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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/28532786/fluxomic-evidence-for-impaired-contribution-of-short-chain-acyl-coa-dehydrogenase-to-mitochondrial-palmitate-%C3%AE-oxidation-in-symptomatic-patients-with-acads-gene-susceptibility-variants
#1
Anne-Frédérique Dessein, Monique Fontaine, Marie Joncquel Chevalier Curt, Gilbert Briand, Claire Sechter, Karine Mention-Mulliez, Dries Dobbelaere, Claire Douillard, Arnaud Lacour, Isabelle Redonnet-Vernhet, Delphine Lamireau, Magalie Barth, Marie-Christine Minot-Myhié, Alice Kuster, Pascale de Lonlay, Niels Gregersen, Cécile Acquaviva, Christine Vianey-Saban, Joseph Vamecq
BACKGROUND: Despite ACADS (acyl-CoA dehydrogenase, short-chain) gene susceptibility variants (c.511C>T and c.625G>A) are considered to be non-pathogenic, encoded proteins are known to exhibit altered kinetics. Whether or not, they might affect overall fatty acid β-oxidation still remains, however, unclear. METHODS: De novo biosynthesis of acylcarnitines by whole blood samples incubated with deuterated palmitate (16-(2)H3,15-(2)H2-palmitate) is suitable as a fluxomic exploration to distinguish between normal and disrupted β-oxidation, abnormal profiles and ratios of acylcarnitines with different chain-lengths being indicative of the site for enzymatic blockade...
May 19, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28531131/the-role-of-interleukin-18-oxidative-stress-and-metabolic-syndrome-in-alzheimer-s-disease
#2
REVIEW
Johanna O Ojala, Elina M Sutinen
The role of interleukins (ILs) and oxidative stress (OS) in precipitating neurodegenerative diseases including sporadic Alzheimer's disease (AD), requires further clarification. In addition to neuropathological hallmarks-extracellular neuritic amyloid-β (Aβ) plaques, neurofibrillary tangles (NFT) containing hyperphosphorylated tau and neuronal loss-chronic inflammation, as well as oxidative and excitotoxic damage, are present in the AD brain. The pathological sequelae and the interaction of these events during the course of AD need further investigation...
May 21, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28528080/toxicological-aspects-of-interesterified-fat-brain-damages-in-rats
#3
Lívia Ferraz D'avila, Verônica Tironi Dias, Luciana Taschetto Vey, Laura Hautrive Milanesi, Karine Roversi, Tatiana Emanuelli, Marilise Escobar Bürger, Fabíola Trevizol
In recent years, interesterified fat (IF) has been used to replace hydrogenated vegetable fat (HVF), rich in trans isomers, being found in processed foods. Studies involving IF have shown deleterious influences on the metabolic system, similarly to HVF, whereas no studies regarding its influence on the central nervous system (CNS) were performed. Rats from first generation born and maintained under supplementation (3g/Kg, p.o.) of soybean-oil or IF until adulthood were assessed on memory, biochemical and molecular markers in the hippocampus...
May 17, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28526925/polyphenolic-extract-attenuates-fatty-acid-induced-steatosis-and-oxidative-stress-in-hepatic-and-endothelial-cells
#4
Laura Vergani, Giulia Vecchione, Francesca Baldini, Elena Grasselli, Adriana Voci, Piero Portincasa, Pier Francesco Ferrari, Bahar Aliakbarian, Alessandro A Casazza, Patrizia Perego
PURPOSE: Phenolic compounds (PC) of virgin olive oil exert several biochemical and pharmacological beneficial effects. Some dietary PC seem to prevent/improve obesity and metabolic-related disorders such as non-alcoholic fatty liver disease (NAFLD). We investigated the possible effects of PC extracted from olive pomace (PEOP) and of the main single molecules present in the extract (tyrosol, apigenin, oleuropein, p-coumaric and caffeic acid) in protecting hepatocytes and endothelial cells against triglyceride accumulation and oxidative stress...
May 19, 2017: European Journal of Nutrition
https://www.readbyqxmd.com/read/28516284/short-chain-acyl-coa-dehydrogenase-deficiency-from-gene-to-cell-pathology-and-possible-disease-mechanisms
#5
REVIEW
Zahra Nochi, Rikke Katrine Jentoft Olsen, Niels Gregersen
Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is an inherited disorder of mitochondrial fatty acid oxidation that is characterized by the presence of increased butyrylcarnitine and ethylmalonic acid (EMA) concentrations in plasma and urine. Individuals with symptomatic SCADD may show relatively severe phenotype, while the majority of those who are diagnosed through newborn screening by tandem mass spectrometry may remain asymptomatic. As such, the associated clinical symptoms are very diverse, ranging from severe metabolic or neuromuscular disabilities to asymptomatic...
May 17, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28516040/a-newborn-case-with-carnitine-palmitoyltransferase-ii-deficiency-initially-judged-as-unaffected-by-acylcarnitine-analysis-soon-after-birth
#6
Kenji Yamada, Ryosuke Bo, Hironori Kobayashi, Yuki Hasegawa, Mako Ago, Seiji Fukuda, Seiji Yamaguchi, Takeshi Taketani
Carnitine palmitoyltransferase II (CPT-2) deficiency, an autosomal recessive disorder of fatty acid oxidation, can be detected by newborn screening using tandem mass spectrometry (TMS). Our case was a boy born at 38 weeks and 6 days of gestation via normal vaginal delivery; his elder sister was affected with CPT-2 deficiency. Acylcarnitine (AC) was analyzed in both dried blood spots (DBS) and serum 2 h after birth to determine whether the boy was also affected. His C16 and C18:1 AC levels in DBS were in the normal range, while his serum long-chain AC levels were marginally increased but lower than those of his sister...
June 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28515471/clinical-and-molecular-investigation-of-14-japanese-patients-with-complete-tfp-deficiency-a-comparison-with-caucasian-cases
#7
Ryosuke Bo, Kenji Yamada, Hironori Kobayashi, Purevsuren Jamiyan, Yuki Hasegawa, Takeshi Taketani, Seiji Fukuda, Ikue Hata, Yo Niida, Yosuke Shigematsu, Kazumoto Iijima, Seiji Yamaguchi
Mitochondrial trifunctional protein (TFP) deficiency is an inherited metabolic disorder of mitochondrial fatty-acid oxidation. Isolated long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is often reported in Caucasian countries due to a common mutation. However, the molecular and clinical basis of complete TFP deficiency has not been extensively reported. In this study, 14 Japanese cases (13 families) with complete TFP deficiency, including 9 previously reported cases, were analyzed to clarify the clinical and molecular characteristics of TFP deficiency...
May 18, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28511159/effect-of-apple-polyphenol-concentrate-on-lipid-metabolism-in-rats-under-experimental-insulin-resistance
#8
Andriy L Zagayko, Ganna B Kravchenko, Viktoriia P Fylymonenko, Oksana A Krasilnikova
INTRODUCTION: Obesity is strongly associated with an increased risk of developing insulin resistance as the metabolic indicator of prediabetes and a major risk factor in diabetes mellitus type 2 pathogenesis. Medicinal products obtained from apples can be used as potent prophylactic and therapeutic remedies in treatment of diabetes mellitus. AIM: Experiment was designed to study the effect of total apple polyphenol food concentrate on lipid metabolism under experimental IR...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28509906/therapeutic-efficacy-of-atypical-antipsychotic-drugs-by-targeting-multiple-stress-related-metabolic-pathways
#9
H L Cai, P Jiang, Q Y Tan, R L Dang, M M Tang, Y Xue, Y Deng, B K Zhang, P F Fang, P Xu, D X Xiang, H D Li, J K Yao
Schizophrenia (SZ) is considered to be a multifactorial brain disorder with defects involving many biochemical pathways. Patients with SZ show variable responses to current pharmacological treatments of SZ because of the heterogeneity of this disorder. Stress has a significant role in the pathophysiological pathways and therapeutic responses of SZ. Atypical antipsychotic drugs (AAPDs) can modulate the stress response of the hypothalamic-pituitary-adrenal (HPA) axis and exert therapeutic effects on stress by targeting the prefrontal cortex (PFC) and hippocampus...
May 16, 2017: Translational Psychiatry
https://www.readbyqxmd.com/read/28501921/cranberry-vaccinium-macrocarpon-extract-treatment-improves-triglyceridemia-liver-cholesterol-liver-steatosis-oxidative-damage-and-corticosteronemia-in-rats-rendered-obese-by-high-fat-diet
#10
Thamara C Peixoto, Egberto G Moura, Elaine de Oliveira, Patrícia N Soares, Deysla S Guarda, Dayse N Bernardino, Xu Xue Ai, Vanessa da S T Rodrigues, Gabriela Rodrigues de Souza, Antonio Jorge Ribeiro da Silva, Mariana S Figueiredo, Alex C Manhães, Patrícia C Lisboa
PURPOSE: Obese individuals have higher production of reactive oxygen species, which leads to oxidative damage. We hypothesize that cranberry extract (CE) can improve this dysfunction in HFD-induced obesity in rats since it has an important antioxidant activity. Here, we evaluated the effects of CE in food intake, adiposity, biochemical and hormonal parameters, lipogenic and adipogenic factors, hepatic morphology and oxidative balance in a HFD model. METHODS: At postnatal day 120 (PN120), male Wistar rats were assigned into two groups: (1) SD (n = 36) fed with a standard diet and (2) HFD (n = 36), fed with a diet containing 44...
May 13, 2017: European Journal of Nutrition
https://www.readbyqxmd.com/read/28493141/therapeutic-strategies-in-adrenoleukodystrophy
#11
Bela R Turk, Ann B Moser, Ali Fatemi
Adrenoleukodystrophy (ALD) is an X‑linked hereditary disorder due to mutations of the ABCD1 gene, which encodes a peroxisomal transport protein necessary for very long-chain fatty acid degradation (VLCFA). Toxic accumulation thereof is associated with a proinflammatory state and eventual cell death in multiple tissues. ALD may manifest either as a fatal, rapidly progressive demyelinating disease in boys and adult men, or as a slowly progressive adult-onset long-tract myelopathy along with peripheral neuropathy...
May 10, 2017: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28483670/role-of-14-3-3%C3%AE-protein-on-cardiac-fatty-acid-metabolism-and-macrophage-polarization-after-high-fat-diet-induced-type-2-diabetes-mellitus
#12
Remya Sreedhar, Somasundaram Arumugam, Rajarajan A Thandavarayan, Vengadeshprabhu Karuppagounder, Yusuke Koga, Takashi Nakamura, Meilei Harima, Kenichi Watanabe
Diabetic cardiomyopathy (DCM), a metabolic disorder, is one of the leading causes of mortality around the world and its pathogenesis involves cardiac inflammation and altered metabolic profile. Altered fatty acid metabolism during DCM can cause macrophage polarization in which inflammatory M1 phenotype dominates over the anti-inflammatory M2 phenotype. Hence, it is essential to identify a specific target, which could revert the metabolic profile and thereby reducing the M1 macrophage polarization. 14-3-3η protein has several cellular protective functions especially in the heart as plenty of reports available in various animal models of heart failure including diabetes mellitus...
May 5, 2017: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/28482236/ablation-of-%C3%AE-%C3%AE-carotene-9-10-oxygenase-2-remodels-the-hypothalamic-metabolome-leading-to-metabolic-disorders-in-mice
#13
Xin Guo, Lei Wu, Yi Lyu, Winyoo Chowanadisai, Stephen L Clarke, Edralin A Lucas, Brenda J Smith, Hui He, Weiqun Wang, Denis M Medeiros, Dingbo Lin
β,β-Carotene-9',10'-oxygenase 2 (BCO2) is a protein localized to the inner membrane of mitochondria. It was initially discovered as an enzyme that catalyzes the asymmetric cleavage of carotenoids. Systemic depletion of BCO2 causes increased food intake and impaired hepatic lipid metabolism in mice. The aim of this current study was to determine the extent to which BCO2 exerts its role in hypothalamic nutrient metabolism and feeding behavior through remodeling the hypothalamic metabolome in mice. Male BCO2 knockout (KO) and the isogenic wild-type 129S6 (WT) mice at 6 weeks of age were used for metabolic and cytokine and hypothalamic metabolomics and biochemical analysis...
April 12, 2017: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/28475111/riboflavin-responsive-mitochondrial-dysfunction-in-neurodegenerative-diseases
#14
REVIEW
Tamilarasan Udhayabanu, Andreea Manole, Mohan Rajeshwari, Perumal Varalakshmi, Henry Houlden, Balasubramaniem Ashokkumar
Mitochondria are the repository for various metabolites involved in diverse energy-generating processes, like the TCA cycle, oxidative phosphorylation, and metabolism of amino acids, fatty acids, and nucleotides, which rely significantly on flavoenzymes, such as oxidases, reductases, and dehydrogenases. Flavoenzymes are functionally dependent on biologically active flavin adenine dinucleotide (FAD) or flavin mononucleotide (FMN), which are derived from the dietary component riboflavin, a water soluble vitamin...
May 5, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28472361/energy-metabolism-of-the-osteoblast-implications-for-osteoporosis
#15
Wen-Chih Lee, Anyonya R Guntur, Fanxin Long, Clifford J Rosen
Osteoblasts, the bone forming cells of the remodeling unit, are essential for growth and maintenance of the skeleton. Clinical disorders of substrate availability (e.g. diabetes mellitus, anorexia nervosa and aging) cause osteoblast dysfunction ultimately leading to skeletal fragility and osteoporotic fractures. Conversely, anabolic treatments for osteoporosis enhance the work of the osteoblast by altering osteoblast metabolism. Emerging evidence supports glycolysis as the major metabolic pathway to meet ATP demand during osteoblast differentiation...
May 1, 2017: Endocrine Reviews
https://www.readbyqxmd.com/read/28469097/effects-of-rapamycin-on-clinical-manifestations-and-blood-lipid-parameters-in-different-preeclampsia-like-mouse-models
#16
Yan-Hong Yi, Zi Yang, Yi-Wei Han, Jing Huai
BACKGROUND: The pathogenesis of some types of preeclampsia is related to fatty acid oxidation disorders. Rapamycin can regulate fatty acid metabolism. This study aimed to investigate the effects of rapamycin on the clinical manifestations and blood lipid parameters in different preeclampsia-like mouse models. METHODS: Two preeclampsia-like mouse models and a control group were established: L-NA (injected with Nω-nitro-L-arginine methyl ester), LPS (injected with lipopolysaccharide), and the control group with normal saline (NS)...
May 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28468933/the-metabolic-syndrome-in-mice-overexpressing-neuropeptide-y-in-noradrenergic-neurons
#17
Liisa Ailanen, Suvi T Ruohonen, Laura Vähätalo, Katja Tuomainen, Kim Eerola, Henriikka Salomäki-Myftari, Matias Röyttä, Asta Laiho, Markku Ahotupa, Helena Gylling, Eriika Savontaus
A gain-of-function polymorphism in human neuropeptide Y (NPY) gene (rs16139) associates with metabolic disorders and earlier onset of type 2 diabetes (T2D). Similarly, mice overexpressing NPY in noradrenergic neurons (OE-NPY(DBH)) display obesity and impaired glucose metabolism. In this study, the metabolic syndrome -like phenotype was characterized and mechanisms of impaired hepatic fatty acid, cholesterol and glucose metabolism in pre-obese (2-month-old) and obese (4-7-month-old) OE-NPY(DBH) mice were elucidated...
May 3, 2017: Journal of Endocrinology
https://www.readbyqxmd.com/read/28451876/an-exploration-of-genetic-test-utilization-genetic-counseling-and-consanguinity-within-the-inborn-errors-of-metabolism-collaborative-ibemc
#18
Quinn P Stein, Cate Walsh Vockley, Mathew J Edick, Shaohui Zhai, Sally J Hiner, Rebecca S Loman, Laura Davis-Keppen, Taylor A Zuck, Cynthia A Cameron, Susan A Berry
The Inborn Errors of Metabolism Collaborative (IBEMC) includes clinicians from 29 institutions collecting data to enhance understanding of metabolic conditions diagnosable by newborn screening. Data collected includes hospitalizations, test results, services, and long-term outcomes. Through evaluation of this data, we sought to determine how frequently genetic counseling had been provided, how often genetic testing was performed, and also determine the consanguinity rate in this population. A data query was performed with the following elements abstracted/analyzed: current age, metabolic condition, whether genetic counseling was provided (and by whom), whether genetic testing was performed, and consanguinity...
April 27, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28450497/integrating-3-omics-data-analyze-rat-lung-tissue-of-copd-states-and-medical-intervention-by-delineation-of-molecular-and-pathway-alterations
#19
Jiansheng Li, Peng Zhao, Liping Yang, Ya Li, Yange Tian, Suyun Li
Chronic obstructive pulmonary disease (COPD) is a serious health problem. However, molecular pathogenesis of COPD remains unknown. Here, we explored the molecular effects of cigarette smoke and bacterial infection in lung tissues of COPD rats. We also investigated therapeutic effects of aminophylline (APL) on the COPD rats, and integrated transcriptome, proteome, and metabolome data for a global view of molecular mechanisms of COPD progression. Using molecular function and pathway analyses, the genes and proteins regulated in COPD and APL-treated rats were mainly attributed to oxidoreductase, antioxidant activity, energy and fatty acid metabolism...
April 27, 2017: Bioscience Reports
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
#20
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
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