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fatty acid oxidation disorder

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https://www.readbyqxmd.com/read/28239368/connection-of-nicotine-to-diet-induced-obesity-and-non-alcoholic-fatty-liver-disease-cellular-and-mechanistic-insights
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
February 3, 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
#8
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
#9
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
#10
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...
25, 2017: Oncotarget
https://www.readbyqxmd.com/read/28117803/analyzing-beneficial-effects-of-nutritional-supplements-on-intestinal-epithelial-barrier-functions-during-experimental-colitis
#11
Hilda Vargas Robles, Karla Fabiola Castro Ochoa, Porfirio Nava, Angélica Silva Olivares, Mineko Shibayama, Michael Schnoor
Inflammatory bowel diseases (IBD), including Crohn's disease and ulcerative colitis, are chronic relapsing disorders of the intestines. They cause severe problems, such as abdominal cramping, bloody diarrhea, and weight loss, in affected individuals. Unfortunately, there is no cure yet, and treatments only aim to alleviate symptoms. Current treatments include anti-inflammatory and immunosuppressive drugs that may cause severe side effects. This warrants the search for alternative treatment options, such as nutritional supplements, that do not cause side effects...
January 5, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28112527/identification-of-a-novel-hadhb-gene-mutation-in-an-iranian-patient-with-mitochondrial-trifunctional-protein-deficiency
#12
Mahdiyeh Shahrokhi, Mohammad Shafiei, Hamid Galehdari, Gholamreza Shariati
INTRODUCTION: Mitochondrial trifunctional protein (MTP) is a hetero-octamer composed of eight parts (subunits): four α-subunits containing LCEH (long-chain 2,3-enoyl-CoA  hydratase) and LCHAD (long-chain 3-hydroxyacyl CoA dehydrogenase) activity, and four β-subunits that possess LCKT (long-chain  3-ketoacyl-CoA thiolase) activity which catalyzes three out of four steps in β-oxidation spiral of long-chain fatty acid. Its deficiency is an autosomal recessive disorder that causes a clinical spectrum of diseases...
January 2017: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/28111084/prepregnancy-maternal-diabetes-combined-with-obesity-impairs-placental-mitochondrial-function-involving-nrf2-are-pathway-and-detrimentally-alters-metabolism-of-offspring
#13
Yang Duan, Fuqiang Sun, Shengshun Que, Yueqin Li, Suyan Yang, Geli Liu
Metabolic disorders usually increase the level of reactive oxygen species (ROS) and damage mitochondrial function. The placenta supplies nutrients and hormonal signals to the fetus for regulating fetal metabolism, and is also prone to injury by oxidants. The aim of this study was to determine the effect of pre-existing maternal type 2 diabetes mellitus (DM) combined with obesity on placental mitochondrial function and metabolism disorders of offspring. The study included 96 pregnant women. The women were put into the following groups: healthy women (control, n=24), women with DM (DM, n=24), women with obesity (OB, n=24) and women with both DM and obesity (DM+OB, n=24)...
January 19, 2017: Obesity Research & Clinical Practice
https://www.readbyqxmd.com/read/28108035/feline-hepatic-lipidosis
#14
REVIEW
Chiara Valtolina, Robert P Favier
Feline hepatic lipidosis (FHL) is a common and potentially fatal liver disorder. Although the pathophysiologic mechanisms of FHL remain elusive, there is an imbalance between the influx of fatty acids from peripheral fat stores into the liver, de novo liposynthesis, and the rate of hepatic oxidation and dispersal of hepatic TAG via excretion of very-low density lipoproteins. The diagnosis of FHL is based on anamnestic, clinical, and clinicopathologic findings, associated with diagnostic imaging of the liver, and cytology, or histological examination of liver biopsies...
January 18, 2017: Veterinary Clinics of North America. Small Animal Practice
https://www.readbyqxmd.com/read/28104914/docosahexaenoic-acid-mediated-protein-aggregates-may-reduce-proteasome-activity-and-delay-myotube-degradation-during-muscle-atrophy-in-vitro
#15
Seung Kyun Shin, Ji Hyeon Kim, Jung Hoon Lee, Young Hoon Son, Min Wook Lee, Hak Joong Kim, Sue Ah Noh, Kwang Pyo Kim, In-Gyu Kim, Min Jae Lee
Proteasomes are the primary degradation machinery for oxidatively damaged proteins that compose a class of misfolded protein substrates. Cellular levels of reactive oxygen species increase with age and this cellular propensity is particularly harmful when combined with the age-associated development of various human disorders including cancer, neurodegenerative disease and muscle atrophy. Proteasome activity is reportedly downregulated in these disease conditions. Herein, we report that docosahexaenoic acid (DHA), a major dietary omega-3 polyunsaturated fatty acid, mediates intermolecular protein cross-linkages through oxidation, and the resulting protein aggregates potently reduce proteasomal activity both in vitro and in cultured cells...
January 20, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/28095910/profile-of-bioactive-compounds-in-nymphaea-alba-l-leaves-growing-in-egypt-hepatoprotective-antioxidant-and-anti-inflammatory-activity
#16
Riham Omar Bakr, Mona Mohamed El-Naa, Soumaya Saad Zaghloul, Mahmoud Mohamed Omar
BACKGROUND: Nymphaea alba L. represents an interesting field of study. Flowers have antioxidant and hepatoprotective effects, rhizomes constituents showed cytotoxic activity against liver cell carcinoma, while several Nymphaea species have been reported for their hepatoprotective effects. Leaves of N. alba have not been studied before. Therefore, in this study, in-depth characterization of the leaf phytoconstituents as well as its antioxidant and hepatoprotective activities have been performed where N...
January 17, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28088395/lactate-and-lactate-pyruvate-ratio-in-the-diagnosis-and-outcomes-of-pediatric-acute-liver-failure
#17
Amy G Feldman, Ronald J Sokol, Regina M Hardison, Estella M Alonso, Robert H Squires, Michael R Narkewicz
OBJECTIVES: To assess the accuracy of blood lactate and lactate: pyruvate molar ratio (L:P) as a screen for mitochondrial, respiratory chain, or fatty acid oxidation disorders in children with pediatric acute liver failure (PALF); to determine whether serum lactate ≥ 2.5 mmol/L or L:P  ≥ 25 correlated with biochemical variables of clinical severity; and to determine whether lactate or L:P is associated with clinical outcome at 21 days. STUDY DESIGN: Retrospective review of demographic, clinical, laboratory, and outcome data for PALF study group participants who had lactate and pyruvate levels collected on the same day...
March 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28070495/an-atypical-presentation-of-acad9-deficiency-diagnosis-by-whole-exome-sequencing-broadens-the-phenotypic-spectrum-and-alters-treatment-approach
#18
H K Aintablian, V Narayanan, N Belnap, K Ramsey, T A Grebe
Acyl-CoA dehydrogenase 9 (ACAD9), linked to chromosome 3q21.3, is one of a family of multimeric mitochondrial flavoenzymes that catalyze the degradation of fatty acyl-CoA from the carnitine shuttle via β-oxidation (He et al. 2007). ACAD9, specifically, is implicated in the processing of palmitoyl-CoA and long-chain unsaturated substrates, but unlike other acyl-CoA dehydrogenases (ACADs), it has a significant role in mitochondrial complex I assembly (Nouws et al. 2010 & 2014). Mutations in this enzyme typically cause mitochondrial complex I deficiency, as well as a mild defect in long chain fatty acid metabolism (Haack et al...
March 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28066829/the-multifaceted-role-of-nrf2-in-mitochondrial-function
#19
REVIEW
Kira M Holmström, Rumen V Kostov, Albena T Dinkova-Kostova
The transcription factor nuclear factor erythroid 2 p45-related factor 2 (Nrf2) is the master regulator of the cellular redox homeostasis. Nrf2 target genes comprise of a large network of antioxidant enzymes, proteins involved in xenobiotic detoxification, repair and removal of damaged proteins, inhibition of inflammation, as well as other transcription factors. In recent years it has emerged that as part of its role as a regulator of cytoprotective gene expression, Nrf2 impacts mitochondrial function. Increased Nrf2 activity defends against mitochondrial toxins...
December 2016: Curr Opin Toxicol
https://www.readbyqxmd.com/read/28054209/long-term-outcome-of-expanded-newborn-screening-at-boston-children-s-hospital-benefits-and-challenges-in-defining-true-disease
#20
Yuval E Landau, Susan E Waisbren, Lawrence M A Chan, Harvey L Levy
INTRODUCTION: There is no universal consensus of the disorders included in newborn screening programs. Few studies so far, mostly short-term, have compared the outcome of disorders detected by expanded newborn screening (ENBS) to the outcome of the same disorders detected clinically. METHODS: We compared the clinical and neurodevelopmental outcomes in patients with metabolic disorders detected by ENBS, including biotinidase testing, with those detected clinically and followed at the Metabolism Clinic at Boston Children's Hospital...
March 2017: Journal of Inherited Metabolic Disease
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