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Mitochondrial mass mitochondrial metabolism

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https://www.readbyqxmd.com/read/29454001/brain-bioenergetics-in-rats-with-acute-hyperphenylalaninemia
#1
Nádia Weber Dimer, Bruna Klippel Ferreira, Jotele Fontana Agostini, Maria Luiza Gomes, Luiza Wilges Kist, Fernanda Malgarin, Milena Carvalho-Silva, Lara Mezari Gomes, Joyce Rebelo, Marisa Jádna Silva Frederico, Fátima Regina Mena Barreto Silva, Eduardo Pacheco Rico, Mauricio Reis Bogo, Emilio Luiz Streck, Gustavo Costa Ferreira, Patrícia Fernanda Schuck
Phenylketonuria (PKU) is a disorder of phenylalanine (Phe) metabolism caused by deficient phenylalanine hydroxylase activity. The deficiency results in increased levels of Phe and its metabolites in fluids and tissues of patients. PKU patients present neurological signals and symptoms including hypomyelination and intellectual deficit. This study assessed brain bioenergetics at 1 h after acute Phe administration to induce hyperphenylalaninemia (HPA) in rats. Wistar rats were randomized in two groups: HPA animals received a single subcutaneous administration of Phe (5...
February 14, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29447265/dynamic-remodeling-of-lipids-coincides-with-dengue-virus-replication-in-the-midgut-of-aedes-aegypti-mosquitoes
#2
Nunya Chotiwan, Barbara G Andre, Irma Sanchez-Vargas, M Nurul Islam, Jeffrey M Grabowski, Amber Hopf-Jannasch, Erik Gough, Ernesto Nakayasu, Carol D Blair, John T Belisle, Catherine A Hill, Richard J Kuhn, Rushika Perera
We describe the first comprehensive analysis of the midgut metabolome of Aedes aegypti, the primary mosquito vector for arboviruses such as dengue, Zika, chikungunya and yellow fever viruses. Transmission of these viruses depends on their ability to infect, replicate and disseminate from several tissues in the mosquito vector. The metabolic environments within these tissues play crucial roles in these processes. Since these viruses are enveloped, viral replication, assembly and release occur on cellular membranes primed through the manipulation of host metabolism...
February 2018: PLoS Pathogens
https://www.readbyqxmd.com/read/29429536/are-patients-affected-by-mitochondrial-disorders-at-nutritional-risk
#3
Emilie Aubry, Carla Aeberhard, Lia Bally, Jean-Marc Nuoffer, Lorenz Risch, Stefan Mühlebach, Jean-Marc Burgunder, Zeno Stanga
OBJECTIVES: Patients with mitochondrial disorders (MD) frequently present with gastrointestinal complaints, mainly gastrointestinal dysmotility, that interfere with their food intake. A deterioration of their nutritional state may worsen the course of the disease. Our study aimed to evaluate a simple screening tool to identify nutritional risk and perform an extended nutritional assessment to explore the potential presence of deficiencies in this population compared with controls. METHODS: A prospective cohort study was conducted to compare outpatients with MD to matched healthy controls...
March 2018: Nutrition
https://www.readbyqxmd.com/read/29424033/mitochondrial-dysfunction-in-human-skeletal-muscle-biopsies-of-lipid-storage-disorder
#4
B Debashree, Manish Kumar, T S Keshava Prasad, Archana Natarajan, Rita Christopher, A Nalini, P S Bindu, N Gayathri, M M Srinivas Bharath
Mitochondria regulate the balance between lipid metabolism and storage in the skeletal muscle. Altered lipid transport, metabolism and storage influence the bioenergetics, redox status and insulin signalling contributing to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neurological disorders which entail intramuscular lipid accumulation and impaired mitochondrial bioenergetics in the skeletal muscle causing progressive myopathy with muscle weakness. However, the mitochondrial changes including molecular events associated with impaired lipid storage have not been completely understood in the human skeletal muscle...
February 9, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29423831/targeted-versus-untargeted-omics-the-cafsa-story
#5
Maria Del Mar Amador, Benoit Colsch, Foudil Lamari, Claude Jardel, Farid Ichou, Agnès Rastetter, Frédéric Sedel, Fabien Jourdan, Clément Frainay, Ronald A Wevers, Emmanuel Roze, Christel Depienne, Christophe Junot, Fanny Mochel
BACKGROUND: In 2009, untargeted metabolomics led to the delineation of a new clinico-biological entity called cerebellar ataxia with elevated cerebrospinal free sialic acid, or CAFSA. In order to elucidate CAFSA, we applied sequentially targeted and untargeted omic approaches. METHODS AND RESULTS: First, we studied five of the six CAFSA patients initially described. Besides increased CSF free sialic acid concentrations, three patients presented with markedly decreased 5-methyltetrahydrofolate (5-MTHF) CSF concentrations...
February 8, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29420990/potential-roles-of-vitamin-e-in-age-related-changes-in-skeletal-muscle-health
#6
REVIEW
Eunhee Chung, Huanbiao Mo, Shu Wang, Yujiao Zu, Manal Elfakhani, Steven R Rios, Ming-Chien Chyu, Rong-Sen Yang, Chwan-Li Shen
Skeletal muscle disorders including sarcopenia are prevalent during the complex biological process of aging. Loss of muscle mass and strength commonly seen in sarcopenia is induced by impaired neuromuscular innervation, transition of skeletal muscle fiber type, and reduced muscle regenerative capacity, all attributable to chronic inflammation, oxidative stress, and mitochondrial dysfunction. Current literature suggests that vitamin E molecules (α-, β-, γ-, δ-tocopherols and the corresponding tocotrienols) with their antioxidant and anti-inflammatory capabilities may mitigate age-associated skeletal dysfunction and enhance muscle regeneration, thus attenuating sarcopenia...
January 2018: Nutrition Research
https://www.readbyqxmd.com/read/29415881/tak1-regulates-skeletal-muscle-mass-and-mitochondrial-function
#7
Sajedah M Hindi, Shuichi Sato, Guangyan Xiong, Kyle R Bohnert, Andrew A Gibb, Yann S Gallot, Joseph D McMillan, Bradford G Hill, Shizuka Uchida, Ashok Kumar
Skeletal muscle mass is regulated by a complex array of signaling pathways. TGF-β-activated kinase 1 (TAK1) is an important signaling protein, which regulates context-dependent activation of multiple intracellular pathways. However, the role of TAK1 in the regulation of skeletal muscle mass remains unknown. Here, we report that inducible inactivation of TAK1 causes severe muscle wasting, leading to kyphosis, in both young and adult mice.. Inactivation of TAK1 inhibits protein synthesis and induces proteolysis, potentially through upregulating the activity of the ubiquitin-proteasome system and autophagy...
February 8, 2018: JCI Insight
https://www.readbyqxmd.com/read/29405372/melatonin-increases-brown-adipose-tissue-mass-and-function-in-z%C3%A3-cker-diabetic-fatty-rats-implications-for-obesity-control
#8
Gumersindo Fernández Vázquez, Russel J Reiter, Ahmad Agil
Melatonin limits obesity in rodents without affecting food intake and activity, suggesting a thermogenic effect. Previously we demonstrated that melatonin browns subcutaneous fat in Zücker diabetic fatty (ZDF) rats. Other works pointed to melatonin as a signal that increases brown adipose tissue (BAT) mass and function in rodents. However, direct proof of thermogenic properties (uncoupled mitochondria) of the newly recruited BAT in response to melatonin is still lacking. Therefore, in the present work we investigated if melatonin recruits thermogenic BAT in ZDF rats...
February 6, 2018: Journal of Pineal Research
https://www.readbyqxmd.com/read/29404440/engineered-fgf19-eliminates-bile-acid-toxicity-and-lipotoxicity-leading-to-resolution-of-steatohepatitis-and-fibrosis-in-mice
#9
Mei Zhou, R Marc Learned, Stephen J Rossi, Alex M DePaoli, Hui Tian, Lei Ling
Nonalcoholic fatty liver disease (NAFLD) is an increasingly prevalent chronic liver disease for which no approved therapies are available. Despite intensive research, the cellular mechanisms that mediate NAFLD pathogenesis and progression are poorly understood. Although obesity, diabetes, insulin resistance, and related metabolic syndrome, all consequences of a Western diet lifestyle, are well-recognized risk factors for NAFLD development, dysregulated bile acid metabolism is emerging as a novel mechanism contributing to NAFLD pathogenesis...
December 2017: Hepatology Communications
https://www.readbyqxmd.com/read/29402948/metabolomics-profiling-reveals-differential-adaptation-of-major-energy-metabolism-pathways-associated-with-autophagy-upon-oxygen-and-glucose-reduction
#10
Katja Weckmann, Philip Diefenthäler, Marius W Baeken, Kamran Yusifli, Christoph W Turck, John M Asara, Christian Behl, Parvana Hajieva
The ability of cells to rearrange their metabolism plays an important role in compensating the energy shortage and may provide cell survival. Our study focuses on identifing the important adaptational changes under the conditions of oxygen and glucose reduction. Employing mass spectrometry-based metabolomics in combination with biochemistry and microscopy techniques we identified metabolites, proteins and biomolecular pathways alterations in primary human IMR90 fibroblasts upon energy deficits. Multivariate statistical analyses revealed significant treatment-specific metabolite level and ratio alterations as well as major energy metabolism pathways like 'glycolysis', 'pentose phosphate pathway', 'mitochondrial electron transport chain' and 'protein biosynthesis (amino acids)' indicating an activation of catabolism and reduction of anabolism as important mechanisms of adaptation towards a bioenergetic demand...
February 5, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29399895/leucine-supplementation-attenuates-macrophage-foam-cell-formation-studies-in-humans-mice-and-cultured-macrophages
#11
Claudia Grajeda-Iglesias, Oren Rom, Shadi Hamoud, Nina Volkova, Tony Hayek, Niroz Abu-Saleh, Michael Aviram
Whereas atherogenicity of dietary lipids has been largely studied, relatively little is known about the possible contribution of dietary amino acids to macrophage foam-cell formation, a hallmark of early atherogenesis. Recently, we showed that leucine has antiatherogenic properties in the macrophage model system. In this study, an in-depth investigation of the role of leucine in macrophage lipid metabolism was conducted by supplementing humans, mice, or cultured macrophages with leucine. Macrophage incubation with serum obtained from healthy adults supplemented with leucine (5 g/d, 3 weeks) significantly decreased cellular cholesterol mass by inhibiting the rate of cholesterol biosynthesis and increasing cholesterol efflux from macrophages...
February 5, 2018: BioFactors
https://www.readbyqxmd.com/read/29388700/neonatal-treatment-with-fluoxetine-improves-mitochondrial-respiration-and-reduces-oxidative-stress-in-liver-of-adult-rats
#12
Aiany C Simões-Alves, Reginaldo C Silva-Filho, Glauber Ruda F Braz, Severina Cassia A Silva, Aline Isabel da Silva, Claudia J Lagranha, Mariana P Fernandes
Recent studies have shown that exposure to fluoxetine treatment induces excessive production of ROS, and alters the antioxidant defense system in various tissues and cell types, mainly the liver. When fluoxetine is administered intraperitoneally, the drug rapidly reaches high concentrations in the liver, has potentially multiple toxic effects on energy metabolism in rat liver mitochondria. The aim of this study was to evaluate the effect of pharmacological treatment with fluoxetine during critical period for development on the mitochondrial bioenergetics and oxidative stress in liver of rat adult...
February 1, 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29384724/update-on-mitochondria-and-muscle-aging-all-wrong-roads-lead-to-sarcopenia
#13
Anna Picca, Riccardo Calvani, Maurizio Bossola, Elena Allocca, Amerigo Menghi, Vito Pesce, Angela Maria Serena Lezza, Roberto Bernabei, Francesco Landi, Emanuele Marzetti
Sarcopenia is a well-known geriatric syndrome that has been endorsed over the years as a biomarker allowing for the discrimination, at a clinical level, of biological from chronological age. Multiple candidate mechanisms have been linked to muscle degeneration during sarcopenia. Among them, there is wide consensus on the central role played by the loss of mitochondrial integrity in myocytes, secondary to dysfunctional quality control mechanisms. Indeed, mitochondria establish direct or indirect contacts with other cellular components (e...
November 27, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/29384213/fto-reduces-mitochondria-and-promotes-hepatic-fat-accumulation-through-rna-demethylation
#14
Huifang Kang, Zhiwang Zhang, Lin Yu, Yixing Li, Mingzhen Liang, Lei Zhou
Fat mass and obesity-associated protein (FTO) is a RNA demethylase, whether FTO regulates fat metabolism through its demethylation is unclear. The results of this study confirmed that N6-methyladenosine (m6 A) is associated with fat accumulation both in vivo and in vitro. The data showed that FTO down-regulated m6 A levels, decreased mitochondrial content, and increased triglyceride (TG) deposition. However, an FTO (R316A) mutant lacking demethylation activity could not regulate mitochondria and TG content, indicating that FTO affects mitochondrial content and fat metabolism by modulating m6 A levels in hepatocytes...
January 31, 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29383631/wheat-plant-selection-for-high-yields-entailed-improvement-of-leaf-anatomical-and-biochemical-traits-including-tolerance-to-non-optimal-temperature-conditions
#15
Marian Brestic, Marek Zivcak, Pavol Hauptvogel, Svetlana Misheva, Konstantina Kocheva, Xinghong Yang, Xiangnan Li, Suleyman I Allakhverdiev
Assessment of photosynthetic traits and temperature tolerance was performed on field-grown modern genotype (MG), and the local landrace (LR) of wheat (Triticum aestivum L.) as well as the wild relative species (Aegilops cylindrica Host.). The comparison was based on measurements of the gas exchange (A/ci, light and temperature response curves), slow and fast chlorophyll fluorescence kinetics, and some growth and leaf parameters. In MG, we observed the highest CO2 assimilation rate [Formula: see text] electron transport rate (Jmax) and maximum carboxylation rate [Formula: see text]...
January 30, 2018: Photosynthesis Research
https://www.readbyqxmd.com/read/29377983/titin-cardiomyopathy-leads-to-altered-mitochondrial-energetics-increased-fibrosis-and-long-term-life-threatening-arrhythmias
#16
Job A J Verdonschot, Mark R Hazebroek, Kasper W J Derks, Arantxa Barandiarán Aizpurua, Jort J Merken, Ping Wang, Jörgen Bierau, Arthur van den Wijngaard, Simon M Schalla, Myrurgia A Abdul Hamid, Marc van Bilsen, Vanessa P M van Empel, Christian Knackstedt, Hans-Peter Brunner-La Rocca, Han G Brunner, Ingrid P C Krapels, Stephane R B Heymans
Aims: Truncating titin variants (TTNtv) are the most prevalent genetic cause of dilated cardiomyopathy (DCM). We aim to study clinical parameters and long-term outcomes related to the TTNtv genotype and determine the related molecular changes at tissue level in TTNtv DCM patients. Methods and results: A total of 303 consecutive and extensively phenotyped DCM patients (including cardiac imaging, Holter monitoring, and endomyocardial biopsy) underwent DNA sequencing of 47 cardiomyopathy-associated genes including TTN, yielding 38 TTNtv positive (13%) patients...
January 25, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29377510/bcaa-metabolism-and-insulin-sensitivity-dysregulated-by-metabolic-status
#17
REVIEW
Nicholas P Gannon, Jamie K Schnuck, Roger A Vaughan
Branched-chain amino acids (BCAAs) appear to influence several synthetic and catabolic cellular signaling cascades leading to altered phenotypes in mammals. BCAAs are most notably known to increase protein synthesis through modulating protein translation, explaining their appeal to resistance and endurance athletes for muscle hypertrophy, expedited recovery, and preservation of lean body mass. In addition to anabolic effects, BCAAs may increase mitochondrial content in skeletal muscle and adipocytes, possibly enhancing oxidative capacity...
January 29, 2018: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/29369849/sirtuin-1-regulates-pulmonary-artery-smooth-muscle-cell-proliferation-role-in-pulmonary-arterial-hypertension
#18
Giada Zurlo, Jérôme Piquereau, Maryline Moulin, Julie Pires Da Silva, Mélanie Gressette, Benoît Ranchoux, Anne Garnier, Renée Ventura-Clapier, Elie Fadel, Marc Humbert, Christophe Lemaire, Frédéric Perros, Vladimir Veksler
OBJECTIVE: Energy metabolism shift from oxidative phosphorylation toward glycolysis in pulmonary artery smooth muscle cells (PASMCs) is suggested to be involved in their hyperproliferation in pulmonary arterial hypertension (PAH). Here, we studied the role of the deacetylase sirtuin1 (SIRT1) in energy metabolism regulation in PASMCs via various pathways including activation of peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α), master regulator of mitochondrial biogenesis...
January 24, 2018: Journal of Hypertension
https://www.readbyqxmd.com/read/29358174/low-density-lipoprotein-uptake-inhibits-the-activation-and-antitumour-functions-of-human-vgamma9vdelta2-t-cells
#19
Neidy Varela Rodrigues, Daniel V Correia, Sofia Mensurado, Sandrina Nobrega-Pereira, Ana deBarros, Fernanda Kyle-Cezar, Andrew Tutt, Adrian C Hayday, Haakan Norell, Bruno Silva-Santos, Sergio Dias
Vgamma9Vdelta2 T cells, the main subset of gamma delta T lymphocytes in human peripheral blood, are endowed with antitumor functions such as cytotoxicity and IFNgamma production. These functions are triggered upon TCR-dependent activation by non-peptidic prenyl pyrophosphates ("phosphoantigens") that are selective agonists of Vgamma9Vdelta2 T cells, and which have been evaluated in clinical studies. Because phosphoantigens have shown inter-individual variation in Vgamma9Vdelta2 T-cell activities, we asked whether metabolic resources, namely lipids such as cholesterol, could affect phosphoantigen-mediated Vgamma9Vdelta2 T-cell activation and function...
January 22, 2018: Cancer Immunology Research
https://www.readbyqxmd.com/read/29356774/the-mechanisms-underlying-lipid-resuscitation-therapy
#20
Michael R Fettiplace, Guy Weinberg
The experimental use of lipid emulsion for local anesthetic toxicity was originally identified in 1998. It was then translated to clinical practice in 2006 and expanded to drugs other than local anesthetics in 2008. Our understanding of lipid resuscitation therapy has progressed considerably since the previous update from the American Society of Regional Anesthesia and Pain Medicine, and the scientific evidence has coalesced around specific discrete mechanisms. Intravenous lipid emulsion therapy provides a multimodal resuscitation benefit that includes both scavenging (eg, the lipid shuttle) and nonscavenging components...
February 2018: Regional Anesthesia and Pain Medicine
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