucolipid metabolism were affected by BBR in rats with NAFLD. METHODS: Three groups of Sprague-Dawley rats were studied: a control group (n = 6) fed a normal chow diet and a NAFLD group (n = 6) and a NAFLD + BBR group (n = 6) fed a high-fat diet...
February 28, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28239368/connection-of-nicotine-to-diet-induced-obesity-and-non-alcoholic-fatty-liver-disease-cellular-and-mechanistic-insights
#11
REVIEW
Amiya P Sinha-Hikim, Indrani Sinha-Hikim, Theodore C Friedman
Non-alcoholic fatty liver disease (NAFLD) poses a serious health hazard affecting 20-40% of adults in the general population in the USA and over 70% of the obese and extremely obese people. In addition to obesity, nicotine is recognized as a risk factor for NAFLD, and it has been reported that nicotine can exaggerate obesity-induced hepatic steatosis. The development of NAFLD has serious clinical complications because of its potential progression from simple hepatic steatosis to non-alcoholic steatohepatitis (NASH), liver cirrhosis, and hepatocellular carcinoma...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28218306/plin5-alleviates-myocardial-ischaemia-reperfusion-injury-by-reducing-oxidative-stress-through-inhibiting-the-lipolysis-of-lipid-droplets
#12
Pengfei Zheng, Zhonglin Xie, Yuan Yuan, Wen Sui, Chao Wang, Xing Gao, Yuanlin Zhao, Feng Zhang, Yu Gu, Peizhen Hu, Jing Ye, Xuyang Feng, Lijun Zhang
Myocardial ischaemia-reperfusion (I/R) injury is a complex pathophysiological process. Current research has suggested that energy metabolism disorders, of which the abnormal consumption of fatty acids is closely related, compose the main pathological basis for myocardial I/R injury. Lipid droplets (LD) are critical regulators of lipid metabolism by LD-associated proteins. Among the lipid droplet proteins, the perilipin family members regulate lipolysis and lipogenesis through different mechanisms. Plin5, an important perilipin protein, promotes LD generation and lowers fatty acid oxidation, thus protecting the myocardium from lipotoxicity...
February 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28216439/role-of-thyroid-hormone-in-hepatic-gene-regulation-chromatin-remodeling-and-autophagy
#13
Brijesh Kumar Singh, Rohit Anthony Sinha, Kenji Ohba, Paul Michael Yen
Thyroid hormone (TH) actions on development and metabolism have been studied ever since the discovery of thyroxine almost a century ago. Initial studies focused on the physiological and biochemical actions of TH. Later, the cloning of the thyroid hormone receptor (THR) isoforms and the development of techniques enabled the study of TH regulation of complex cellular processes (such as gene transcription). Recently we found that TH activates secondary transcription factors such as FOXO1, to amplify gene transcription; and also is a potent inducer of autophagy that was critical for fatty acid β-oxidation in the liver...
February 16, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28214879/thiamine-deprivation-produces-a-liver-atp-deficit-and-metabolic-and-genomic-effects-in-mice-findings-are-parallel-to-those-of-biotin-deficiency-and-have-implications-for-energy-disorders
#14
Alain de J Hernandez-Vazquez, Josue Andres Garcia-Sanchez, Elizabeth Moreno-Arriola, Ana Salvador-Adriano, Daniel Ortega-Cuellar, Antonio Velazquez-Arellano
Thiamine is one of several essential cofactors for ATP generation. Its deficiency, like in beriberi and in the Wernicke-Korsakoff syndrome, has been studied for many decades. However, its mechanism of action is still not completely understood at the cellular and molecular levels. Since it acts as a coenzyme for dehydrogenases of pyruvate, branched-chain keto acids, and ketoglutarate, its nutritional privation is partly a phenocopy of inborn errors of metabolism, among them maple syrup urine disease. In the present paper, we report metabolic and genomic findings in mice deprived of thiamine...
February 18, 2017: Journal of Nutrigenetics and Nutrigenomics
https://www.readbyqxmd.com/read/28212715/novel-biomarkers-of-heart-failure
#15
A Savic-Radojevic, M Pljesa-Ercegovac, M Matic, D Simic, S Radovanovic, T Simic
Although substantial improvements have been made in majority of cardiac disorders, heart failure (HF) remains a major health problem, with both increasing incidence and prevalence over the past decades. For that reason, the number of potential biomarkers that could contribute to diagnosis and treatment of HF patients is, almost exponentially, increasing over the recent years. The biomarkers that are, at the moment, more or less ready for use in everyday clinical practice, reflect different pathophysiological processes present in HF...
2017: Advances in Clinical Chemistry
https://www.readbyqxmd.com/read/28212710/ctrp1-in-liver-disease
#16
P Shabani, S Emamgholipour, M Doosti
Nonalcoholic fatty liver disease (NAFLD) is a chronic liver disease occurs in significant percentage of general population. NAFLD is closely associated with entire spectrum of metabolic-related disorders including diabetes, obesity, and cardiovascular diseases. Considering several similar pathways underpinning metabolic disorders, presence of common molecular mediators contributing to pathomechanism of these disorders is expected. Mounting evidence has demonstrated important role of adipokines in the context of NAFLD...
2017: Advances in Clinical Chemistry
https://www.readbyqxmd.com/read/28157411/novel-lipidomic-biomarkers-in-hyperlipidemia-and-cardiovascular-diseases-an-integrative-biology-analysis
#17
Sneha Rai, Sonika Bhatnagar
Lipidomics is a new frontier of omics research and offers much promise for new-generation biomarkers for common complex phenotypes such as hyperlipidemia (HL) and cardiovascular diseases (CVDs). HL is a disorder characterized by increased levels of blood lipids and is a well-established risk factor for CVD. Traditional clinical markers for prognosis of hyperlipidemic individuals are inadequate to forecast or diagnose cardiac events. In this expert review, lipidomic profiles from recent HL and CVD studies were compared with the normolipidemic profile prepared from the Standard Reference Material...
March 2017: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/28139281/non-alcoholic-fatty-liver-disease-and-its-treatment-with-n-3-polyunsaturated-fatty-acids
#18
REVIEW
Gabriela S de Castro, Philip C Calder
BACKGROUND & AIMS: Non-alcoholic fatty liver disease (NAFLD) is a common liver disease in Western countries. Metabolic disorders which are increasing in prevalence, such as dyslipidaemias, obesity and type 2 diabetes, are closely related to NAFLD. Insulin resistance is a prominent risk factor for NAFLD. Marine omega-3 (n-3) polyunsaturated fatty acids (PUFAs) are able to decrease plasma triacylglycerol and diets rich in marine n-3 PUFAs are associated with a lower cardiovascular risk...
January 19, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28132977/mitochondrial-trifunctional-protein-deficiency-an-adult-patient-with-similar-progress-to-charcot-marie-tooth-disease
#19
Yuki Yamamoto, Naoko Matsui, Yu Hiramatsu, Yoshimichi Miyazaki, Hiroyuki Nodera, Yuishin Izumi, Hiroshi Takashima, Ryuji Kaji
A 45-year-old man presented to us due to slowly progressive muscle weakness and sensory disturbances in his lower limbs since his 40's. He reported multiple episodes of exercise-induced severe muscle fatigue and brown urine in his childhood, which disappeared by age 20. A nerve conduction study showed peripheral axonal neuropathy and then Charcot-Marie-Tooth disease (CMT) was considered as the most likely diagnosis; however, exome sequencing failed to identify a mutation in the known genes of CMTs. Since age 55, he recurrently developed severe rhabdomyolysis that required hospitalization...
February 25, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28129657/physiological-characterization-of-a-novel-ppar-pan-agonist-2-4-5-6-methylenedioxybenzo-d-thiazol-2-yl-2-methylphenoxy-2-methylpropanoic-acid-mhy2013
#20
Hye Jin An, Bonggi Lee, Dae Hyun Kim, Eun Kyeong Lee, Ki Wung Chung, Min Hi Park, Hyoung Oh Jeong, Sung Min Kim, Kyoung Mi Moon, Ye Ra Kim, Seong Jin Kim, Hwi Young Yun, Pusoon Chun, Byung Pal Yu, Hyung Ryong Moon, Hae Young Chung
Recently, agonists targeting multiple peroxisome proliferator-activated receptors (PPARs) have been developed to improve metabolic disorders and minimize the side effects of selective PPAR agonists such as weight gain and dyslipidemia. We newly synthesized six 2-methyl-2-(o-tolyloxy)propanoic acid derivatives based on the structure of a well-known PPAR pan agonist, bezafibrate. Of six compounds, MHY2013 was screened as the strongest activator of three PPAR subtypes based on protein docking simulation and luciferase assays...
January 25, 2017: Oncotarget
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