keyword
MENU ▼
Read by QxMD icon Read
search

OXPHOS disease

keyword
https://www.readbyqxmd.com/read/28623342/hif-1%C3%AE-knockdown-reduces-glycolytic-metabolism-and-induces-cell-death-of-human-synovial-fibroblasts-under-normoxic-conditions
#1
Manuel J Del Rey, Álvaro Valín, Alicia Usategui, Carmen M García-Herrero, María Sánchez-Aragó, José M Cuezva, María Galindo, Beatriz Bravo, Juan D Cañete, Francisco J Blanco, Gabriel Criado, José L Pablos
Increased glycolysis and HIF-1α activity are characteristics of cells under hypoxic or inflammatory conditions. Besides, in normal O2 environments, elevated rates of glycolysis support critical cellular mechanisms such as cell survival. The purpose of this study was to analyze the contribution of HIF-1α to the energy metabolism and survival of human synovial fibroblasts (SF) under normoxic conditions. HIF-1α was silenced using lentiviral vectors or small-interfering RNA (siRNA) duplexes. Expression analysis by qRT-PCR and western blot of known HIF-1α target genes in hypoxia demonstrated the presence of functional HIF-1α in normoxic SF and confirmed the glycolytic enzyme glyceraldehyde-3-phosphate dehydrogenase (GAPDH) as a HIF-1α target even in normoxia...
June 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28618997/vdac1-as-a-player-in-mitochondria-mediated-apoptosis-and-target-for-modulating-apoptosis
#2
Varda Shoshan-Barmatz, Yakov Krelin, Quan Chen
The voltage-dependent anion channel 1 (VDAC1), an outer mitochondria membrane protein, functions as a mitochondrial governor, controlling transport of metabolites in and out of the mitochondria and energy production, while also coordinating glycolysis and oxidative phosphorylation (OXPHOS). . VDAC1 plays a key role in mitochondria-mediated apoptosis by functioning in the release of apoptotic proteins located in the inter-membranal space (IMS) and due to its association with pro- and anti-apoptotic proteins...
June 16, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28592294/exercise-in-claudicants-increase-or-decrease-walking-ability-and-the-response-relates-to-mitochondrial-function
#3
Michel van Schaardenburgh, Martin Wohlwend, Øivind Rognmo, Erney J R Mattsson
BACKGROUND: Exercise of patients with intermittent claudication improves walking performance. Exercise does not usually increase blood flow, but seems to increase muscle mitochondrial enzyme activities. Although exercise is beneficial in most patients, it might be harmful in some. The mitochondrial response to exercise might therefore differ between patients. Our hypothesis was that changes in walking performance relate to changes in mitochondrial function after 8 weeks of exercise. At a subgroup level, negative responders decrease and positive responders increase mitochondrial capacity...
June 7, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28591633/cytochrome-c-oxidase-activity-is-a-metabolic-checkpoint-that-regulates-cell-fate-decisions-during-t-cell-activation-and-differentiation
#4
Tatyana N Tarasenko, Susan E Pacheco, Mary Kay Koenig, Julio Gomez-Rodriguez, Senta M Kapnick, Francisca Diaz, Patricia M Zerfas, Emanuele Barca, Jessica Sudderth, Ralph J DeBerardinis, Raul Covian, Robert S Balaban, Salvatore DiMauro, Peter J McGuire
T cells undergo metabolic reprogramming with major changes in cellular energy metabolism during activation. In patients with mitochondrial disease, clinical data were marked by frequent infections and immunodeficiency, prompting us to explore the consequences of oxidative phosphorylation dysfunction in T cells. Since cytochrome c oxidase (COX) is a critical regulator of OXPHOS, we created a mouse model with isolated dysfunction in T cells by targeting a gene, COX10, that produces mitochondrial disease in humans...
June 6, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28585712/a-novel-non-apoptotic-role-of-procaspase-3-in-the-regulation-of-mitochondrial-biogenesis-activators
#5
Ji-Soo Kim, Ji-Young Ha, Sol-Ji Yang, Jin H Son
The executioner caspase-3 has been proposed as a pharmacological intervention target to preserve degenerating dopaminergic (DA) neurons because apoptotic mechanisms involving caspase-3 contribute, at least in part, to the loss of DA neurons in patients and experimental models of Parkinson's disease (PD). Here, we determined that genetic intervention of caspase-3 was sufficient to prevent cell death against oxidative stress (OS), accompanied by unexpected severe mitochondrial dysfunction. Specifically, as we expected, caspase-3-deficient DA neuronal cells were very significantly resistant to OS-induced cell death, while the activation of the initiator caspase-9 by OS was preserved...
June 6, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28499833/mitochondrial-dysfunction-in-cancer-potential-roles-of-atf5-and-the-mitochondrial-upr
#6
REVIEW
Pan Deng, Cole M Haynes
Mitochondria form a cellular network of organelles, or cellular compartments, that efficiently couple nutrients to energy production in the form of ATP. As cancer cells rely heavily on glycolysis, historically mitochondria and the cellular pathways in place to maintain mitochondrial activities were thought to be more relevant to diseases observed in non-dividing cells such as muscles and neurons. However, more recently it has become clear that cancers rely heavily on mitochondrial activities including lipid, nucleotide and amino acid synthesis, suppression of mitochondria-mediated apoptosis as well as oxidative phosphorylation (OXPHOS) for growth and survival...
May 10, 2017: Seminars in Cancer Biology
https://www.readbyqxmd.com/read/28499253/the-role-of-sodium-hydrosulfide-in-attenuating-the-aging-process-via-pi3k-akt-and-camkk%C3%AE-ampk-pathways
#7
Xubo Chen, Xueyan Zhao, Hua Cai, Haiying Sun, Yujuan Hu, Xiang Huang, Wen Kong, Weijia Kong
Age-related dysfunction of the central auditory system, known as central presbycusis, is characterized by defects in speech perception and sound localization. It is important to determine the pathogenesis of central presbycusis in order to explore a feasible and effective intervention method. Recent work has provided fascinating insight into the beneficial function of H2S on oxidative stress and stress-related disease. In this study, we investigated the pathogenesis of central presbycusis and tried to explore the mechanism of H2S action on different aspects of aging by utilizing a mimetic aging rat and senescent cellular model...
August 2017: Redox Biology
https://www.readbyqxmd.com/read/28416471/chemotherapy-resistant-human-acute-myeloid-leukemia-cells-are-not-enriched-for-leukemic-stem-cells-but-require-oxidative-metabolism
#8
Thomas Farge, Estelle Saland, Fabienne de Toni, Nesrine Aroua, Mohsen Hosseini, Robin Perry, Claudie Bosc, Mayumi Sugita, Lucille Stuani, Marine Fraisse, Sarah Scotland, Clément Larrue, Héléna Boutzen, Virginie Féliu, Marie-Laure Nicolau-Travers, Stéphanie Cassant-Sourdy, Nicolas Broin, Marion David, Nizar Serhan, Audrey Sarry, Suzanne Tavitian, Tony Kaoma, Laurent Vallar, Jason Iacovoni, Laetitia K Linares, Camille Montersino, Rémy Castellano, Emmanuel Griessinger, Yves Collette, Olivier Duchamp, Yara Barreira, Pierre Hirsch, Tony Palama, Lara Gales, François Delhommeau, Barbara H Garmy-Susini, Jean-Charles Portais, François Vergez, Mary Selak, Gwenn Danet-Desnoyers, Martin Carroll, Christian Récher, Jean-Emmanuel Sarry
Chemotherapy-resistant human acute myeloid leukemia (AML) cells are thought to be enriched in quiescent immature leukemic stem cells (LSC). To validate this hypothesis in vivo, we developed a clinically relevant chemotherapeutic approach treating patient-derived xenografts (PDX) with cytarabine (AraC). AraC residual AML cells are enriched in neither immature, quiescent cells nor LSCs. Strikingly, AraC-resistant preexisting and persisting cells displayed high levels of reactive oxygen species, showed increased mitochondrial mass, and retained active polarized mitochondria, consistent with a high oxidative phosphorylation (OXPHOS) status...
April 17, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28404750/human-adenine-nucleotide-translocases-physically-and-functionally-interact-with-respirasomes
#9
Ya-Wen Lu, Michelle Grace Acoba, Kandasamy Selvaraju, Tai-Chung Huang, Raja S Nirujogi, Gajanan Sathe, Akhilesh Pandey, Steven M Claypool
Members of the adenine nucleotide translocase (ANT) family exchange ADP for ATP across the mitochondrial inner membrane, an activity that is essential for oxidative phosphorylation (OXPHOS). Mutations in or dysregulation of ANTs is associated with progressive external ophthalmoplegia, cardiomyopathy, nonsyndromic intellectual disability, apoptosis, and the Warburg effect. Binding partners of human ANTs have not been systematically identified. The absence of such information has prevented a detailed molecular understanding of the assorted ANT-associated diseases, including insight into their disparate phenotypic manifestations...
June 1, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28392417/pharmacological-inhibition-of-carnitine-palmitoyltransferase-1-restores-mitochondrial-oxidative-phosphorylation-in-human-trifunctional-protein-deficient-fibroblasts
#10
Bruno Lefort, Elodie Gouache, Cécile Acquaviva, Marine Tardieu, Jean François Benoist, Jean-François Dumas, Stéphane Servais, Stéphan Chevalier, Christine Vianey-Saban, François Labarthe
BACKGROUND: Mitochondrial Trifunctional Protein deficiency (TFPD) is a severe genetic disease characterized by altered energy metabolism and accumulation of long-chain (LC) acylcarnitines in blood and tissues. This accumulation could impair the mitochondrial oxidative phosphorylation (OxPhos), contributing to the non-optimal outcome despite conventional diet therapy with medium-chain triglycerides (MCT). METHOD: Acylcarnitine and OxPhos parameters were measured in TFPD-fibroblasts obtained from 8 children and cultured in medium mimicking fasting (LCFA) or conventional treatment (MCT), with or without Etomoxir (ETX) an inhibitor of carnitine palmitoyltransferase 1 (CPT1) activity, and were compared to results obtained with fibroblasts from 5 healthy-control children...
April 6, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28362806/evolved-genetic-and-phenotypic-differences-due-to-mitochondrial-nuclear-interactions
#11
Tara Z Baris, Dominique N Wagner, David I Dayan, Xiao Du, Pierre U Blier, Nicolas Pichaud, Marjorie F Oleksiak, Douglas L Crawford
The oxidative phosphorylation (OxPhos) pathway is responsible for most aerobic ATP production and is the only pathway with both nuclear and mitochondrial encoded proteins. The importance of the interactions between these two genomes has recently received more attention because of their potential evolutionary effects and how they may affect human health and disease. In many different organisms, healthy nuclear and mitochondrial genome hybrids between species or among distant populations within a species affect fitness and OxPhos functions...
March 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28357370/attenuation-of-polyglutamine-induced-toxicity-by-enhancement-of-mitochondrial-oxphos-in-yeast-and-fly-models-of-aging
#12
Andrea L Ruetenik, Alejandro Ocampo, Kai Ruan, Yi Zhu, Chong Li, R Grace Zhai, Antoni Barrientos
Defects in mitochondrial biogenesis and function are common in many neurodegenerative disorders, including Huntington's disease (HD). We have previously shown that in yeast models of HD, enhancement of mitochondrial biogenesis through overexpression of Hap4, the catalytic subunit of the transcriptional complex that regulates mitochondrial gene expression, alleviates the growth arrest induced by expanded polyglutamine (polyQ) tract peptides in rapidly dividing cells. However, the mechanism through which HAP4 overexpression exerts this protection remains unclear...
July 26, 2016: Microbial Cell
https://www.readbyqxmd.com/read/28351484/hypocapnic-hypothesis-of-leigh-disease
#13
Ewa Pronicka
Leigh syndrome (LS) is a neurogenetic disorder of children caused by mutations in at least 75 genes which impair mitochondrial bioenergetics. The changes have typical localization in basal ganglia and brainstem, and typical histological picture of spongiform appearance, vascular proliferation and gliosis. ATP deprivation, free radicals and lactate accumulation are suspected to be the causes. Hypocapnic hypothesis proposed in the paper questions the energy deprivation as the mechanism of LS. We assume that the primary harmful factor is hypocapnia (decrease in pCO2) and respiratory alkalosis (increase in pH) due to hyperventilation, permanent or in response to stress...
April 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28345766/%C3%AE-linolenic-acid-and-exercise-training-independently-and-additively-decrease-blood-pressure-and-prevent-diastolic-dysfunction-in-obese-zucker-rats
#14
Pierre-Andre Barbeau, Tanya M Holloway, Jamie Whitfield, Brittany L Baechler, Joe Quadrilatero, Luc J C van Loon, Adrian Chabowski, Graham P Holloway
KEY POINTS: α-linolenic acid (ALA) and exercise training both attenuate hyperlipidaemia-related cardiovascular derangements, however, there is a paucity of information pertaining to their mechanisms of action when combined. We investigated both the independent and combined effects of exercise training and ALA consumption in obese Zucker rats, aiming to determine the potential for additive improvements in cardiovascular function. ALA and exercise training independently improved cardiac output, end-diastolic volume, left ventricular fibrosis and mean blood pressure following a 4 week intervention...
March 27, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28328323/an-adaptable-high-throughput-technology-enabling-the-identification-of-specific-transcription-modulators
#15
Tim Bergbrede, Emily Hoberg, Nils-Göran Larsson, Maria Falkenberg, Claes M Gustafsson
Mitochondria harbor the oxidative phosphorylation (OXPHOS) system, which under aerobic conditions produces the bulk of cellular adenosine triphosphate (ATP). The mitochondrial genome encodes key components of the OXPHOS system, and it is transcribed by the mitochondrial RNA polymerase, POLRMT. The levels of mitochondrial transcription correlate with the respiratory activity of the cell. Therefore, compounds that can increase or decrease mitochondrial gene transcription may be useful for fine-tuning metabolism and could be used to treat metabolic diseases or certain forms of cancer...
April 2017: SLAS Discovery
https://www.readbyqxmd.com/read/28276029/assessing-mitochondrial-selective-autophagy-in-the-nematode-caenorhabditis-elegans
#16
Konstantinos Palikaras, Nektarios Tavernarakis
Eukaryotic cells heavily depend on ATP generated by oxidative phosphorylation (OXPHOS) within mitochondria. Besides being the main suppliers of cell's energy, mitochondria also provide an additional compartment for a wide range of cellular processes and metabolic pathways. Mitochondria constantly undergo fusion/fission events and form a mitochondrial network, which is a highly dynamic, tubular structure allowing for rapid and continuous exchange of genetic material, as well as, targeting dysfunctional mitochondria for degradation through mitochondrial selective autophagy (mitophagy)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28273955/lack-of-xpc-leads-to-a-shift-between-respiratory-complexes-i-and-ii-but-sensitizes-cells-to-mitochondrial-stress
#17
Mateus P Mori, Rute A P Costa, Daniela T Soltys, Thiago de S Freire, Franco A Rossato, Ignácio Amigo, Alicia J Kowaltowski, Aníbal E Vercesi, Nadja C de Souza-Pinto
Genomic instability drives tumorigenesis and DNA repair defects are associated with elevated cancer. Metabolic alterations are also observed during tumorigenesis, although a causal relationship between these has not been clearly established. Xeroderma pigmentosum (XP) is a DNA repair disease characterized by early cancer. Cells with reduced expression of the XPC protein display a metabolic shift from OXPHOS to glycolysis, which was linked to accumulation of nuclear DNA damage and oxidants generation via NOX-1...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28256533/myofibroblast-differentiation-and-its-functional-properties-are-inhibited-by-nicotine-and-e-cigarette-via-mitochondrial-oxphos-complex-iii
#18
Wei Lei, Chad Lerner, Isaac K Sundar, Irfan Rahman
Nicotine is the major stimulant in tobacco products including e-cigarettes. Fibroblast to myofibroblast differentiation is a key process during wound healing and is dysregulated in lung diseases. The role of nicotine and e-cigarette derived nicotine on cellular functions including profibrotic response and other functional aspects is not known. We hypothesized that nicotine and e-cigarettes affect myofibroblast differentiation, gel contraction, and wound healing via mitochondria stress through nicotinic receptor-dependent mechanisms...
March 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28243190/maternal-l-carnitine-supplementation-improves-brain-health-in-offspring-from-cigarette-smoke-exposed-mothers
#19
Yik Lung Chan, Sonia Saad, Ibrahim Al-Odat, Brian G Oliver, Carol Pollock, Nicole M Jones, Hui Chen
Maternal cigarette smoke exposure (SE) causes detrimental changes associated with the development of chronic neurological diseases in the offspring as a result of oxidative mitochondrial damage. Maternal L-Carnitine administration has been shown to reduce renal oxidative stress in SE offspring, but its effect in the brain is unknown. Here, we investigated the effects of maternal L-Carnitine supplementation on brain markers of oxidative stress, autophagy, mitophagy and mitochondrial energy producing oxidative phosphorylation (OXPHOS) complexes in SE offspring...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28242647/inhibiting-oxidative-phosphorylation-in-vivo-restrains-th17-effector-responses-and-ameliorates-murine-colitis
#20
Luigi Franchi, Ivan Monteleone, Ling-Yang Hao, Mark A Spahr, Wenpu Zhao, Xikui Liu, Kellie Demock, Aditi Kulkarni, Chuck A Lesch, Brian Sanchez, Laura Carter, Irene Marafini, Xiao Hu, Oksana Mashadova, Min Yuan, John M Asara, Harinder Singh, Costas A Lyssiotis, Giovanni Monteleone, Anthony W Opipari, Gary D Glick
Integration of signaling and metabolic pathways enables and sustains lymphocyte function. Whereas metabolic changes occurring during T cell activation are well characterized, the metabolic demands of differentiated T lymphocytes are largely unexplored. In this study, we defined the bioenergetics of Th17 effector cells generated in vivo. These cells depend on oxidative phosphorylation (OXPHOS) for energy and cytokine production. Mechanistically, the essential role of OXPHOS in Th17 cells results from their limited capacity to increase glycolysis in response to metabolic stresses...
April 1, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
keyword
keyword
47895
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"