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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/29332575/protective-role-of-italian-juglans-regia-l-nut-ethanolic-extract-in-human-keratinocytes-under-oxidative-and-inflammatory-stress
#1
Cinzia Calcabrini, Umberto Mancini, Roberta De Bellis, Alessandra Frati, Anna Rita Mastrogiacomo, Giosue Annibalini, Piero Sestili, Luigi Cucchiarini, Vilberto Stocchi, Lucia Potenza
OBJECTIVE: In this research, fatty acid profile and polyphenolic content of an ethanolic extract of walnut from Juglans regia L. collected in Central Italy, were performed. The potential antioxidant and anti-inflammatory effects of the extract were investigated in the human keratinocytes cell line. METHODS: Fatty acid profile was determined by gas chromatography/mass spectrometry analysis, total phenolic content by Folin-Ciocalteu method and aluminum chloride colorimetric method was used for determination of total flavonoids...
January 12, 2018: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/29323266/aif-loss-deregulates-hematopoiesis-and-reveals-different-adaptive-metabolic-responses-in-bone-marrow-cells-and-thymocytes
#2
Lauriane Cabon, Audrey Bertaux, Marie-Noëlle Brunelle-Navas, Ivan Nemazanyy, Laurianne Scourzic, Laure Delavallée, Laura Vela, Mathieu Baritaud, Sandrine Bouchet, Cécile Lopez, Vu Quang Van, Kevin Garbin, Danielle Chateau, Françoise Gilard, Marika Sarfati, Thomas Mercher, Olivier A Bernard, Santos A Susin
Mitochondrial metabolism is a tightly regulated process that plays a central role throughout the lifespan of hematopoietic cells. Herein, we analyze the consequences of the mitochondrial oxidative phosphorylation (OXPHOS)/metabolism disorder associated with the cell-specific hematopoietic ablation of apoptosis-inducing factor (AIF). AIF-null (AIF-/Y ) mice developed pancytopenia that was associated with hypocellular bone marrow (BM) and thymus atrophy. Although myeloid cells were relatively spared, the B-cell and erythroid lineages were altered with increased frequencies of precursor B cells, pro-erythroblasts I, and basophilic erythroblasts II...
January 11, 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29317722/loss-of-the-mitochondrial-fatty-acid-%C3%AE-oxidation-protein-medium-chain-acyl-coenzyme-a-dehydrogenase-disrupts-oxidative-phosphorylation-protein-complex-stability-and-function
#3
Sze Chern Lim, Makiko Tajika, Masaru Shimura, Kirstyn T Carey, David A Stroud, Kei Murayama, Akira Ohtake, Matthew McKenzie
Medium-chain acyl-Coenzyme A dehydrogenase (MCAD) is involved in the initial step of mitochondrial fatty acid β-oxidation (FAO). Loss of function results in MCAD deficiency, a disorder that usually presents in childhood with hypoketotic hypoglycemia, vomiting and lethargy. While the disruption of mitochondrial fatty acid metabolism is the primary metabolic defect, secondary defects in mitochondrial oxidative phosphorylation (OXPHOS) may also contribute to disease pathogenesis. Therefore, we examined OXPHOS activity and stability in MCAD-deficient patient fibroblasts that have no detectable MCAD protein...
January 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29312822/effect-of-dark-sweet-cherry-powder-consumption-on-the-gut-microbiota-short-chain-fatty-acids-and-biomarkers-of-gut-health-in-obese-db-db-mice
#4
Jose F Garcia-Mazcorro, Nara N Lage, Susanne Mertens-Talcott, Stephen Talcott, Boon Chew, Scot E Dowd, Jorge R Kawas, Giuliana D Noratto
Cherries are fruits containing fiber and bioactive compounds (e.g., polyphenolics) with the potential of helping patients with diabetes and weight disorders, a phenomenon likely related to changes in the complex host-microbiota milieu. The objective of this study was to investigate the effect of cherry supplementation on the gut bacterial composition, concentrations of caecal short-chain fatty acids (SCFAs) and biomarkers of gut health using an in vivo model of obesity. Obese diabetic (db/db) mice received a supplemented diet with 10% cherry powder (supplemented mice, n = 12) for 12 weeks; obese (n = 10) and lean (n = 10) mice served as controls and received a standard diet without cherry...
2018: PeerJ
https://www.readbyqxmd.com/read/29289598/why-should-neuroscientists-worry-about-iron-the-emerging-role-of-ferroptosis-in-the-pathophysiology-of-neuroprogressive-diseases
#5
REVIEW
Gerwyn Morris, Michael Berk, André F Carvalho, Michael Maes, Adam J Walker, Basant K Puri
Ferroptosis is a unique form of programmed death, characterised by cytosolic accumulation of iron, lipid hydroperoxides and their metabolites, and effected by the fatal peroxidation of polyunsaturated fatty acids in the plasma membrane. It is a major driver of cell death in neurodegenerative neurological diseases. Moreover, cascades underpinning ferroptosis could be active drivers of neuropathology in major psychiatric disorders. Oxidative and nitrosative stress can adversely affect mechanisms and proteins governing cellular iron homeostasis, such as the iron regulatory protein/iron response element system, and can ultimately be a source of abnormally high levels of iron and a source of lethal levels of lipid membrane peroxidation...
December 28, 2017: Behavioural Brain Research
https://www.readbyqxmd.com/read/29282281/unbalanced-lipolysis-results-in-lipotoxicity-and-mitochondrial-damage-in-peroxisome-deficient-pex19-mutants
#6
Margret H Bülow, Christian Wingen, Deniz Senyilmaz, Dominic Gosejacob, Mariangela Sociale, Reinhard Bauer, Heike Schulze, Konrad Sandhoff, Aurelio A Teleman, Michael Hoch, Julia Sellin
Inherited Peroxisomal biogenesis disorders (PBDs) are characterized by the absence of functional peroxisomes. They are caused by mutations of peroxisomal biogenesis factors encoded by PEX genes, and result in childhood lethality. Due to the many metabolic functions fulfilled by peroxisomes, PBD pathology is complex and incompletely understood. Besides accumulation of peroxisomal educts (like very long chain fatty acids (VLCFA) or branched chain fatty acids) and lack of products (like bile acids or plasmalogens), many peroxisomal defects lead to detrimental mitochondrial abnormalities for unknown reasons...
December 27, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29249211/remodelling-of-primary-human-cd4-t-cell-plasma-membrane-order-by-n-3-pufa
#7
Yang-Yi Fan, Natividad R Fuentes, Tim Y Hou, Rola Barhoumi, Xian C Li, Nicolaas E P Deutz, Marielle P K J Engelen, David N McMurray, Robert S Chapkin
Cell membrane fatty acids influence fundamental properties of the plasma membrane, including membrane fluidity, protein functionality, and lipid raft signalling. Evidence suggests that dietary n-3 PUFA may target the plasma membrane of immune cells by altering plasma membrane lipid dynamics, thereby regulating the attenuation of immune cell activation and suppression of inflammation. As lipid-based immunotherapy might be a promising new clinical strategy for the treatment of inflammatory disorders, we conducted in vitro and in vivo experiments to examine the effects of n-3 PUFA on CD4+ T cell membrane order, mitochondrial bioenergetics and lymphoproliferation...
December 18, 2017: British Journal of Nutrition
https://www.readbyqxmd.com/read/29241711/l-carnitine-and-heart-disease
#8
REVIEW
Zhong-Yu Wang, Ying-Yi Liu, Guo-Hui Liu, Hai-Bin Lu, Cui-Ying Mao
Cardiovascular disease (CVD) is a key cause of deaths worldwide, comprising 15-17% of healthcare expenditure in developed countries. Current records estimate an annual global average of 30 million cardiac dysfunction cases, with a predicted escalation by two-three folds for the next 20-30years. Although β-blockers and angiotensin-converting-enzymes are commonly prescribed to control CVD risk, hepatotoxicity and hematological changes are frequent adverse events associated with these drugs. Search for alternatives identified endogenous cofactor l-carnitine, which is capable of promoting mitochondrial β-oxidation towards a balanced cardiac energy metabolism...
December 11, 2017: Life Sciences
https://www.readbyqxmd.com/read/29235891/lipid-biomarkers-for-the-peroxisomal-and-lysosomal-disorders-their-formation-metabolism-and-measurement
#9
Ryuichi Mashima, Masamitsu Maekawa
Lipid biomarkers play important roles in the diagnosis of and monitoring of treatment in peroxisomal disorders and lysosomal storage disorders. Today, a variety of lipids, including very long chain fatty acids, glycolipids, bile acids and the oxidation products of cholesterol, have been considered as biomarkers for these disorders. In this brief review, the authors summarized the recent advances regarding these lipid biomarkers in terms of their formation, metabolism and measurement in these disorders. An understanding of these biomarkers will offer a key to the development of novel diagnoses and help create more effective therapies in the future...
December 13, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/29206907/a-novel-bioactive-derivative-of-eicosapentaenoic-acid-epa-suppresses-intestinal-tumor-development-in-apc%C3%AE-14-mice
#10
Masako Nakanishi, Matthew P Hanley, Ruochen Zha, Yuichi Igarashi, Mark A Hull, Gary Mathias, Frank Sciavolino, James J Grady, Daniel W Rosenberg
Familial adenomatous polyposis (FAP) is a genetic disorder characterized by the development of hundreds of polyps throughout the colon. Without prophylactic colectomy, most individuals with FAP develop colorectal cancer at an early age. Treatment with eicosapentaenoic acid (EPA) in the free fatty acid form (EPA-FFA) has been shown to reduce polyp burden in FAP patients. Since high-purity EPA-FFA is subject to rapid oxidation, a stable form of EPA compound has been developed in the form of magnesium l-lysinate bis-eicosapentaenoate (TP-252)...
December 1, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/29197803/redox-system-and-phospholipid-metabolism-in-the-kidney-of-hypertensive-rats-after-faah-inhibitor-urb597-administration
#11
Michał Biernacki, Ewa Ambrożewicz, Agnieszka Gęgotek, Marek Toczek, Katarzyna Bielawska, Elżbieta Skrzydlewska
Primary and secondary hypertension is associated with kidney redox imbalance resulting in enhanced reactive oxygen species (ROS) and enzymes dependent phospholipid metabolism. The fatty acid amide hydrolase inhibitor, URB597, modulates the levels of endocannabinoids, particularly of anandamide, which is responsible for controlling blood pressure and regulating redox balance. Therefore, this study aimed to compare the effects of chronic URB597 administration to spontaneously hypertensive rats (SHR) and rats with secondary hypertension (DOCA-salt rats) on the kidney metabolism associated with the redox and endocannabinoid systems...
November 27, 2017: Redox Biology
https://www.readbyqxmd.com/read/29196872/seleno-compounds-and-carnosic-acid-added-to-diets-with-rapeseed-and-fish-oils-affect-concentrations-of-selected-elements-and-chemical-composition-in-the-liver-heart-and-muscles-of-lambs
#12
Marian Czauderna, Anna Ruszczyńska, Ewa Bulska, Katarzyna Agnieszka Krajewska
The objective of our studies was to investigate effects of carnosic acid (CA), selenized yeast (SeY) and selenate (SeVI) added to the diet including rapeseed oil (RO) and fish oil (FO) on concentrations of elements, fatty acids (FAs), tocopherols, cholesterol, and malondialdehyde in the liver, heart, musculus longissimus dorsi (MLD), and musculus biceps femoris (MBF) of lambs. Lambs were fed diets: group I-the basal diet (BD) with RO; group II-BD with RO and FO; group III-BD with RO, FO, and CA; group IV-BD with RO, FO, CA, and SeY; group V-BD with RO, FO, CA, and SeVI...
December 2, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/29191637/spontaneous-development-of-hepatosteatosis-in-perilipin-1-null-mice-with-adipose-tissue-dysfunction
#13
Suning Wei, Shangxin Liu, Xueying Su, Weiyi Wang, Fengjuan Li, Jingna Deng, Ying Lyu, Bin Geng, Guoheng Xu
Fatty liver features triglyceride accumulation in hepatocytes and often occurs with obesity and lipodystrophy in humans. Here, we investigated the mechanism of maladaptive hepatosteatosis with adipose-tissue dysfunction. Perilipin 1 (Plin1) did not exist in hepatocytes but was expressed exclusively in adipocytes as a dual modulator for regulating two principal adipose-tissue functions, triglyceride storage and breakdown. Plin1-/- mice showed decreased fat storage but increased lipolysis and efflux of fatty acids from adipose tissue, and hepatosteatosis spontaneously developed without altered circulating inflammatory adipocytokine levels...
November 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29188895/supplementation-of-mussel-peptides-reduces-aging-phenotype-lipid-deposition-and-oxidative-stress-in-d-galactose-induce-aging-mice
#14
Y Zhou, Q Xu, Y Dong, S Zhu, S Song, S Sun
OBJECTIVES: Aging is associated with glucose and lipid metabolism disorder. We aimed to examine the effects of mussel peptides on protecting against aging by regulating glucose and lipid metabolism. METHODS: For the aging model, d-galactose (200 mg/kg) was administered subcutaneously to 8-month-old mice for 8 weeks. Mussel peptides (1,000 mg/kg) were simultaneously administered by intragastric gavage. The glucose and lipid metabolism profiles, aging phenotype and peroxisome proliferator-activated receptors (PPARs) expression in the liver and adipose tissue of ICR mice were measured...
2017: Journal of Nutrition, Health & Aging
https://www.readbyqxmd.com/read/29185933/severe-riboflavin-deficiency-induces-alterations-in-the-hepatic-proteome-of-starter-pekin-ducks
#15
Jing Tang, Maria A Hegeman, Jian Hu, Ming Xie, Wenbiao Shi, Yong Jiang, Vincent de Boer, Yuming Guo, Shuisheng Hou, Jaap Keijer
Suboptimal vitamin B2 status is encountered globally. Riboflavin deficiency depresses growth and results in a fatty liver. The underlying mechanisms remain to be established and an overview of molecular alterations is lacking. We investigated hepatic proteome changes induced by riboflavin deficiency to explain its effects on growth and hepatic lipid metabolism. In all, 360 1-d-old Pekin ducks were divided into three groups of 120 birds each, with twelve replicates and ten birds per replicate. For 21 d, the ducks were fed ad libitum a control diet (CAL), a riboflavin-deficient diet (RD) or were pair-fed with the control diet to the mean daily intake of the RD group (CPF)...
November 2017: British Journal of Nutrition
https://www.readbyqxmd.com/read/29178253/kefir-peptides-prevent-hyperlipidemia-and-obesity-in-high-fat-diet-induced-obese-rats-via-lipid-metabolism-modulation
#16
Yu-Tang Tung, Hsiao-Ling Chen, Hsin-Shan Wu, Mei-Hsuan Ho, Kowit-Yu Chong, Chuan-Mu Chen
SCOPE: Obesity has reached epidemic proportions worldwide. Obesity is a complex metabolic disorder that is linked to numerous serious health complications with high morbidity. The present study evaluated the effects of kefir peptides on high fat diet-induced obesity in rats. METHODS AND RESULTS: Kefir peptides markedly improved obesity, including body weight gain, inflammatory reactions and the formation of adipose tissue fat deposits around the epididymis and kidney, and adipocyte size...
November 24, 2017: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/29177110/advances-in-the-understanding-and-treatment-of-mitochondrial-fatty-acid-oxidation-disorders
#17
Eric S Goetzman
Purpose of review: This review focuses on advances made in the past three years with regards to understanding the mitochondrial fatty acid oxidation (FAO) pathway, the pathophysiological ramifications of genetic lesions in FAO enzymes, and emerging therapies for FAO disorders. Recent findings: FAO has now been recognized to play a key energetic role in pulmonary surfactant synthesis, T-cell differentiation and memory, and the response of the proximal tubule to kidney injury...
September 2017: Current Genetic Medicine Reports
https://www.readbyqxmd.com/read/29176715/inhalation-exposure-to-pm2-5-counteracts-hepatic-steatosis-in-mice-fed-high-fat-diet-by-stimulating-hepatic-autophagy
#18
Yining Qiu, Ze Zheng, Hyunbae Kim, Zhao Yang, Gary Zhang, Xiangyang Shi, Fei Sun, Changya Peng, Yuchuan Ding, Aixia Wang, Lung-Chi Chen, Sanjay Rajagopalan, Qinghua Sun, Kezhong Zhang
Air pollution is associated with the increased risk of metabolic syndrome. In this study, we performed inhalation exposure of mice fed normal chow or a high-fat diet to airborne fine particulate matters (PM2.5), and then investigated the complex effects and mechanisms of inhalation exposure to PM2.5 on hepatic steatosis, a precursor or manifestation of metabolic syndrome. Our studies demonstrated that inhalation exposure of mice fed normal chow to concentrated ambient PM2.5 repressed hepatic transcriptional regulators involved in fatty acid oxidation and lipolysis, and thus promoted hepatic steatosis...
November 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29172946/postprandial-gut-microbiota-driven-choline-metabolism-links-dietary-cues-to-adipose-tissue-dysfunction
#19
Rebecca C Schugar, Belinda Willard, Zeneng Wang, J Mark Brown
The human body is an integrated circuit between microbial symbionts and our Homo sapien genome, which communicate bi-directionally to maintain homeostasis within the human meta-organism. There is now strong evidence that microbes resident in the human intestine can directly contribute to the pathogenesis of obesity and associated cardiometabolic disorders. In fact, gut microbes represent a filter of our greatest environmental exposure - the foods we consume. It is now clear that we each experience a given meal differently, based on our unique gut microbial communities...
November 27, 2017: Adipocyte
https://www.readbyqxmd.com/read/29167330/endothelial-cell-metabolism
#20
REVIEW
Guy Eelen, Pauline de Zeeuw, Lucas Treps, Ulrike Harjes, Brian W Wong, Peter Carmeliet
Endothelial cells (ECs) are more than inert blood vessel lining material. Instead, they are active players in the formation of new blood vessels (angiogenesis) both in health and (life-threatening) diseases. Recently, a new concept arose by which EC metabolism drives angiogenesis in parallel to well-established angiogenic growth factors (e.g., vascular endothelial growth factor). 6-Phosphofructo-2-kinase/fructose-2,6-bisphosphatase-3-driven glycolysis generates energy to sustain competitive behavior of the ECs at the tip of a growing vessel sprout, whereas carnitine palmitoyltransferase 1a-controlled fatty acid oxidation regulates nucleotide synthesis and proliferation of ECs in the stalk of the sprout...
January 1, 2018: Physiological Reviews
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