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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/27906582/fiat-deletion-increases-bone-mass-but-does-not-prevent-high-fat-diet-induced-metabolic-complications
#1
Bahareh Hekmatnejad, Vionnie W C Yu, William Addison, Vice Mandic, Martin Pellicelli, Alice Arabian, René St-Arnaud
FIAT (Factor Inhibiting ATF4-mediated Transcription) interacts with ATF4 to repress its transcriptional activity. We performed a phenotypic analysis of Fiat-deficient male mice (Fiat(-/Y)) at 8 and 16 weeks of age. Fiat(-/Y) mice appeared normal at birth and weight gain was comparable between genotypes. μCT analysis of proximal femur demonstrated 46% and 13% age-dependent increases in trabecular bone volume and thickness, respectively, in Fiat(-/Y) mice. Cortical bone measurements at the femoral midshaft revealed a significant increase in cortical thickness in older Fiat(-/Y) mice...
December 1, 2016: Endocrinology
https://www.readbyqxmd.com/read/27903579/medium-chain-acyl-coenzyme-a-dehydrogenase-deficiency-mcadd-a-cause-of-severe-hypoglycaemia-in-an-apparently-well-child
#2
Kene Ebuka Maduemem
Medium-chain acyl-Coenzyme A dehydrogenase deficiency (MCADD) is a disorder of fatty acid β oxidation inherited in an autosomal recessive manner. The enzyme is useful in hepatic ketogenesis, a major source of energy once hepatic glycogen stores become depleted during prolonged fasting. It is a cause of hypoketotic hypoglycaemia in a previously well child. MCADD is not part of newborn screening in Ireland; children are likely to be missed if routine hypoglycaemic screen is not instituted when blood glucose level is below 2...
November 30, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27899449/deficiency-of-a-retinal-dystrophy-protein-acbd5-impairs-peroxisomal-%C3%AE-oxidation-of-very-long-chain-fatty-acids
#3
Yuichi Yagita, Kyoko Shinohara, Yuichi Abe, Keiko Nakagawa, Mohammed Al-Owain, Fowzan S Alkuraya, Yukio Fujiki
Acyl-CoA binding domain containing 5 (ACBD5) is a peroxisomal protein that carries an acyl-CoA binding domain (ACBD) at its N-terminal region. The recent identification of a mutation in the ACBD5 gene in patients with a syndromic form of retinal dystrophy highlights the physiological importance of ACBD5 in humans. However, the underlying pathogenic mechanisms and the precise function of ACBD5 remain unclear. We herein report that ACBD5 is a peroxisomal tail-anchored membrane protein exposing its ACBD to the cytosol...
November 29, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27896927/a-retrospective-review-of-anesthesia-and-perioperative-care-in-children-with-medium-chain-acyl-coa-dehydrogenase-deficiency
#4
Claire Allen, Russell Perkins, Bernd Schwahn
BACKGROUND: Medium-chain acyl-CoA dehydrogenase deficiency is the most common genetically determined disorder of mitochondrial fatty acid oxidation. Decompensation can result in hypoglycemia, seizures, coma, and death but may be prevented by ensuring glycogen stores do not become depleted. Perioperative care is of interest as surgery, fasting, and infection may all trigger decompensation and the safety of anesthetic agents has been questioned. Current guidelines from the British Inherited Metabolic Disease Group advise on administering fluid containing 10% glucose during the perioperative period...
November 29, 2016: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27889747/alisol-a-24-acetate-prevents-hepatic-steatosis-and-metabolic-disorders-in-hepg2-cells
#5
Lu Zeng, WaiJiao Tang, JinJin Yin, LiJuan Feng, Yabing Li, Xiaorui Yao, BenJie Zhou
BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is closely associated with metabolic disorders including hepatic lipid accumulation and inflammation. Alisol A 24-acetate, a triterpene from Alismatis rhizome, has multiple biologic activities such as hypolipidemic, anti-inflammatory and anti-diabetic. Thus we hypothesized that Alisol A 24 -acetate would have effect on NAFLD. The present study was conducted to investigate the therapeutic effects and potential mechanisms of Alisol A 24-acetate against hepatic steatosis in a free fatty acids (FFAs) induced NAFLD cell model...
November 25, 2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27888287/wnt-signaling-and-cellular-metabolism-in-osteoblasts
#6
REVIEW
Courtney M Karner, Fanxin Long
The adult human skeleton is a multifunctional organ undergoing continuous remodeling. Homeostasis of bone mass in a healthy adult requires an exquisite balance between bone resorption by osteoclasts and bone formation by osteoblasts; disturbance of such balance is the root cause for various bone disorders including osteoporosis. To develop effective and safe therapeutics to modulate bone formation, it is essential to elucidate the molecular mechanisms governing osteoblast differentiation and activity. Due to their specialized function in collagen synthesis and secretion, osteoblasts are expected to consume large amounts of nutrients...
November 26, 2016: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/27884962/triheptanoin-long-term-effects-in-the-very-long-chain-acyl-coa-dehydrogenase-vlcad-deficient-mouse
#7
Sara Tucci, Ulrich Floegel, Frauke Beermann, Sidney Behringer, Ute Spiekerkoetter
A rather new approach in the treatment of long-chain fatty acid oxidation disorders is represented by triheptanoin, a triglyceride with three medium-odd-chain heptanoic acid (C7) due to its anaplerotic potential. We here investigate the effects of a one-year triheptanoin-based diet on the clinical phenotype of very long-chain-acyl-CoA-dehydrogenase-deficient (VLCAD-/-) mice. The cardiac function was assessed in VLCAD-/- mice by in vivo MRI. Metabolic adaptations were identified by the expression of genes regulating energy metabolism and anaplerotic processes using real-time-PCR and the results were correlated with the measurement of the glycolytic enzymes pyruvate dehydrogenase (PDH) and pyruvate kinase (PK)...
November 24, 2016: Journal of Lipid Research
https://www.readbyqxmd.com/read/27870652/fatty-acid-metabolism-in-macrophages-a-target-in-cardio-metabolic-diseases
#8
Louise Ménégaut, Charles Thomas, Laurent Lagrost, David Masson
PURPOSE OF REVIEW: Recent studies have highlighted that macrophages dynamically and autonomously handle all the facets of fatty acid (FA) metabolism including FA oxidation and FA synthesis as well as the synthesis of monounsaturated FAs and long chain n-3 and n-6 polyunsaturated FAs. RECENT FINDINGS: Macrophage M2 polarization is associated with an increase of FA oxidation. However, whether increased FA oxidation simply correlates with or is required for M2 polarization needs to be further evaluated...
November 18, 2016: Current Opinion in Lipidology
https://www.readbyqxmd.com/read/27856258/aspirin-increases-mitochondrial-fatty-acid-oxidation
#9
Radha Uppala, Brianne Dudiak, Megan E Beck, Sivakama S Bharathi, Yuxun Zhang, Donna B Stolz, Eric S Goetzman
The metabolic effects of salicylates are poorly understood. This study investigated the effects of aspirin on fatty acid oxidation. Aspirin increased mitochondrial long-chain fatty acid oxidation, but inhibited peroxisomal fatty acid oxidation, in two different cell lines. Aspirin increased mitochondrial protein acetylation and was found to be a stronger acetylating agent in vitro than acetyl-CoA. However, aspirin-induced acetylation did not alter the activity of fatty acid oxidation proteins, and knocking out the mitochondrial deacetylase SIRT3 did not affect the induction of long-chain fatty acid oxidation by aspirin...
November 14, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27856190/screening-of-mcad-deficiency-in-japan-16years-experience-of-enzymatic-and-genetic-evaluation
#10
Go Tajima, Keiichi Hara, Miyuki Tsumura, Reiko Kagawa, Satoshi Okada, Nobuo Sakura, Ikue Hata, Yosuke Shigematsu, Masao Kobayashi
BACKGROUND: Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a representative disorder of fatty acid oxidation and is one of the most prevalent inborn errors of metabolism among Caucasian populations. In Japan, however, it was as late as 2000 when the first patient was found, and enzymatic and genetic evaluation of MCAD deficiency began. METHODS: We measured octanoyl-CoA dehydrogenase activity in lymphocytes of symptomatic children and newborn screening (NBS)-positive subjects who showed elevated levels of C8-acylcarnitine in blood...
October 21, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27855608/peroxisome-proliferator-activated-receptor-ppar-gamma-agonists-as-therapeutic-agents-for-cardiovascular-disorders-focus-on-atherosclerosis
#11
Ekaterina A Ivanova, Veronika A Myasoedova, Alexandra A Melnichenko, Alexander N Orekhov
Peroxisome proliferation-activated receptors (PPARs) are ligand-activated transcription factors that mainly regulate genes responsible for fatty acid (FA) and energy metabolism. There are three members of the PPAR family, PPAR-α, PPAR- β/δ and PPAR-γ. All three isoforms have therapeutic potential for treatment of cardiovascular disorders, and PPAR agonists are currently being actively studied in pre-clinical and clinical trials. PPAR γ agonists, main tissue expressing isoform, have potential to influence on inflammation processes, reduce oxidative stress, improve endothelial function and plays an important role in lipid metabolism...
November 18, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27815837/fish-oil-prevents-lipopolysaccharide-induced-depressive-like-behavior-by-inhibiting-neuroinflammation
#12
Zhe Shi, Huixia Ren, Zhijian Huang, Yu Peng, Baixuan He, Xiaoli Yao, Ti-Fei Yuan, Huanxing Su
Depression is associated with somatic immune changes, and neuroinflammation is now recognized as hallmark for depressive disorders. N-3 (or omega-3) polyunsaturated fatty acids (PUFAs) are well known to suppress neuroinflammation, reduce oxidative stress, and protect neuron from injury. We pretreated animals with fish oil and induced acute depression-like behaviors with systemic lipopolysaccharide (LPS) injection. The levels of cytokines and stress hormones were determined from plasma and different brain areas...
November 4, 2016: Molecular Neurobiology
https://www.readbyqxmd.com/read/27810341/diosgenin-ameliorates-gestational-diabetes-through-inhibition-of-sterol-regulatory-element-binding-protein-1
#13
Shaofang Hua, Yueqin Li, Lijun Su, Xiajun Liu
Gestational diabetes (GD) is a pathological condition, affecting 2-5% of pregnant women. Diosgenin (DSG) possesses a variety of biological activities. The present study was designed to examine the effect of DSG on GD and to investigate the possible mechanism in C57BL/KsJ-Lep(db/+) (db/+) mice. We found that DSG could remarkably ameliorated GD in pregnant db/+ mice, as reflected by the improvement of glucose and insulin intolerance, and the decrease of fasting blood glucose and insulin level and the increase of hepatic glycogen content...
October 31, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27809262/benzene-exposure-alters-expression-of-enzymes-involved-in-fatty-acid-%C3%AE-oxidation-in-male-c3h-he-mice
#14
Rongli Sun, Meng Cao, Juan Zhang, Wenwen Yang, Haiyan Wei, Xing Meng, Lihong Yin, Yuepu Pu
Benzene is a well-known hematotoxic carcinogen that can cause leukemia and a variety of blood disorders. Our previous study indicated that benzene disturbs levels of metabolites in the fatty acid β-oxidation (FAO) pathway, which is crucial for the maintenance and function of hematopoietic and leukemic cells. The present research aims to investigate the effects of benzene on changes in the expression of key enzymes in the FAO pathway in male C3H/He mice. Results showed that benzene exposure caused reduced peripheral white blood cell (WBC), red blood cell (RBC), platelet (Pit) counts, and hemoglobin (Hgb) concentration...
October 31, 2016: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/27807421/cancer-and-chemotherapy-contribute-to-muscle-loss-by-activating-common-signaling-pathways
#15
Rafael Barreto, Giorgia Mandili, Frank A Witzmann, Francesco Novelli, Teresa A Zimmers, Andrea Bonetto
Cachexia represents one of the primary complications of colorectal cancer due to its effects on depletion of muscle and fat. Evidence suggests that chemotherapeutic regimens, such as Folfiri, contribute to cachexia-related symptoms. The purpose of the present study was to investigate the cachexia signature in different conditions associated with severe muscle wasting, namely Colon-26 (C26) and Folfiri-associated cachexia. Using a quantitative LC-MS/MS approach, we identified significant changes in 386 proteins in the quadriceps muscle of Folfiri-treated mice, and 269 proteins differentially expressed in the C26 hosts (p < 0...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27791398/timing-of-glucocorticoid-administration-determines-severity-of-lipid-metabolism-and-behavioral-effects-in-rats
#16
Tao Wu, Jianguo Jiang, Luna Yang, Haifeng Li, Wanjing Zhang, Yangyang Chen, Binggong Zhao, Boda Kong, Ping Lu, Zhenzhen Zhao, Jiawei Zhu, Zhengwei Fu
Glucocorticoids (GCs) are a group of steroid hormones secreted by the adrenal glands in circadian cycles, and the dysregulation of GC signaling has been suggested to cause metabolic syndrome. Even though prolonged GC exposure is associated with serious side effects such as metabolic syndrome and central nervous system disorders, the use of GCs in anti-inflammatory and immunosuppressive therapies has been continuously rising. Meanwhile, the exact mechanisms by which GCs can influence the lipid metabolism as well as behavior and how they are affected by time remain unknown...
October 28, 2016: Chronobiology International
https://www.readbyqxmd.com/read/27789292/role-of-4-hydroxy-2-nonenal-hne-in-the-pathogenesis-of-alzheimer-disease-and-other-selected-age-related-neurodegenerative-disorders
#17
Fabio Di Domenico, Antonella Tramutola, D Allan Butterfield
Oxidative stress is involved in various and numerous pathological states including several age-related neurodegenerative diseases. Peroxidation of the membrane lipid bilayer is one of the major sources of free radical-mediated injury that directly damages neurons causing increased membrane rigidity, decreased activity of membrane-bound enzymes, impairment of membrane receptors and altered membrane permeability and eventual cell death. Moreover, the peroxidation of polyunsaturated fatty acids leads to the formation of aldehydes, which can act as toxic by-products...
October 24, 2016: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/27785012/docosahexaenoic-acid-liposomes-for-targeting-chronic-inflammatory-diseases-and-cancer-an-in-vitro-assessment
#18
Amr Alaarg, Nan Yeun Jordan, Johan Jf Verhoef, Josbert M Metselaar, Gert Storm, Robbert J Kok
Inflammation, oxidative stress, and uncontrolled cell proliferation are common key features of chronic inflammatory diseases, such as atherosclerosis and cancer. ω3 polyunsaturated fatty acids (PUFAs; also known as omega3 fatty acids or fish oil) have beneficial effects against inflammation upon dietary consumption. However, these effects cannot be fully exploited unless diets are enriched with high concentrations of fish oil supplements over long periods of time. Here, a nanomedicine-based approach is presented for delivering effective levels of PUFAs to inflammatory cells...
2016: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/27776119/fetus-derived-dlk1-is-required-for-maternal-metabolic-adaptations-to-pregnancy-and-is-associated-with-fetal-growth-restriction
#19
Mary A M Cleaton, Claire L Dent, Mark Howard, Jennifer A Corish, Isabelle Gutteridge, Ulla Sovio, Francesca Gaccioli, Nozomi Takahashi, Steven R Bauer, D Steven Charnock-Jones, Theresa L Powell, Gordon C S Smith, Anne C Ferguson-Smith, Marika Charalambous
Pregnancy is a state of high metabolic demand. Fasting diverts metabolism to fatty acid oxidation, and the fasted response occurs much more rapidly in pregnant women than in non-pregnant women. The product of the imprinted DLK1 gene (delta-like homolog 1) is an endocrine signaling molecule that reaches a high concentration in the maternal circulation during late pregnancy. By using mouse models with deleted Dlk1, we show that the fetus is the source of maternal circulating DLK1. In the absence of fetally derived DLK1, the maternal fasting response is impaired...
December 2016: Nature Genetics
https://www.readbyqxmd.com/read/27775420/the-role-of-ppargamma-in-cardiovascular-diseases
#20
M Kvandová, M Majzúnová, I Dovinová
The peroxisome proliferator-activated receptors (PPAR) belong to the nuclear superfamily of ligand-activated transcription factors. PPARgamma acts as a nutrient sensor that regulates several homeostatic functions. Its disruption can lead to vascular pathologies, disorders of fatty acid/lipid metabolism and insulin resistance. PPARgamma can modulate several signaling pathways connected with blood pressure regulation. Firstly, it affects the insulin signaling pathway and endothelial dysfunction by modulation of expression and/or phosphorylation of signaling molecules through the PI3K/Akt/eNOS or MAPK/ET-1 pathways...
October 24, 2016: Physiological Research
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