keyword
MENU ▼
Read by QxMD icon Read
search

fatty acid oxidation disorder

keyword
https://www.readbyqxmd.com/read/29768358/spastic-paraparesis-caused-by-x-linked-adrenoleukodystrophy-mimicking-vacuolar-myelopathy-in-a-human-immunodeficiency-virus-patient-a-case-report
#1
Jin-Sung Park, Donghwi Park
RATIONALE: Vacuolar myelopathy is one of most common cause of spastic paresis in patients with human immunodeficiency virus (HIV) infection. However, X-linked adrenoleukodystrophy (X-ALD), which is a metabolic disorder caused by impairment of peroxisomal beta-oxidation of very-long-chain fatty acids (VLCFA), also manifests as various neurological deteriorations including adult onset spastic paraparesis. To the best of our knowledge, there has been no report of newly developed spastic paresis due to X-ALD in a patient with HIV infection...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29757724/neuroimaging-findings-of-organic-acidemias-and-aminoacidopathies
#2
Nihaal Reddy, Sonia F Calloni, Hilary J Vernon, Eugen Boltshauser, Thierry A G M Huisman, Bruno P Soares
Although individual cases of inherited metabolic disorders are rare, overall they account for a substantial number of disorders affecting the central nervous system. Organic acidemias and aminoacidopathies include a variety of inborn errors of metabolism that are caused by defects in the intermediary metabolic pathways of carbohydrates, amino acids, and fatty acid oxidation. These defects can lead to the abnormal accumulation of organic acids and amino acids in multiple organs, including the brain. Early diagnosis is mandatory to initiate therapy and prevent permanent long-term neurologic impairments or death...
May 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29755356/anti-parkinson-potential-of-silymarin-mechanistic-insight-and-therapeutic-standing
#3
REVIEW
Hammad Ullah, Haroon Khan
Parkinson's disease (PD) involves aggregation of α-synuclein and progressive loss of dopaminergic neurons. Pathogenesis of PD may also be related to one's genetic background. PD is most common among geriatric population and approximately 1-2% of population suffers over age 65 years. Currently no successful therapies are in practice for the management of PD and available therapies tend to decrease the symptoms of PD only. Furthermore, these are associated with diverse range of adverse effects profile. The neuroprotective effects of polyphenols are widely studied and documented...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29743968/altered-redox-homeostasis-in-branched-chain-amino-acid-disorders-organic-acidurias-and-homocystinuria
#4
REVIEW
Eva Richard, Lorena Gallego-Villar, Ana Rivera-Barahona, Alfonso Oyarzábal, Belén Pérez, Pilar Rodríguez-Pombo, Lourdes R Desviat
Inborn errors of metabolism (IEMs) are a group of monogenic disorders characterized by dysregulation of the metabolic networks that underlie development and homeostasis. Emerging evidence points to oxidative stress and mitochondrial dysfunction as major contributors to the multiorgan alterations observed in several IEMs. The accumulation of toxic metabolites in organic acidurias, respiratory chain, and fatty acid oxidation disorders inhibits mitochondrial enzymes and processes resulting in elevated levels of reactive oxygen species (ROS)...
2018: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/29742447/the-discovery-of-nitro-fatty-acids-as-products-of-metabolic-and-inflammatory-reactions-and-mediators-of-adaptive-cell-signaling
#5
REVIEW
Bruce A Freeman, Valerie B O'Donnell, Francisco J Schopfer
Foundational advances in eicosanoid signaling, the free radical biology of oxygen and nitric oxide and mass spectrometry all converged to enable the discovery of nitrated unsaturated fatty acids. Due to the unique biochemical characteristics of fatty acid nitroalkenes, these species undergo rapid and reversible Michael addition of biological nucleophiles such as cysteine, leading to the post-translational modification of low molecular weight and protein thiols. This capability has led to the present understanding that nitro-fatty acid reaction with the alkylation-sensitive cysteine proteome leads to physiologically-beneficial alterations in transcriptional regulatory protein function, gene expression and in vivo rodent model responses to metabolic and inflammatory stress...
May 6, 2018: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/29731937/carnitine-palmitoyltransferase-ii-deficiency-cpt-ii-followed-by-rhabdomyolysis-and-acute-kidney-injury
#6
Nikola Gjorgjievski, Pavlina Dzekova-Vidimliski, Zvezdana Petronijevic, Gjulsen Selim, Petar Dejanov, Liljana Tozija, Aleksandar Sikole
BACKGROUND: Carnitine palmitoyltransferase II deficiency (CPT II) is an autosomal recessive disorder and the most common inherited disorder of mitochondrial long-chain fatty acid oxidation, characterised by attacks of myalgia and myoglobinuria. The most common "classic" myopathic form occurs in young adults and is characterised by recurrent episodes of rhabdomyolysis triggered by prolonged exercise, fasting or febrile illness. CASE PRESENTATION: We present a case of a 22-year-old Caucasian male admitted to our hospital with fever, dyspnea, fatigue, myalgia and dark urine (brown-coloured)...
April 15, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29729919/maternal-late-gestation-metabolic-stress-is-associated-with-changes-in-immune-and-metabolic-responses-of-dairy-calves
#7
Tahlia Ling, Marta Hernandez-Jover, Lorraine M Sordillo, Angel Abuelo
Metabolic stress in periparturient dairy cows is characterized by excessive lipid mobilization, inflammation, and oxidative stress that is associated with immune dysfunction. Thus, metabolic stress around the time calving is linked to the development of various early-lactation health disorders. Maternal status during late pregnancy can have carryover effects on several health and production variables of neonatal calves. However, the effects of metabolic stress during gestation on metabolic and immune responses of newborn calves remain unknown...
May 2, 2018: Journal of Dairy Science
https://www.readbyqxmd.com/read/29727630/theacrine-protects-against-nonalcoholic-fatty-liver-disease-by-regulating-acylcarnitine-metabolism
#8
Guo-En Wang, Yi-Fang Li, Yu-Jia Zhai, Lian Gong, Jing-Yu Tian, Mo Hong, Nan Yao, Yan-Ping Wu, Hiroshi Kurihara, Rong-Rong He
OBJECTIVE: Acylcarnitine metabolism disorder contributes significantly to the pathogenesis of nonalcoholic fatty liver disease (NAFLD). There are, however, few ideal medications for NAFLD, which work by targeting acylcarnitine metabolism. The aim of this study was to investigate the protective effects of theacrine, a rare purine alkaloid isolated from Camellia assamica var. kucha, against acylcarnitine metabolism disorder in NAFLD. METHODS: The pharmacological activities of theacrine were studied using high-fat diet (HFD)-fed ApoE-/- and C57BL/6 J mice models...
May 1, 2018: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/29725308/the-exposure-to-different-photoperiods-strongly-modulates-the-glucose-and-lipid-metabolisms-of-normoweight-fischer-344-rats
#9
Roger Mariné-Casadó, Cristina Domenech-Coca, Josep M Del Bas, Cinta Bladé, Lluís Arola, Antoni Caimari
Seasonal variations in day length trigger clear changes in the behavior, growth, food intake, and reproductive status of photoperiod-sensitive animals, such as Fischer 344 rats. However, there is little information about the effects of seasonal fluctuations in day length on glucose and lipid metabolisms and their underlying mechanisms in this model. To gain knowledge on these issues, three groups of male Fischer 344 rats were fed with a standard diet and exposed to different photoperiods for 14 weeks: normal photoperiod (L12, 12 h light/day), long photoperiod (L18, 18 h light/day), and short photoperiod (L6, 6 h light/day)...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29723692/a-learning-collaborative-approach-increases-specificity-of-diagnosis-of-acute-liver-failure-in-pediatric-patients
#10
Michael R Narkewicz, Simon Horslen, Regina M Hardison, Benjamin L Shneider, Norberto Rodriguez-Baez, Estella M Alonso, Vicky L Ng, Mike A Leonis, Kathleen M Loomes, David A Rudnick, Philip Rosenthal, Rene Romero, Girish C Subbarao, Ruosha Li, Steven H Belle, Robert H Squires
BACKGROUND & AIMS: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)-they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation...
April 30, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29710765/a-vitamin-e-enriched-antioxidant-diet-interferes-with-the-acute-adaptation-of-the-liver-to-physical-exercise-in-mice
#11
Miriam Hoene, Martin Irmler, Johannes Beckers, Martin Hrabě de Angelis, Hans-Ulrich Häring, Cora Weigert
Physical exercise is beneficial for general health and is an effective treatment for metabolic disorders. Vitamin E is widely used as dietary supplement and is considered to improve non-alcoholic fatty liver disease by reducing inflammation and dyslipidemia. However, increased vitamin E intake may interfere with adaptation to exercise training. Here, we explored how vitamin E alters the acute exercise response of the liver, an organ that plays an essential metabolic role during physical activity. Mice fed a control or an α-tocopherol-enriched diet were subjected to a non-exhaustive treadmill run...
April 28, 2018: Nutrients
https://www.readbyqxmd.com/read/29710478/essential-role-of-nuclear-receptors-for-the-evaluation-of-the-benefits-of-bioactive-herbal-extracts-on-liver-function
#12
REVIEW
Fengling Wang, Yifan Wu, Xiaoting Xie, Jing Sun, Weidong Chen
Nuclear receptors (NRs), ligand-dependent transcription factors, play important regulatory roles in diverse metabolic processes including hepatic lipid, glucose and bile acids (BAs) metabolism involved in liver and metabolic disorders. Therefore, NRs are attractive targets for treatment or prevention of liver diseases. However, the cost-effectiveness and safety of currently available synthetic NRs agonists or antagonists are under question. Herbal extracts and their derivatives with superior cost-effectiveness and less toxicity have attracted much attention as the potential option for NRs agonists or antagonists...
March 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29706933/the-role-for-myc-in-coordinating-glycolysis-oxidative-phosphorylation-glutaminolysis-and-fatty-acid-metabolism-in-normal-and-neoplastic-tissues
#13
REVIEW
Eric S Goetzman, Edward V Prochownik
That cancer cells show patterns of metabolism different from normal cells has been known for over 50 years. Yet, it is only in the past decade or so that an appreciation of the benefits of these changes has begun to emerge. Altered cancer cell metabolism was initially attributed to defective mitochondria. However, we now realize that most cancers do not have mitochondrial mutations and that normal cells can transiently adopt cancer-like metabolism during periods of rapid proliferation. Indeed, an encompassing, albeit somewhat simplified, conceptual framework to explain both normal and cancer cell metabolism rests on several simple premises...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29704496/salusin-%C3%AE-attenuates-hepatic-steatosis-and-atherosclerosis-in-high-fat-diet-fed-low-density-lipoprotein-receptor-deficient-mice
#14
Kun Tang, Fei Wang, Yi Zeng, XueMeng Chen, XiaoLe Xu
Salusin-α is an endogenous bioactive peptide and likely to prevent atherosclerosis. But its protective effect against atherosclerosis in vivo remains poorly understood. The aim of the present study was to determine the potential effects of salusin-α on atherosclerosis and its associated metabolic disorders in high fat diet (HFD)-fed low density lipoprotein receptor deficient (LDLr-/- ) mice, and also explore the possible underlying mechanisms involved. Our data showed that after 12 weeks treatment, salusin-α ameliorated HFD-induced weight gain, hyperlipidemia, and serum levels of interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α)...
April 25, 2018: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29689727/vascular-cognitive-impairment-and-the-gut-microbiota
#15
Sinian Li, Yiming Shao, Kanglan Li, Changmei HuangFu, Wenjie Wang, Zhou Liu, Zhiyou Cai, Bin Zhao
Vascular cognitive impairment (VCI), the second most common cause of dementia in elderly people, is a term that refers to all forms of cognitive disorders that can be attributed to cerebrovascular disease such as manifestations of discrete infarctions, brain hemorrhages, and white matter lesions. The gut microbiota (GM) has emerged recently as an essential player in the development of VCI. The GM may affect the brain's physiological, behavioral, and cognitive functions through the brain-gut axis via neural, immune, endocrine, and metabolic pathways...
April 17, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29681858/aqueous-extract-of-black-maca-prevents-metabolism-disorder-via-regulating-the-glycolysis-gluconeogenesis-tca-cycle-and-ppar%C3%AE-signaling-activation-in-golden-hamsters-fed-a-high-fat-high-fructose-diet
#16
Wenting Wan, Hongxiang Li, Jiamei Xiang, Fan Yi, Lijia Xu, Baoping Jiang, Peigen Xiao
Maca ( Lepidium meyenii Walpers) has been used as a dietary supplement and ethnomedicine for centuries. Recently, maca has become a high profile functional food worldwide because of its multiple biological activities. This study is the first explorative research to investigate the prevention and amelioration capacity of the aqueous extract of black maca (AEM) on high-fat, high-fructose diet (HFD)-induced metabolism disorder in golden hamsters and to identify the potential mechanisms involved in these effects...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29679909/mechanisms-of-the-active-components-from-korean-pine-nut-preventing-and-treating-d-galactose-induced-aging-rats
#17
Jianmei Zhang, Weiwei Lin, Ruijun Wu, Yanmeng Liu, Kai Zhu, Jinyu Ren, Sufang Zhang, Xiaomei Ling
Age-related neuronal injury and oxidative damage are the predominant factors for neurodegenerative diseases like Alzheimer's disease (AD). The aim of this study was to explore whether chronic administration of d-galactose (d-gal) can cause neuronal injury and oxidative damage, and to investigate the neuroprotective and antioxidative effects of the active components (UPNO-1) from Korean pine nut (Pinus koraiensis). Two dosing regimens were designed, one for the evaluation of preventive effects in which the rats were simultaneously administrated d-gal and UPNO-1/fishoil for 12 weeks, the other for the evaluation of therapeutic effects in which the rats were given d-gal for 8 weeks before treated with UPNO-1/selegiline for 8 weeks...
April 18, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29674247/apple-peel-polyphenols-reduce-mitochondrial-dysfunction-in-mice-with-dss-induced-ulcerative-colitis
#18
Pantea Rahmani Yeganeh, Jade Leahy, Schohraya Spahis, Natalie Patey, Yves Desjardins, Denis Roy, Edgard Delvin, Carole Garofalo, Jean-Philippe Leduc-Gaudet, David St-Pierre, Jean-François Beaulieu, André Marette, Gilles Gouspillou, Emile Levy
Inflammatory bowel diseases (IBDs) are multifaceted and relapsing immune disorders, which necessitate long-term dependence on powerful drugs. As the use of natural product-based therapies has emerged as a promising intervention, the present study aimed to further characterize dried apple peel powder (DAPP) mechanisms of action and evaluate the preventive and curative effects of DAPP on mitochondrial functions in a murine model. Induction of intestinal inflammation in mice is performed by oral administration of the dextran sodium sulfate (DSS) at 2...
March 21, 2018: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/29664677/silac-based-quantitative-proteomic-analysis-of-the-livers-of-spontaneous-obese-and-diabetic-rhesus-monkeys
#19
Junlong Wang, Shimeng Xu, Jing Gao, Linqiang Zhang, Zhiguo Zhang, Wenhui Yang, Yunhai Li, Shasha Liao, Hu Zhou, Pingsheng Liu, Bin Liang
Type 2 diabetes mellitus (T2DM) is a severely metabolic disorder that affects above 10% worldwide population. Obesity is a major cause of insulin resistance and contributes to the development of T2DM. Liver is an essential metabolic organ that plays crucial roles in the pathogenesis of obesity and diabetes. However, the underlying mechanisms of liver in the transition of obesity to diabetes are not fully understood. Nonhuman primate (NHP) rhesus monkey is an appropriate animal for research of human diseases...
April 17, 2018: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29648643/mutations-in-glycyl-trna-synthetase-impair-mitochondrial-metabolism-in-neurons
#20
Veronika Boczonadi, Kathrin Meyer, Humberto Gonczarowska-Jorge, Helen Griffin, Andreas Roos, Marina Bartsakoulia, Boglarka Bansagi, Giulia Ricci, Fanni Palinkas, René P Zahedi, Francesco Bruni, Brian Kaspar, Hanns Lochmüller, Kym M Boycott, Juliane S Müller, Rita Horvath
The nuclear-encoded glycyl-tRNA synthetase gene (GARS) is essential for protein translation in both cytoplasm and mitochondria. In contrast, different genes encode the mitochondrial and cytosolic forms of most other tRNA synthetases. Dominant GARS mutations were described in inherited neuropathies, while recessive mutations cause severe childhood-onset disorders affecting skeletal muscle and heart. The downstream events explaining tissue-specific phenotype-genotype relations remained unclear. We investigated the mitochondrial function of GARS in human cell lines and in the GarsC210R mouse model...
April 10, 2018: Human Molecular Genetics
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"