keyword
MENU ▼
Read by QxMD icon Read
search

fatty acid oxidation disorder

keyword
https://www.readbyqxmd.com/read/29143862/a20-prevents-obesity-induced-development-of-cardiac-dysfunction
#1
Wenjing Xu, Cheng Wang, Minglu Liang, Long Chen, Qin Fu, Fengxiao Zhang, Yan Wang, Dan Huang, Kai Huang
Obesity and an increased free fatty acid (FFA) level are tightly linked, leading to aberrant oxidative stress, inflammation, apoptosis, and progression to cardiovascular disorders. A20 is a ubiquitin-modifying enzyme that plays a significant role in the negative regulation of inflammatory response. Here, we study the role of A20 in obesity-induced heart injury and explore the underlying mechanisms. A20 expression was first increased in mouse hearts after 4 weeks of a high-fat diet (HFD) and then was gradually decreased in the following 20 weeks...
November 16, 2017: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/29141966/nutritional-factors-affecting-adult-neurogenesis-and-cognitive-function
#2
REVIEW
Shibu M Poulose, Marshall G Miller, Tammy Scott, Barbara Shukitt-Hale
Adult neurogenesis, a complex process by which stem cells in the hippocampal brain region differentiate and proliferate into new neurons and other resident brain cells, is known to be affected by many intrinsic and extrinsic factors, including diet. Neurogenesis plays a critical role in neural plasticity, brain homeostasis, and maintenance in the central nervous system and is a crucial factor in preserving the cognitive function and repair of damaged brain cells affected by aging and brain disorders. Intrinsic factors such as aging, neuroinflammation, oxidative stress, and brain injury, as well as lifestyle factors such as high-fat and high-sugar diets and alcohol and opioid addiction, negatively affect adult neurogenesis...
November 2017: Advances in Nutrition
https://www.readbyqxmd.com/read/29137068/carnitine-acylcarnitine-translocase-deficiency-with-c-199-10-t-g-and-novel-c-1a-g-mutation-two-case-reports-and-brief-literature-review
#3
Hui-Ming Yan, Hao Hu, Aisha Ahmed, Bing-Bing Feng, Jing Liu, Zheng-Jun Jia, Hua Wang
RATIONALE: Carnitine-acylcarnitine translocate deficiency (CACTD) is a rare and life-threatening, autosomal recessive disorder of fatty acid β-oxidation characterized by hypoketotic hypoglycemia, hyperammonemia, cardiomyopathy, liver dysfunction, and muscle weakness; culminating in early death. To date, CACTD cases screened from the Chinese mainland population, especially patient with compound heterozygote with c.199-10T>G and a novel c.1A>G mutation in the SLC25A20 gene has never been described...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29124685/mitochondrial-trifunctional-protein-deficiency-severe-cardiomyopathy-and-cardiac-transplantation
#4
C Bursle, R Weintraub, C Ward, R Justo, J Cardinal, D Coman
We describe mitochondrial trifunctional protein deficiency (MTPD) in two male siblings who presented with severe cardiomyopathy in infancy. The first sibling presented in severe cardiac failure at 6 months of age and succumbed soon after. The second sibling came to attention after newborn screening identified a possible fatty acid oxidation defect. Dietary therapy and carnitine supplementation commenced in the neonatal period. Despite this the second child required cardiac transplantation at 3 years of age after a sudden and rapid decline in cardiac function...
November 10, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29104115/lowered-ipla2%C3%AE-activity-causes-increased-mitochondrial-lipid-peroxidation-and-mitochondrial-dysfunction-in-a-rotenone-induced-model-of-parkinson-s-disease
#5
Honglu Chao, Yinlong Liu, Xian Fu, Xiupeng Xu, Zhongyuan Bao, Chao Lin, Zheng Li, Yan Liu, Xiaoming Wang, Yongping You, Ning Liu, Jing Ji
iPLA2γ, calcium-independent phospholipase A2γ, discerningly hydrolyses glycerophospholipids to liberate free fatty acids. iPLA2γ-deficiency has been associated with abnormal mitochondrial function. More importantly, the iPLA2 family is causative proteins in mitochondrial neurodegenerative disorders such as parkinsonian disorders. However, the mechanisms by which iPLA2γ affects Parkinson's disease (PD) remain unknown. Mitochondrion stress has a key part in rotenone-induced dopaminergic neuronal degeneration...
November 2, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/29098735/glucose-and-lipid-metabolism-disorders-in-the-chickens-with-dexamethasone-induced-oxidative-stress
#6
Z-P Lv, Y-Z Peng, B-B Zhang, H Fan, D Liu, Y-M Guo
The purpose of this study was to investigate the effects of long-term treatment with dexamethasone (DEX) on the antioxidation and nutrition metabolism in broiler chickens. Broilers were placed on a high-nutrient diet for 41 days, and half were given orally DEX-supplemented water at 20 mg/L every other day from 19 to 41 days of age. DEX treatment downregulated superoxide dismutase activity as well as the mRNA expression of CuZn-superoxide dismutase and glutathione peroxidase with a decrease in GSH/GSSG ratio and an increase in malondialdehyde level in the liver of broilers...
November 3, 2017: Journal of Animal Physiology and Animal Nutrition
https://www.readbyqxmd.com/read/29095929/high-prevalence-of-carriers-of-variant-c-1528g-c-of-hadha-gene-causing-long-chain-3-hydroxyacyl-coa-dehydrogenase-deficiency-lchadd-in-the-population-of-adult-kashubians-from-north-poland
#7
Bogusław Nedoszytko, Alicja Siemińska, Dominik Strapagiel, Sławomir Dąbrowski, Marcin Słomka, Marta Sobalska-Kwapis, Błażej Marciniak, Jolanta Wierzba, Jarosław Skokowski, Marcin Fijałkowski, Roman Nowicki, Leszek Kalinowski
BACKGROUND/OBJECTIVES: The mitochondrial β-oxidation of fatty acids is a complex catabolic pathway. One of the enzymes of this pathway is the heterooctameric mitochondrial trifunctional protein (MTP), composed of four α- and β-subunits. Mutations in MTP genes (HADHA and HADHB), both located on chromosome 2p23, cause MTP deficiency, a rare autosomal recessive metabolic disorder characterized by decreased activity of MTP. The most common MTP mutation is long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency caused by the c...
2017: PloS One
https://www.readbyqxmd.com/read/29093663/selection-and-characterization-of-palmitic-acid-responsive-patients-with-an-oxphos-complex-i-defect
#8
Tom E J Theunissen, Mike Gerards, Debby M E I Hellebrekers, Florence H van Tienen, Rick Kamps, Suzanne C E H Sallevelt, Elvira N M M-D Hartog, Hans R Scholte, Robert M Verdijk, Kees Schoonderwoerd, Irenaeus F M de Coo, Radek Szklarczyk, Hubert J M Smeets
Mitochondrial disorders are genetically and clinically heterogeneous, mainly affecting high energy-demanding organs due to impaired oxidative phosphorylation (OXPHOS). Currently, effective treatments for OXPHOS defects, with complex I deficiency being the most prevalent, are not available. Yet, clinical practice has shown that some complex I deficient patients benefit from a high-fat or ketogenic diet, but it is unclear how these therapeutic diets influence mitochondrial function and more importantly, which complex I patients could benefit from such treatment...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29077970/identification-of-the-niacin-blunted-subgroup-of-schizophrenia-patients-from-mood-disorders-and-healthy-individuals-in-chinese-population
#9
Liya Sun, Xuhan Yang, Jie Jiang, Xiaowen Hu, Ying Qing, Dandan Wang, Tianqi Yang, Chao Yang, Juan Zhang, Ping Yang, Peng Wang, Changqun Cai, Jijun Wang, Lin He, Chunling Wan
Schizophrenia (SZ) is a devastating mental disease caused by complex genetic and environmental factors. The pathological process and clinical manifestation of SZ are heterogeneous among patients, which hampers precise diagnosis and treatment of the disease. Since no objective marker for SZ has been established today, to identify a subgroup of the patients with homogeneous biochemical traits will provide a new angle for both researchers and clinicians to understand and manage the disease. In this study, we employed the niacin skin-flushing test in Chinese population and confirmed a niacin-blunted subgroup of SZ patients distinguishable from mood disorders (MD) and normal individuals...
October 25, 2017: Schizophrenia Bulletin
https://www.readbyqxmd.com/read/29061958/%C3%AE-linolenic-acid-inhibits-receptor-activator-of-nf-%C3%AE%C2%BAb-ligand-induced-rankl-induced-osteoclastogenesis-and-prevents-inflammatory-bone-loss-via-downregulation-of-nuclear-factor-kappab-inducible-nitric-oxide-synthases-nf-%C3%AE%C2%BAb-inos-signaling-pathways
#10
Jiefu Song, Zhizhen Jing, Wei Hu, Jianping Yu, Xiaoping Cui
BACKGROUND Inflammation is a major cellular strain causing increased risk of osteo-degenerative diseases. Omega-3 fatty acids have been great source in suppressing inflammation. We investigated the effect of α-linolenic acid (ALA) on RANKL-stimulated osteoclast differentiation, LPS-induced and ovariectomized bone loss in mice models. MATERIAL AND METHODS The bone marrow macrophages (BMMs) were isolated from femurs of ICR mice, stimulated with RANKL, and treated with ALA (100, 200, 300 µM). Major analytical methods include histological analysis, osteoclasts viability assay, serum cytokines and chemokines ELISA, and gene expression by qPCR...
October 24, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29039165/-screening-for-fatty-acid-oxidation-disorders-of-newborns-in-zhejiang-province-prevalence-outcome-and-follow-up
#11
Jing Zheng, Yu Zhang, Fang Hong, Jianbin Yang, Fan Tong, Huaqing Mao, Xiaolei Huang, Xuelian Zhou, Rulai Yang, Zhengyan Zhao, Xinwen Huang
OBJECTIVE: To screen fatty acid oxidation disorders (FAOD) in newborns in Zhejiang province. METHODS: A total of 1 861 262 newborns were screened for FAOD in Neonatal Screening Center of Zhejiang Province during January 2009 and December 2016. The blood samples from newborns were screened by tandem mass spectrometry, and diagnosis of FAOD was confirmed by urine organic acid measurement combined with genetic analysis. The prognosis and follow-up of patients with FAOD were also evaluated...
May 25, 2017: Zhejiang da Xue Xue Bao. Yi Xue Ban, Journal of Zhejiang University. Medical Sciences
https://www.readbyqxmd.com/read/29034200/promising-effect-of-rosa-damascena-extract-on-high-fat-diet-induced-nonalcoholic-fatty-liver
#12
Ida Davoodi, Roja Rahimi, Mohammad Abdollahi, Fatemeh Farzaei, Mohammad Hosein Farzaei, Zahra Memariani, Fariba Najafi
NAFLD is a chronic liver disease that affects a high proportion of the world's population which causes metabolic and hepatic damages. Rosa damascena Mill is traditionally used as a dietary supplement for liver disorders. This study was carried out to determine the beneficial effect of standardized extract of R. damascena on animal model of nonalcoholic fatty liver disease (NAFLD). NAFLD was induced by high-fat diet (HFD) in Wistar rats. HFD rats showed an increase (p < 0.05) in the plasma lipid levels of total cholesterol (TC), triglyceride (TG), low-density lipoprotein (LDL), and reduced the high-density lipoprotein (HDL) levels...
October 2017: Journal of Traditional and Complementary Medicine
https://www.readbyqxmd.com/read/29030856/mitochondrial-3-hydroxy-3-methylglutaryl-coa-synthase-deficiency-unique-presenting-laboratory-values-and-a-review-of-biochemical-and-clinical-features
#13
Erin Conboy, Filippo Vairo, Matthew Schultz, Katherine Agre, Ross Ridsdale, David Deyle, Devin Oglesbee, Dimitar Gavrilov, Eric W Klee, Brendan Lanpher
We report an 8-month-old infant with decreased consciousness after a febrile episode and reduced oral intake. He was profoundly acidotic but his lactate was normal. Serum triglycerides were markedly elevated and HDL cholesterol was very low. The urine organic acid analysis during the acute episode revealed a complex pattern of relative hypoketotic dicarboxylic aciduria, suggestive of a potential fatty acid oxidation disorder. MRI showed extensive brain abnormalities concerning for a primary energy deficiency...
October 14, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29027371/differential-proteomic-analysis-of-dimethylnitrosamine-dmn-induced-liver-fibrosis
#14
Xiujie Liu, Rongji Dai, Ming Ke, Imran Suheryani, Weiwei Meng, Yulin Deng
Liver fibrosis is a common pathological feature of many chronic liver diseases. To characterize the entire panorama of proteome changes in DMN-induced liver fibrosis, iTRAQ-based differential proteomic analysis was performed with DMN-induced liver fibrosis rats. A total of 4155 confidently identified proteins were found, with 365 proteins showing significant changes (fold changes of >1.5 or < 0.67, P < 0.05). In metabolic activation, proteins assigned to drug metabolism enzymes (e.g., CYP2D1) changed, suggesting that the liver protection mechanism was activated to relieve DMN toxicity...
October 13, 2017: Proteomics
https://www.readbyqxmd.com/read/29018783/effects-of-erythrocyte-membrane-polyunsaturated-fatty-acids-in-overweight-obese-and-morbidly-obese-korean-women
#15
Ha-Neul Choi, Jung-Eun Yim
BACKGROUND: The levels of erythrocyte polyunsaturated fatty acids (FAs) may be associated with obesity, metabolic syndrome, and cancer. Thus, we investigated the association between erythrocyte n-3 and n-6 FA composition, body mass index (BMI), and biochemical profiles. METHODS: The body composition, dietary intake, and blood parameters, including serum lipid, glucose, insulin, adipokines, oxidative stress, and erythrocyte FA, were assessed in 66 overweight and obese women (average age, 43...
September 2017: Journal of Cancer Prevention
https://www.readbyqxmd.com/read/28987035/the-psychoneuroimmunological-role-of-omega-3-polyunsaturated-fatty-acids-in-major-depressive-disorder-and-bipolar-disorder
#16
Ian Hunter Rutkofsky, Anser Saeed Khan, Sindhu Sahito, Vikram Kumar
Context • Psychoneuroimmunology is the interdisciplinary study that links behavioral health with the neuroendocrinal system and investigates that link's bidirectional impact on the human immune system. Mechanistic studies have shown how omega-3 polyunsaturated fatty acids (PUFAs), like those found in fish oil, can modulate key pathways involved in inflammation, sympathetic activity, oxidative stress, transcription factors, and inflammatory cytokine production. Objective • The research team intended to investigate the effects that PUFAs have on the brain and the immune system, including the effects of proinflammatory cytokines and oxidative stress, and their therapeutic benefits in major depressive disorder (MDD) and bipolar disorder, either as an alternative monotherapy or a complementary adjunct treatment...
July 2017: Advances in Mind-body Medicine
https://www.readbyqxmd.com/read/28980402/is-palmitoleic-acid-a-plausible-non-pharmacological-strategy-to-prevent-or-control-chronic-metabolic-and-inflammatory-disorders
#17
REVIEW
Camila O Souza, Gretchen K Vannice, José C Rosa Neto, Philip C Calder
Although dietary fatty acids can modulate metabolic and immune responses, the effects of palmitoleic acid (16:1n-7) remain unclear. Since this monounsaturated fatty acid was described as a lipokine, studies with cell culture and rodent models have suggested it enhances whole body insulin sensitivity, stimulates insulin secretion by β-cells, increases hepatic fatty acid oxidation, improves the blood lipid profile, and alters macrophage differentiation. However, human studies report elevated blood levels of palmitoleic acid in people with obesity and metabolic syndrome...
October 5, 2017: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/28980192/clinical-biochemical-and-molecular-features-in-37-saudi-patients-with-very-long-chain-acyl-coa-dehydrogenase-deficiency
#18
Abdulrahman Obaid, Marwan Nashabat, Majid Alfadhel, Ali Alasmari, Fuad Al Mutairi, Abdulrahman Alswaid, Eissa Faqeih, Aziza Mushiba, Marwah Albanyan, Maryam Alalwan, Deborah Marsden, Wafaa Eyaid
BACKGROUND: Very long chain acyl CoA dehydrogenase (VLCAD) deficiency (OMIM#201475) is an autosomal recessive disorder of fatty acid beta oxidation caused by defect in the ACADVL. The aim of this study was to analyze the clinical, biochemical, and molecular features of VLCAD deficiency in Saudi Arabia, including the treatment and outcome. METHODS: We carried out a retrospective chart review analysis of 37 VLCAD deficiency patients from two tertiary centers in Saudi Arabia, over a 14-year period (2002-2016)...
October 5, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28978222/the-eicosanoids-redox-regulated-lipid-mediators-in-immunometabolic-disorders
#19
Ana Carolina Araujo, Craig E Wheelock, Jesper Z Haeggström
SIGNIFICANCE: The oxidation of arachidonic acid via cyclooxygenase and lipoxygenase activity to produce eicosanoids during inflammation is a well-known biosynthetic pathway. These lipid mediators are involved in fever, pain and thrombosis and are generated from a variety of cell types as well as through interactions between immune cells and epithelial/endothelial cells. Metabolic disorders including hyperlipidemia, hypertension, and diabetes are associated with chronic low-grade inflammation, impacting the immune system and promoting a variety of chronic diseases...
October 5, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28956034/metabolomics-reveal-mitochondrial-and-fatty-acid-metabolism-disorders-that-contribute-to-the-development-of-dkd-in-t2dm-patients
#20
Ling Li, Chengshi Wang, Hongliu Yang, Shuyun Liu, Yanrong Lu, Ping Fu, Jingping Liu
Diabetic kidney disease (DKD) is the leading cause of ESRD; however, early intervention can greatly prevent the progression of DKD; thus, sensitive biomarkers for DKD are still required. This study was aimed at the identification of potential biomarkers and revelation of underlying pathways in DKD patients by non-targeted metabolomics. Gas chromatography-mass spectrometry was used to analyze urine obtained from the control and type 2 diabetes mellitus (T2DM) and DKD patients, and the renal histological changes in DKD patients were assessed...
October 24, 2017: Molecular BioSystems
keyword
keyword
41234
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"