keyword
MENU ▼
Read by QxMD icon Read
search

OXPHOS disease

keyword
https://www.readbyqxmd.com/read/28328323/an-adaptable-high-throughput-technology-enabling-the-identification-of-specific-transcription-modulators
#1
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 Discov
https://www.readbyqxmd.com/read/28276029/assessing-mitochondrial-selective-autophagy-in-the-nematode-caenorhabditis-elegans
#2
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
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28220896/mitochondrial-dysregulation-secondary-to-endoplasmic-reticulum-stress-in-autosomal-dominant-tubulointerstitial-kidney-disease-umod-adtkd-umod
#7
Elisabeth Kemter, Thomas Fröhlich, Georg J Arnold, Eckhard Wolf, Rüdiger Wanke
'Autosomal dominant tubulointerstitial kidney disease - UMOD' (ADTKD-UMOD) is caused by impaired maturation and secretion of mutant uromodulin (UMOD) in thick ascending limb of Henle loop (TAL) cells, resulting in endoplasmic reticulum (ER) stress and unfolded protein response (UPR). To gain insight into pathophysiology, we analysed proteome profiles of TAL-enriched outer renal medulla samples from ADTKD-UMOD and control mice by quantitative LC-MS/MS. In total, 212 differentially abundant proteins were identified...
February 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28216230/activation-of-a-cryptic-splice-site-in-the-mitochondrial-elongation-factor-gfm1-causes-combined-oxphos-deficiency
#8
Mariella T Simon, Bobby G Ng, Marisa W Friederich, Raymond Y Wang, Monica Boyer, Martin Kircher, Renata Collard, Kati J Buckingham, Richard Chang, Jay Shendure, Deborah A Nickerson, Michael J Bamshad, Johan L K Van Hove, Hudson H Freeze, Jose E Abdenur
We report the clinical, biochemical, and molecular findings in two brothers with encephalopathy and multi-systemic disease. Abnormal transferrin glycoforms were suggestive of a type I congenital disorder of glycosylation (CDG). While exome sequencing was negative for CDG related candidate genes, the testing revealed compound heterozygous mutations in the mitochondrial elongation factor G gene (GFM1). One of the mutations had been reported previously while the second, novel variant was found deep in intron 6, activating a cryptic splice site...
February 12, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28208064/mitochondrial-genes-are-altered-in-blood-early-in-alzheimer-s-disease
#9
Katie Lunnon, Aoife Keohane, Ruth Pidsley, Stephen Newhouse, Joanna Riddoch-Contreras, Elisabeth B Thubron, Matthew Devall, Hikka Soininen, Iwona Kłoszewska, Patrizia Mecocci, Magda Tsolaki, Bruno Vellas, Leonard Schalkwyk, Richard Dobson, Afshan N Malik, John Powell, Simon Lovestone, Angela Hodges
Although mitochondrial dysfunction is a consistent feature of Alzheimer's disease in the brain and blood, the molecular mechanisms behind these phenomena are unknown. Here we have replicated our previous findings demonstrating reduced expression of nuclear-encoded oxidative phosphorylation (OXPHOS) subunits and subunits required for the translation of mitochondrial-encoded OXPHOS genes in blood from people with Alzheimer's disease and mild cognitive impairment. Interestingly this was accompanied by increased expression of some mitochondrial-encoded OXPHOS genes, namely those residing closest to the transcription start site of the polycistronic heavy chain mitochondrial transcript (MT-ND1, MT-ND2, MT-ATP6, MT-CO1, MT-CO2, MT-C03) and MT-ND6 transcribed from the light chain...
January 7, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28188263/overexpression-of-mitochondrial-oxodicarboxylate-carrier-odc1-preserves-oxidative-phosphorylation-in-a-yeast-model-of-the-barth-syndrome
#10
Maxence de Taffin de Tilques, Déborah Tribouillard-Tanvier, Emmanuel Tétaud, Eric Testet, Jean-Paul di Rago, Jean-Paul Lasserre
Cardiolipin (CL) is a diglycerol phospholipid mostly found in mitochondria where it optimizes numerous processes including oxidative phosphorylation (OXPHOS). To function properly CL needs to be unsaturated, which requires the acyltransferase tafazzin. Loss-of-function mutations in this protein are responsible for the Barth syndrome (BTHS), presumably because of a diminished OXPHOS capacity. Here we show that overexpressing Odc1p, a conserved oxodicarboxylic acid carrier located in the mitochondrial inner membrane, fully restores oxidative phosphorylation in a yeast model (taz1Δ) of the Barth syndrome...
February 10, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28132645/5-aminolevulinic-acid-combined-with-ferrous-ion-reduces-adiposity-and-improves-glucose-tolerance-in-diet-induced-obese-mice-via-enhancing-mitochondrial-function
#11
Urara Ota, Takeshi Hara, Hitoshi Nakagawa, Emi Tsuru, Masayuki Tsuda, Atsuko Kamiya, Yasushi Kuroda, Yuya Kitajima, Aya Koda, Masahiro Ishizuka, Hideo Fukuhara, Keiji Inoue, Taro Shuin, Motowo Nakajima, Tohru Tanaka
BACKGROUND: Mitochondrial dysfunction is associated with obesity and various obesity-associated pathological conditions including glucose intolerance. 5-Aminolevulinic acid (ALA), a precursor of heme metabolites, is a natural amino acid synthesized in the mitochondria, and various types of cytochromes containing heme contribute to aerobic energy metabolism. Thus, ALA might have beneficial effects on the reduction of adiposity and improvement of glucose tolerance through its promotion of heme synthesis...
January 30, 2017: BMC Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28117598/cr6-interacting-factor-1-deficiency-impairs-vascular-function-by-inhibiting-the-sirt1-enos-pathway
#12
Harsha Nagar, Saetbyel Jung, Min Jeong Ryu, Su Jung Choi, Shuyu Piao, Hee Jung Song, Shin Kwang Kang, Nara Shin, Dong Woon Kim, Seon-Ah Jin, Jin-Ok Jeong, Kaikobad Irani, Byeong Hwa Jeon, Minho Shong, Gi Ryang Kweon, Cuk Seong Kim
AIMS: Mitochondrial dysfunction has emerged as a major contributing factor to endothelial dysfunction and vascular disease, but the key mechanisms underlying mitochondrial dysfunction-induced endothelial dysfunction remain to be elucidated. In this study, we aim to determine whether mitochondrial dysfunction in endothelial cells plays a key role in vascular disease, by examining the phenotype of endothelial-specific CR6-interacting factor 1 (CRIF1) knockout mice. We also used siRNA mediated downregulation of CRIF1 gene in the endothelial cells to study about the in vitro pathophysiological underlying mechanisms...
January 24, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28068639/effects-of-argan-oil-on-the-mitochondrial-function-antioxidant-system-and-the-activity-of-nadph-generating-enzymes-in-acrylamide-treated-rat-brain
#13
Birsen Aydın
Argan oil (AO) is rich in minor compounds such as polyphenols and tocopherols which are powerful antioxidants. Acrylamide (ACR) has been classified as a neurotoxic agent in animals and humans. Mitochondrial oxidative stress and dysfunction is one of the most probable molecular mechanisms of neurodegenerative diseases. Female Sprague Dawley rats were exposed to ACR (50mg/kg i.p. three times a week), AO (6ml/kg,o.p, per day) or together for 30days. The activities of cytosolic enzymes such as xanthine oxidase (XO), glucose 6-phosphate dehydrogenase (G6PDH), glutathione-S-transferase (GST), mitochondrial oxidative stress, oxidative phosphorylation (OXPHOS) and tricarboxylic acid cycle (TCA) enzymes, mitochondrial metabolic function, adenosine triphosphate (ATP) level and acetylcholinesterase (AChE) activity were assessed in rat brain...
March 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28064117/differential-proteomic-and-oxidative-profiles-unveil-dysfunctional-protein-import-to-adipocyte-mitochondria-in-obesity-associated-aging-and-diabetes
#14
María Gómez-Serrano, Emilio Camafeita, Juan A López, Miguel A Rubio, Irene Bretón, Inés García-Consuegra, Eva García-Santos, Jesús Lago, Andrés Sánchez-Pernaute, Antonio Torres, Jesús Vázquez, Belén Peral
Human age-related diseases, including obesity and type 2 diabetes (T2DM), have long been associated to mitochondrial dysfunction; however, the role for adipose tissue mitochondria in these conditions remains unknown. We have tackled the impact of aging and T2DM on adipocyte mitochondria from obese patients by quantitating not only the corresponding abundance changes of proteins, but also the redox alterations undergone by Cys residues thereof. For that, we have resorted to a high-throughput proteomic approach based on isobaric labeling, liquid chromatography and mass spectrometry...
April 2017: Redox Biology
https://www.readbyqxmd.com/read/28049038/trimethylamine-n-oxide-impairs-pyruvate-and-fatty-acid-oxidation-in-cardiac-mitochondria
#15
Marina Makrecka-Kuka, Kristine Volska, Unigunde Antone, Reinis Vilskersts, Solveiga Grinberga, Dace Bandere, Edgars Liepinsh, Maija Dambrova
Increased plasma concentration of trimethylamine N-oxide (TMAO), a proatherogenic metabolite, has been linked to adverse cardiovascular outcomes; however, it remains unclear whether TMAO is a biomarker or whether it induces direct detrimental cardiovascular effects. Because altered cardiac energy metabolism and mitochondrial dysfunction play crucial roles in the development of cardiovascular diseases, we hypothesized that increased TMAO concentration may alter mitochondrial energy metabolism. The aim of the present study was to determine the effects of TMAO on cardiac mitochondrial energy metabolism...
February 5, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28025489/the-effects-of-ascorbate-n-acetylcysteine-and-resveratrol-on-fibroblasts-from-patients-with-mitochondrial-disorders
#16
Liza Douiev, Devorah Soiferman, Corinne Alban, Ann Saada
Reactive oxygen species (ROS) are assumed to be implicated in the pathogenesis of inborn mitochondrial diseases affecting oxidative phosphorylation (OXPHOS). In the current study, we characterized the effects of three small molecules with antioxidant properties (N-acetylcysteine, ascorbate, and resveratrol) on ROS production and several OXPHOS parameters (growth in glucose free medium, ATP production, mitochondrial content and membrane potential (MMP)), in primary fibroblasts derived from seven patients with different molecularly defined and undefined mitochondrial diseases...
December 22, 2016: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/27973917/mitochondrial-variations-in-the-mt-nd4-and-mt-tl1-genes-are-associated-with-male-infertility
#17
Feng Ni, Yun Zhou, Wen-Xiang Zhang, Xue-Mei Wang, Xiao-Min Song, Hong Jiang
Mitochondrial gene mutations have been reported to be associated with sperm motility and the quality of semen. The aim of this study was to investigate whether the two mitochondrial genes (MT-ND4 and MT-TL1) are involved in Chinese male infertility. A total of 97 asthenospermia patients and 80 fertile controls were recruited in this case-control study. Genomic DNA were extracted from the sperm of all participants. Two mitochondrial DNA genes (MT-ND4 and MT-TL1) were amplified by using polymerase chain reaction (PCR) with the gene-specific primers and sequenced on an ABI 3730XL DNA sequencer...
December 14, 2016: Systems Biology in Reproductive Medicine
https://www.readbyqxmd.com/read/27940153/fyn-kinase-regulates-translation-in-mammalian-mitochondria
#18
Emine C Koc, Jennifer L Miller-Lee, Hasan Koc
BACKGROUND: Mitochondrial translation machinery solely exists for the synthesis of 13 mitochondrially-encoded subunits of the oxidative phosphorylation (OXPHOS) complexes in mammals. Therefore, it plays a critical role in mitochondrial energy production. However, regulation of the mitochondrial translation machinery is still poorly understood. In comprehensive proteomics studies with normal and diseased tissues and cell lines, we and others have found the majority of mitochondrial ribosomal proteins (MRPs) to be phosphorylated...
March 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27871875/pyruvate-dehydrogenase-has-a-major-role-in-mast-cell-function-and-its-activity-is-regulated-by-mitochondrial-microphthalmia-transcription-factor
#19
Israa Sharkia, Tal Hadad Erlich, Nadine Landolina, Miri Assayag, Alex Motzik, Inbal Rachmin, Gillian Kay, Ziv Porat, Sagi Tshori, Neville Berkman, Francesca Levi-Schaffer, Ehud Razin
BACKGROUND: We have recently observed that oxidative phosphorylation-mediated ATP production is essential for mast cell function. Pyruvate dehydrogenase (PDH) is the main regulator of the Krebs cycle and is located upstream of the electron transport chain. However, the role of PDH in mast cell function has not been described. Microphthalmia transcription factor (MITF) regulates the development, number, and function of mast cells. Localization of MITF to the mitochondria and its interaction with mitochondrial proteins has not been explored...
November 18, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27812541/muscle-oxidative-phosphorylation-quantitation-using-creatine-chemical-exchange-saturation-transfer-crcest-mri-in-mitochondrial-disorders
#20
Catherine DeBrosse, Ravi Prakash Reddy Nanga, Neil Wilson, Kevin D'Aquilla, Mark Elliott, Hari Hariharan, Felicia Yan, Kristin Wade, Sara Nguyen, Diana Worsley, Chevonne Parris-Skeete, Elizabeth McCormick, Rui Xiao, Zuela Zolkipli Cunningham, Lauren Fishbein, Katherine L Nathanson, David R Lynch, Virginia A Stallings, Marc Yudkoff, Marni J Falk, Ravinder Reddy, Shana E McCormack
Systemic mitochondrial energy deficiency is implicated in the pathophysiology of many age-related human diseases. Currently available tools to estimate mitochondrial oxidative phosphorylation (OXPHOS) capacity in skeletal muscle in vivo lack high anatomic resolution. Muscle groups vary with respect to their contractile and metabolic properties. Therefore, muscle group-specific estimates of OXPHOS would be advantageous. To address this need, a noninvasive creatine chemical exchange saturation transfer (CrCEST) MRI technique has recently been developed, which provides a measure of free creatine...
November 3, 2016: JCI Insight
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"