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https://www.readbyqxmd.com/read/28726122/beta-ketothiolase-deficiency-presenting-with-metabolic-stroke-after-a-normal-newborn-screen-in-two-individuals
#1
Monica H Wojcik, Klaas J Wierenga, Lance H Rodan, Inderneel Sahai, Sacha Ferdinandusse, Casie A Genetti, Meghan C Towne, Roy W A Peake, Philip M James, Alan H Beggs, Catherine A Brownstein, Gerard T Berry, Pankaj B Agrawal
Beta-ketothiolase (mitochondrial acetoacetyl-CoA thiolase) deficiency is a genetic disorder characterized by impaired isoleucine catabolism and ketone body utilization that predisposes to episodic ketoacidosis. It results from biallelic pathogenic variants in the ACAT1 gene, encoding mitochondrial beta-ketothiolase. We report two cases of beta-ketothiolase deficiency presenting with acute ketoacidosis and "metabolic stroke." The first patient presented at 28 months of age with metabolic acidosis and pallidal stroke in the setting of a febrile gastrointestinal illness...
July 20, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28724414/effects-of-feeding-drunken-horse-grass-infected-with-epichlo%C3%A3-gansuensis-endophyte-on-animal-performance-clinical-symptoms-and-physiological-parameters-in-sheep
#2
Ying Liang, Hucheng Wang, Chunjie Li, Zhibiao Nan, Fadi Li
BACKGROUND: Many reports showed that grass-endophyte symbiosis induced livestock poisoned. Yet, there is no study evaluating clinical symptoms and physiological parameters in sheep fed Epichloë gansuensis endophyte-infected grass. The objective of the present study was to investigate these indexes by feeding sheep with endophyte-infected A. inebrians (E+ Group) or endophyte-free A. inebrians (E- Group) drunken horse grass or alfalfa hay (Control Group). RESULTS: The Epichloë endophyte caused obvious toxicity symptoms in the sheep fed E+ A...
July 19, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28714487/screening-mentally-retarded-children-for-inborn-errors-of-metabolism
#3
N K Shreevastava, A S Pandey
BACKGROUND: Most inborn errors of metabolism result in mental retardation and death due to accumulation of abnormal metabolites in the tissues. The presence of abnormal metabolites in the urine of mentally retarded individuals has been used worldwide for detection of inborn errors of metabolism. The purpose of the study is to determine the prevalence of inborn error of metabolism in mentally retarded children. METHODS: Random urine samples were collected from mentally retarded children at two institutes in Kathmandu, and also from 60 normal children from Duwakot, Nepal after obtaining consent from their parents...
January 2017: Journal of Nepal Health Research Council
https://www.readbyqxmd.com/read/28709938/a-cross-sectional-comparison-of-brain-glucose-and-ketone-metabolism-in-cognitively-healthy-older-adults-mild-cognitive-impairment-and-early-alzheimer-s-disease
#4
E Croteau, C A Castellano, M Fortier, C Bocti, T Fulop, N Paquet, S C Cunnane
INTRODUCTION: Deteriorating brain glucose metabolism precedes the clinical onset of Alzheimer's disease (AD) and appears to contribute to its etiology. Ketone bodies, mainly β-hydroxybutyrate and acetoacetate, are the primary alternative brain fuel to glucose. Some reports suggest that brain ketone metabolism is unchanged in AD but, to our knowledge, no such data are available for MCI. OBJECTIVE: To compare brain energy metabolism (glucose and acetoacetate) and some brain morphological characteristics in cognitively healthy older adult controls (CTL), mild cognitive impairment (MCI) and early AD...
July 11, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28701250/the-ketogenic-diet-making-a-comeback
#5
Thomas Walczyk, Jeannette Y Wick
Americans have embraced a large number of diets in an attempt to manage obesity, improve quality of life, and address specific health problems. Among diets developed to address health problems, the ketogenic diet has had a long and variable history. Developed in the 1920s by a faith healer to help children with epilepsy, this diet induces a state that mimics carbohydrate starvation. As medications became available and effectively addressed seizures, the diet fell out of favor. During the last few decades, researchers and clinicians have learned that it can be useful in children and adults with refractory epilepsy and a variety of other conditions...
July 1, 2017: Consultant Pharmacist: the Journal of the American Society of Consultant Pharmacists
https://www.readbyqxmd.com/read/28696163/evaluation-of-dietary-and-lifestyle-changes-as-modifiers-of-s100%C3%AE-levels-in-alzheimer-s-disease
#6
Nathan M D'Cunha, Andrew J McKune, Demosthenes B Panagiotakos, Ekavi N Georgousopoulou, Jackson Thomas, Duane D Mellor, Nenad Naumovski
There is a significant body of research undertaken in order to elucidate the mechanisms underlying the pathology of Alzheimer's disease (AD), as well as to discover early detection biomarkers and potential therapeutic strategies. One such proposed biomarker is the calcium binding protein S100β, which, depending on its local concentration, is known to exhibit both neurotrophic and neuroinflammatory properties in the central nervous system. At present, relatively little is known regarding the effect of chronic S100β disruption in AD...
July 11, 2017: Nutritional Neuroscience
https://www.readbyqxmd.com/read/28695376/heterozygous-carriers-of-succinyl-coa-3-oxoacid-coa-transferase-deficiency-can-develop-severe-ketoacidosis
#7
Hideo Sasai, Yuka Aoyama, Hiroki Otsuka, Elsayed Abdelkreem, Yasuhiro Naiki, Mitsuru Kubota, Yuji Sekine, Masatsune Itoh, Mina Nakama, Hidenori Ohnishi, Ryoji Fujiki, Osamu Ohara, Toshiyuki Fukao
Succinyl-CoA:3-oxoacid CoA transferase (SCOT, gene symbol OXCT1) deficiency is an autosomal recessive disorder in ketone body utilization that results in severe recurrent ketoacidotic episodes in infancy, including neonatal periods. More than 30 patients with this disorder have been reported and to our knowledge, their heterozygous parents and siblings have had no apparent ketoacidotic episodes. Over 5 years (2008-2012), we investigated several patients that presented with severe ketoacidosis and identified a heterozygous OXCT1 mutation in four of these cases (Case1 p...
July 10, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28693220/remodeling-of-energy-metabolism-by-a-ketone-body-and-medium-chain-fatty-acid-suppressed-the-proliferation-of-ct26-mouse-colon-cancer-cells
#8
Yui Kadochi, Shiori Mori, Rina Fujiwara-Tani, Yi Luo, Yukiko Nishiguchi, Shingo Kishi, Kiyomu Fujii, Hitoshi Ohmori, Hiroki Kuniyasu
Normal and cancerous cells are suggested to have differential utilization of fatty acids and ketone bodies, which could be exploited in cancer therapy. The present study examined the effect of 3-hydroxybutyric acid (3-HBA), which is a ketone body generating acetyl-CoA, and lauric acid (LAA, C12:0), which is a medium-chain saturated fatty acid translocated to mitochondria in a carnitine-independent manner to produce acetyl-CoA, on the energy metabolism of mouse CT26 colon cancer cells. In CT26 cells expressing 3-HBA and LAA transporters, 3-HBA and LAA reduced cell proliferation, mitochondrial volume and lactate production, and increased oxidative stress, particularly in low-glucose conditions...
July 2017: Oncology Letters
https://www.readbyqxmd.com/read/28689740/clinical-presentation-and-outcome-in-a-series-of-32-patients-with-2-methylacetoacetyl-coenzyme-a-thiolase-mat-deficiency
#9
Sarah Catharina Grünert, Robert Niklas Schmitt, Sonja Marina Schlatter, Corinne Gemperle-Britschgi, Mehmet Cihan Balcı, Volker Berg, Mahmut Çoker, Anibh M Das, Mübeccel Demirkol, Terry G J Derks, Gülden Gökçay, Sema Kalkan Uçar, Vassiliki Konstantopoulou, G Christoph Korenke, Amelie Sophia Lotz-Havla, Andrea Schlune, Christian Staufner, Christel Tran, Gepke Visser, Karl Otfried Schwab, Toshiyuki Fukao, Jörn Oliver Sass
2-methylacetoacetyl-coenzyme A thiolase (MAT) deficiency, also known as beta-ketothiolase deficiency, is an inborn error of ketone body utilization and isoleucine catabolism. It is caused by mutations in the ACAT1 gene and may present with metabolic ketoacidosis. In order to obtain a more comprehensive view on this disease, we have collected clinical and biochemical data as well as information on ACAT1 mutations of 32 patients from 12 metabolic centers in five countries. Patients were between 23months and 27years old, more than half of them were offspring of a consanguineous union...
June 27, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28687765/reduction-of-epileptiform-activity-in-ketogenic-mice-the-role-of-monocarboxylate-transporters
#10
Linda S Forero-Quintero, Joachim W Deitmer, Holger M Becker
Epilepsy is a chronic neurological disorder that affects approximately 50 million people worldwide. Ketogenic diet (KD) can be a very effective treatment for intractable epilepsy. Potential mechanisms of action for KD have been proposed, including the re-balance among excitatory and inhibitory neurotransmission and decrease in the glycolytic rate in brain cells. KD has been shown to have an effect on the expression pattern of monocarboxylate transporters (MCT), however, it is unknown whether MCT transport activity is affected by KD and linked to the reduction of seizures during KD...
July 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28685447/diet-induced-ketosis-protects-against-focal-cerebral-ischemia-in-mouse
#11
Kui Xu, Lena Ye, Katyayini Sharma, Yongming Jin, Matthew M Harrison, Tylor Caldwell, Jessica M Berthiaume, Yu Luo, Joseph C LaManna, Michelle A Puchowicz
Over the past decade we have consistently shown that ketosis is neuroprotective against ischemic insults in rats. We reported that diet-induced ketotic rats had a significant reduction in infarct volume when subjected to middle cerebral artery occlusion (MCAO), and improved survival and recovery after cardiac arrest and resuscitation. The neuroprotective mechanisms of ketosis (via ketogenic diet; KG) include (i) ketones are alternate energy substrates that can restore energy balance when glucose metabolism is deficient and (ii) ketones modulate cell-signalling pathways that are cytoprotective...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28684065/the-role-of-oxct1-in-the-pathogenesis-of-cancer-as-a-rate-limiting-enzyme-of-ketone-body-metabolism
#12
REVIEW
Song Zhang, Caifeng Xie
Cancer cells are well documented to reprogram their metabolism in order to support the maintenance and reproduction. 3-oxoacid CoA-transferase 1 (OXCT1) is a key enzyme in ketone body metabolism that catalyzes the first and rate-determining step of ketolysis. The product of OXCT1 converts to acetyl-CoA and finally fed into the tricarboxylic acid cycle for oxidation and ATP production. However, little is known of its regulation right now. Recently, some studies suggested that OXCT1 participates in tumorigenesis and signaling in cancer cells...
July 3, 2017: Life Sciences
https://www.readbyqxmd.com/read/28667864/an-acidosis-sparing-ketogenic-ask-diet-to-improve-efficacy-and-reduce-adverse-effects-in-the-treatment-of-refractory-epilepsy
#13
REVIEW
Alan W C Yuen, Isabel A Walcutt, Josemir W Sander
Diets that increase production of ketone bodies to provide alternative fuel for the brain are evolving from the classic ketogenic diet for epilepsy devised nearly a century ago. The classic ketogenic diet and its more recent variants all appear to have similar efficacy with approximately 50% of users showing a greater than 50% seizure reduction. They all require significant medical and dietetic support, and there are tolerability issues. A review suggests that low-grade chronic metabolic acidosis associated with ketosis is likely to be an important contributor to the short term and long term adverse effects of ketogenic diets...
June 28, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28658838/hemi-chorea-in-a-patient-with-ketotic-hyperglycemia-an-unusual-presentation
#14
Pendela Venkata Satish, Kudaravalli Pujitha, Neha Agrawal, Thomas Mathew, Sudha Vidyasagar
Chorea has often been associated with lesions in the basal ganglia and in the sub thalamic nucleus. It is possible for a patient with chorea-ballismus to have hyperglycemia at the initial presentation. We hereby present a case of an 81-year-old female, who was on treatment for type 2 diabetes mellitus and presented to us with sub acute onset of abnormal movements of right side of the body. She had semi purposeful, rapid and jerky movements of right upper limb and lower limb along with abnormal tongue movements...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28652565/ketoacidosis-in-neonatal-diabetes-mellitus-part-of-wolcott-rallison-syndrome
#15
Poorna Gopal Azad Sreeramaneni, Subba Rao V Ambula
BACKGROUND Neonatal diabetes mellitus is a rare condition and it is important to differentiate it from other causes, such as hyperglycemia in infancy, for better outcomes. We report a case of an infant who presented to our neonatal intensive care unit in ketoacidosis and a comatose state. CASE REPORT Our case was an infant who presented to the neonatal intensive care unit at 38 days of age in ketoacidosis. The female infant, born to consanguineous parents (first cousins), weighing 2,300 grams at birth, presented with extreme dehydration and pale skin...
June 27, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28647176/mitochondrial-oxidative-phosphorylation-efficiency-is-upregulated-during-fasting-in-two-major-oxidative-tissues-of-ducklings
#16
Pierre-Axel Monternier, Loïc Teulier, Jocelyne Drai, Aurore Bourguignon, Delphine Collin-Chavagnac, Frédéric Hervant, Jean-Louis Rouanet, Damien Roussel
Fasted endothermic vertebrates must develop physiological responses to maximize energy conservation and survival. The aim of this study was to determine the effect of 1-wk. fasting in 5-wk. old ducklings (Cairina moschata) from whole-body resting metabolic rate and body temperature to metabolic phenotype of tissues and mitochondrial coupling efficiency. At the level of whole organism, the mass-specific metabolic rate of ducklings was decreased by 40% after 1-wk. of fasting, which was associated with nocturnal Tb declines and shallow diurnal hypothermia during fasting...
June 21, 2017: Comparative Biochemistry and Physiology. Part A, Molecular & Integrative Physiology
https://www.readbyqxmd.com/read/28646031/metabolism-and-acetylation-contribute-to-leucine-mediated-inhibition-of-cardiac-glucose-uptake
#17
Edith Renguet, Audrey Ginion, Roselle Gélinas, Laurent Bultot, Julien Auquier, Isabelle Robillard Frayne, Caroline Daneault, Jean-Louis Vanoverschelde, Christine Des Rosiers, Louis Hue, Sandrine Horman, Christophe Beauloye, Luc Bertrand
High plasma leucine levels strongly correlate with type 2 diabetes. Studies of muscle cells suggest that leucine alters the insulin response for glucose transport by activating an insulin-negative feedback loop driven by the mammalian target of rapamycin/p70 ribosomal S6 kinase (mTOR/p70S6K) pathway. Here, we examined the molecular mechanism involved in leucine's action on cardiac glucose uptake. Leucine was indeed able to curb glucose uptake after insulin stimulation in both cultured cardiomyocytes and perfused hearts...
June 23, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28638409/differential-fasting-plasma-glucose-and-ketone-body-levels-in-ghrko-versus-3xtg-ad-mice-a-potential-contributor-to-aging-related-cognitive-status
#18
Chelsea M Griffith, Lauren N Macklin, Andrzej Bartke, Peter R Patrylo
Cognitive function declines with age and appears to correlate with decreased cerebral metabolic rate (CMR). Caloric restriction, an antiaging manipulation that extends life-span and can preserve cognitive function, is associated with decreased glucose uptake, decreased lactate levels, and increased ketone body (KB) levels in the brain. Since the majority of brain nutrients come from the periphery, this study examined whether the capacity to regulate peripheral glucose levels and KB production differs in animals with successful cognitive aging (growth hormone receptor knockouts, GHRKOs) versus unsuccessful cognitive aging (the 3xTg-AD mouse model of Alzheimer's disease)...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/28637015/differential-metabonomic-profiles-of-primary-hepatocellular-carcinoma-tumors-from-alcoholic-liver-disease-hbv-infected-and-hcv-infected-cirrhotic-patients
#19
Ding Cao, Can Cai, Mingxin Ye, Junhua Gong, Menghao Wang, Jinzheng Li, Jianping Gong
Our objective was to comparatively profile the metabolite composition of primary hepatocellular carcinoma (HCC) tumors from alcoholic liver disease (ALD), hepatitis B virus (HBV)-infected, and hepatitis C virus (HCV)-infected cirrhotic patients. Primary HCC tumors were collected from ALD, HBV-infected, and HCV-infected cirrhotic patients (n=20 each). High-resolution magic-angle spinning proton nuclear magnetic resonance spectroscopy and metabonomic data analysis were performed to compare HCC tumors from the three groups...
June 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28627374/characterization-of-the-concurrent-metabolic-changes-in-brain-and-plasma-during-insulin-induced-moderate-hypoglycemia-using-1-h-nmr-spectroscopy-in-juvenile-rats
#20
Kathleen Ennis, Elizabeth Lusczek, Raghavendra Rao
Treatment of hypoglycemia in children is currently based on plasma glucose measurements. This approach may not ensure neuroprotection since plasma glucose does not reflect the dynamic state of cerebral energy metabolism. To determine whether cerebral metabolic changes during hypoglycemia could be better characterized using plasma metabolomic analysis, insulin-induced acute hypoglycemia was induced in 4-week-old rats. Brain tissue and concurrent plasma samples were collected from hypoglycemic (N=7) and control (N=7) rats after focused microwave fixation to prevent post-mortem metabolic changes...
July 13, 2017: Neuroscience Letters
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