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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/28447390/high-non-esterified-fatty-acid-concentrations-promote-expression-and-secretion-of-fibroblast-growth-factor-21-in-calf-hepatocytes-cultured-in-vitro
#1
J G Wang, Y Z Guo, Y Z Kong, S Dai, B Y Zhao
Negative energy balance is considered as the pathological basis of energy metabolic disorders in periparturient dairy cows. Serum non-esterified fatty acids (NEFA) are one of the most important indicators of energy balance status. Fibroblast growth factor 21 (FGF21) has been identified as a hepatokine involved in regulation of metabolic adaptations, such as promoting hepatic lipid oxidation and ketogenesis, during energy deprivation. However, the direct effects of NEFA on FGF21 expression and secretion in bovine hepatocytes are not entirely clear...
April 26, 2017: Journal of Animal Physiology and Animal Nutrition
https://www.readbyqxmd.com/read/28429344/up-regulation-of-microrna-367-promotes-liver-steatosis-through-repressing-tbl1-in-obese-mice
#2
D-D Li, Y Liu, L Xue, D-Y Su, W-Y Pang
OBJECTIVE: Increasing evidence has demonstrated that microRNAs (miRNAs) play a critical role in the progression of metabolic disorders, including obesity-induced non-alcoholic fatty liver disease (NAFLD). In the present study, the expression and function of miR-367 were investigated. MATERIALS AND METHODS: C57BL/6 male mice aged 8 weeks were fed with a normal diet (ND) or high-fat-diet (HFD). The expression levels of miR-367 were analyzed in livers from two groups of mice by quantitative real-time PCR...
April 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28421791/association-of-4-hydroxynonenal-with-classical-adipokines-and-insulin-resistance-in-a-chinese-non-diabetic-obese-population
#3
Lin Guo, Xi-Mei Zhang, Yun-Bo Zhang, Xiang Huang, Mei-Hua Chi
BACKGROUND: The prevalence of obesity is increasing worldwide. Oxidative stress plays an etiological role in a variety of obesity-related metabolic disorders. 4-hydroxynonenal (4-HNE) is the most abundant and reactive aldehydic product derived from the peroxidation of n-6 polyunsaturated fatty acids with diverse biological effects that are not well detailed. Obesity is associated with decreased plasma adiponectin concentrations and increased production of lipid peroxidation products, including 4-HNE, in adipose tissue...
March 30, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28412321/egcg-ameliorates-diet-induced-metabolic-syndrome-associating-with-the-circadian-clock
#4
Yashi Mi, Guoyuan Qi, Rong Fan, Xiaohua Ji, Zhigang Liu, Xuebo Liu
In response to the daily light-dark (LD) cycle, organisms on Earth have evolved with the approximately 24-h endogenous oscillations to coordinate behavioral and physiological processes, including feeding, sleep, and metabolism homeostasis. Circadian desynchrony triggered by an energy-dense diet rich in fats and fructose is intimately connected with a series of metabolic disorders. Previous studies revealed that (-)-Epigallocatechin-3-gallate (EGCG) could mitigate metabolic misalignment; however, only a few reports have focused on its potential effect on directly manipulating circadian rhythms to ameliorate metabolic syndrome...
April 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28409465/peroxisomal-membrane-and-matrix-protein-import-using-a-semi-intact-mammalian-cell-system
#5
Kanji Okumoto, Masanori Honsho, Yuqiong Liu, Yukio Fujiki
Peroxisomes are essential intracellular organelles that catalyze a number of essential metabolic pathways including β-oxidation of very long chain fatty acids, synthesis of plasmalogen, bile acids, and generation and degradation of hydrogen peroxide. These peroxisomal functions are accomplished by strictly and spatiotemporally regulated compartmentalization of the enzymes catalyzing these reactions. Defects in peroxisomal protein import result in inherited peroxisome biogenesis disorders in humans. Peroxisomal matrix and membrane proteins are synthesized on free ribosomes and transported to peroxisomes in a manner dependent on their specific targeting signals and their receptors...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28409271/unique-presentation-of-cutis-laxa-with-leigh-like-syndrome-due-to-echs1-deficiency
#6
S Balasubramaniam, L G Riley, D Bratkovic, D Ketteridge, N Manton, M J Cowley, V Gayevskiy, T Roscioli, M Mohamed, T Gardeitchik, E Morava, J Christodoulou
Clinical finding of cutis laxa, characterized by wrinkled, redundant, sagging, nonelastic skin, is of growing significance due to its occurrence in several different inborn errors of metabolism (IEM). Metabolic cutis laxa results from Menkes syndrome, caused by a defect in the ATPase copper transporting alpha (ATP7A) gene; congenital disorders of glycosylation due to mutations in subunit 7 of the component of oligomeric Golgi (COG7)-congenital disorders of glycosylation (CDG) complex; combined disorder of N- and O-linked glycosylation, due to mutations in ATPase H+ transporting V0 subunit a2 (ATP6VOA2) gene; pyrroline-5-carboxylate reductase 1 deficiency; pyrroline-5-carboxylate synthase deficiency; macrocephaly, alopecia, cutis laxa, and scoliosis (MACS) syndrome, due to Ras and Rab interactor 2 (RIN2) mutations; transaldolase deficiency caused by mutations in the transaldolase 1 (TALDO1) gene; Gerodermia osteodysplastica due to mutations in the golgin, RAB6-interacting (GORAB or SCYL1BP1) gene; and mitogen-activated pathway (MAP) kinase defects, caused by mutations in several genes [protein tyrosine phosphatase, non-receptor-type 11 (PTPN11), RAF, NF, HRas proto-oncogene, GTPase (HRAS), B-Raf proto-oncogene, serine/threonine kinase (BRAF), MEK1/2, KRAS proto-oncogene, GTPase (KRAS), SOS Ras/Rho guanine nucleotide exchange factor 2 (SOS2), leucine rich repeat scaffold protein (SHOC2), NRAS proto-oncogene, GTPase (NRAS), and Raf-1 proto-oncogene, serine/threonine kinase (RAF1)], which regulate the Ras-MAPK cascade...
April 13, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28404815/the-hepatokine-fgf21-is-crucial-for-peroxisome-proliferator-activated-receptor-%C3%AE-agonist-induced-amelioration-of-metabolic-disorders-in-obese-mice
#7
Tsuyoshi Goto, Mariko Hirata, Yumeko Aoki, Mari Iwase, Haruya Takahashi, Minji Kim, Yongjia Li, Huei-Fen Jheng, Wataru Nomura, Nobuyuki Takahashi, Chu-Sook Kim, Rina Yu, Shigeto Seno, Hideo Matsuda, Megumi Aizawa-Abe, Ken Ebihara, Nobuyuki Itoh, Teruo Kawada
Obesity causes excess fat accumulation in white adipose tissues (WAT) and also in other insulin-responsive organs such as the skeletal muscle, increasing the risk for insulin resistance, which can lead to obesity-related metabolic disorders. Peroxisome proliferator-activated receptor-α (PPARα) is a master regulator of fatty acid oxidation, whose activator is known to improve hyperlipidemia. However, the molecular mechanisms underlying PPARα activator-mediated reduction in adiposity and improvement of metabolic disorders are largely unknown...
April 12, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28397872/organochloride-pesticides-impaired-mitochondrial-function-in-hepatocytes-and-aggravated-disorders-of-fatty-acid-metabolism
#8
Qian Liu, Qihan Wang, Cheng Xu, Wentao Shao, Chunlan Zhang, Hui Liu, Zhaoyan Jiang, Aihua Gu
p,p'-dichlorodiphenyldichloroethylene (p, p'-DDE) and β-hexachlorocyclohexane (β-HCH) were two predominant organochlorine pesticides (OCPs) metabolites in human body associated with disorders of fatty acid metabolism. However, the underlying mechanisms have not been fully clarified. In this study, adult male C57BL/6 mice were exposed to low dose of p, p'-DDE and β-HCH for 8 wk. OCPs accumulation in organs, hepatic fatty acid composition, tricarboxylic acid cycle (TCA) metabolites and other metabolite profiles were analyzed...
April 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28394042/metabolic-profiling-of-parkinson-s-disease-and-mild-cognitive-impairment
#9
Florence Burté, David Houghton, Hannah Lowes, Angela Pyle, Sarah Nesbitt, Alison Yarnall, Patrick Yu-Wai-Man, David J Burn, Mauro Santibanez-Koref, Gavin Hudson
BACKGROUND: Early diagnosis of Parkinson's disease and mild cognitive impairment is important to enable prompt treatment and improve patient welfare, yet no standard diagnostic test is available. Metabolomics is a powerful tool used to elucidate disease mechanisms and identify potential biomarkers. OBJECTIVES: The objective of this study was to use metabolic profiling to understand the pathoetiology of Parkinson's disease and to identify potential disease biomarkers...
April 10, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28387565/the-therapeutic-potential-of-piceatannol-a-natural-stilbene-in-metabolic-diseases-a-review
#10
Jonathan Kershaw, Kee-Hong Kim
Metabolic disease comprises a set of risk factors highly associated with obesity and insulin resistance and is a consequence of central adiposity, hyperglycemia, and dyslipidemia. Furthermore, obesity increases the risk of the development of metabolic disease due to ectopic fat deposition, low-grade inflammation, and systemic energy disorders caused by dysregulated adipose tissue function. Piceatannol is a naturally occurring polyphenolic stilbene found in various fruits and vegetables and has been reported to exhibit anticancer and anti-inflammatory properties...
April 7, 2017: Journal of Medicinal Food
https://www.readbyqxmd.com/read/28377388/targeting-the-gut-microbiota-with-inulin-type-fructans-preclinical-demonstration-of-a-novel-approach-in-the-management-of-endothelial-dysfunction
#11
Emilie Catry, Laure B Bindels, Anne Tailleux, Sophie Lestavel, Audrey M Neyrinck, Jean-François Goossens, Irina Lobysheva, Hubert Plovier, Ahmed Essaghir, Jean-Baptiste Demoulin, Caroline Bouzin, Barbara D Pachikian, Patrice D Cani, Bart Staels, Chantal Dessy, Nathalie M Delzenne
OBJECTIVE: To investigate the beneficial role of prebiotics on endothelial dysfunction, an early key marker of cardiovascular diseases, in an original mouse model linking steatosis and endothelial dysfunction. DESIGN: We examined the contribution of the gut microbiota to vascular dysfunction observed in apolipoprotein E knockout (Apoe(-/-)) mice fed an n-3 polyunsaturated fatty acid (PUFA)-depleted diet for 12 weeks with or without inulin-type fructans (ITFs) supplementation for the last 15 days...
April 4, 2017: Gut
https://www.readbyqxmd.com/read/28368364/high-fat-diets-sex-specifically-affect-the-renal-transcriptome-and-program-obesity-kidney-injury-and-hypertension-in-the-offspring
#12
You-Lin Tain, Yu-Ju Lin, Jiunn-Ming Sheen, Hong-Ren Yu, Mao-Meng Tiao, Chih-Cheng Chen, Ching-Chou Tsai, Li-Tung Huang, Chien-Ning Hsu
Obesity and related disorders have increased concurrently with an increased consumption of saturated fatty acids. We examined whether post-weaning high fat (HF) diet would exacerbate offspring vulnerability to maternal HF-induced programmed hypertension and kidney disease sex-specifically, with a focus on the kidney. Next, we aimed to elucidate the gene-diet interactions that contribute to maternal HF-induced renal programming using the next generation RNA sequencing (NGS) technology. Female Sprague-Dawley rats received either a normal diet (ND) or HF diet (D12331, Research Diets) for five weeks before the delivery...
April 3, 2017: Nutrients
https://www.readbyqxmd.com/read/28361102/gene-centric-analysis-implicates-nuclear-encoded-mitochondrial-protein-gene-variants-in-migraine-susceptibility
#13
Shani Stuart, Miles C Benton, David A Eccles, Heidi G Sutherland, Larisa M Haupt, Rodney A Lea, Lyn R Griffiths
BACKGROUND: Migraine is a common neurological disorder which affects a large proportion of the population. The Norfolk Island population is a genetically isolated population and is an ideal discovery cohort for genetic variants involved in complex disease susceptibility given the reduced genetic and environmental heterogeneity. Given that the majority of proteins responsible for mitochondrial function are nuclear encoded, this study aimed to investigate the role of Nuclear Encoded Mitochondrial Protein (NEMP) genes in relation to migraine susceptibility...
March 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28353532/acute-illness-protocol-for-fatty-acid-oxidation-and-carnitine-disorders
#14
Saud H Aldubayan, Lance H Rodan, Gerard T Berry, Harvey L Levy
Inborn errors of metabolism (IEMs) are genetic disorders that disrupt enzyme activity, cellular transport, or energy production. They are individually rare but collectively have an incidence of 1:1000. Most patients with IEMs are followed by a physician with expertise in biochemical genetics (metabolism) but may present outside this setting. Because IEMs can present acutely with life-threatening crises that require specific interventions, it is critical for the emergency medicine physicians, pediatricians, internists, critical care physicians, and biochemical geneticists to be familiar with the initial assessment and management of patients with these disorders...
April 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28339706/differential-proliferation-and-metabolic-activity-of-sertoli-cells-in-the-testes-of-broiler-and-layer-breeder-chickens
#15
Mélanie Faure, Edith Guibert, Sabine Crochet, Pascal Chartrin, Jean-Pierre Brillard, Anne Collin, Pascal Froment
Decades of genetic selection have generated 2 different, highly specialized types of chickens in which 1 type, known as the layer-type chicken, expresses high laying performance while the other type, known as the broiler-type chicken, is dedicated to the production of fast-growing birds. Selected lines for the latter type often express disorders in their reproductive performance including early sexual maturation and accelerated, non-reversible seasonal decline of their semen production and mating behavior. The aim of the present study was to characterize some metabolic markers of the Sertoli cell populations...
February 21, 2017: Poultry Science
https://www.readbyqxmd.com/read/28324172/enzymes-involved-in-branched-chain-amino-acid-metabolism-in-humans
#16
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
#17
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
#18
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
#19
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
#20
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
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