Genomes and Genes
Summary: Electron transfer through the cytochrome system liberating free energy which is transformed into high-energy phosphate bonds.
Publications277 found, 100 shown here
- PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetesVamsi K Mootha
Whitehead Institute MIT Center for Genome Research, Cambridge, Massachusetts, USA
Nat Genet 34:267-73. 2003..Using this approach, we identify a set of genes involved in oxidative phosphorylation whose expression is coordinately decreased in human diabetic muscle...
- Multiparameter metabolic analysis reveals a close link between attenuated mitochondrial bioenergetic function and enhanced glycolysis dependency in human tumor cellsMin Wu
Seahorse Bioscience, 16 Esquire Road, North Billerica, MA 01862, USA
Am J Physiol Cell Physiol 292:C125-36. 2007..The converse, however, was not true. The cell lines were attenuated in oxidative phosphorylation (OXPHOS) capacity and were unable to sufficiently upregulate mitochondrial OXPHOS when glycolysis was ..
- Energy substrate modulates mitochondrial structure and oxidative capacity in cancer cellsRodrigue Rossignol
Institute of Molecular Biology, University of Oregon, Eugene, Oregon, USA
Cancer Res 64:985-93. 2004..tumors relative to normal tissues generally reveals a strong diminution in mitochondrial content and in oxidative phosphorylation capacity...
- The role of mitochondria in the pathogenesis of type 2 diabetesMary Elizabeth Patti
Research Division, Joslin Diabetes Center, Boston, Massachusetts 02215, USA
Endocr Rev 31:364-95. 2010....
- Mitochondrial dysfunction and type 2 diabetesBradford B Lowell
Department of Medicine, Beth Israel Deaconess Medical Center, 99 Brookline Avenue, Harvard Medical School, Boston, MA 02215, USA
Science 307:384-7. 2005..Emerging evidence supports the potentially unifying hypothesis that both of these prominent features of type 2 diabetes are caused by mitochondrial dysfunction...
- Mitochondrial diseases in man and mouseD C Wallace
Center for Molecular Medicine, Emory University, 1462 Clifton Road, Suite 420, Atlanta, GA 30322, USA
Science 283:1482-8. 1999..The essential role of mitochondrial oxidative phosphorylation in cellular energy production, the generation of reactive oxygen species, and the initiation of ..
- Corepressor SMRT promotes oxidative phosphorylation in adipose tissue and protects against diet-induced obesity and insulin resistanceSungsoon Fang
Gene Expression Laboratory, Salk Institute for Biological Studies, La Jolla, CA, USA
Proc Natl Acad Sci U S A 108:3412-7. 2011....
- Coordinated reduction of genes of oxidative metabolism in humans with insulin resistance and diabetes: Potential role of PGC1 and NRF1Mary Elizabeth Patti
Research Division, Joslin Diabetes Center, Boston, MA 02215, USA
Proc Natl Acad Sci U S A 100:8466-71. 2003..Decreased PGC1 expression may be responsible for decreased expression of NRF-dependent genes, leading to the metabolic disturbances characteristic of insulin resistance and DM...
- Amyloid-beta and tau synergistically impair the oxidative phosphorylation system in triple transgenic Alzheimer's disease miceVirginie Rhein
Neurobiology Laboratory for Brain Aging and Mental Health, Psychiatric University Clinics, University of Basel, 4025 Basel, Switzerland
Proc Natl Acad Sci U S A 106:20057-62. 2009..24 proteins, of which one-third were mitochondrial proteins mainly related to complexes I and IV of the oxidative phosphorylation system (OXPHOS)...
- Mitochondrial medicine--molecular pathology of defective oxidative phosphorylationE Fosslien
Department of Pathology, College of Medicine, University of Illinois at Chicago, 60612, USA
Ann Clin Lab Sci 31:25-67. 2001Different tissues display distinct sensitivities to defective mitochondrial oxidative phosphorylation (OXPHOS)...
- Choosing between glycolysis and oxidative phosphorylation: a tumor's dilemma?Caroline Jose
MRGM, Laboratoire Maladies Rares Génétique et Métabolisme, F 33076 Bordeaux, France
Biochim Biophys Acta 1807:552-61. 2011..cell lines is well described and recent analyses give a better view of the changes in mitochondrial oxidative phosphorylation during oncogenesis...
- LRPPRC and SLIRP interact in a ribonucleoprotein complex that regulates posttranscriptional gene expression in mitochondriaFlorin Sasarman
Department of Human Genetics, Montreal Neurological Institute, McGill University, Montreal, QC, H3A 2B4, Canada
Mol Biol Cell 21:1315-23. 2010..Further knockdown of LRPPRC produces a generalized assembly defect in all oxidative phosphorylation complexes containing mtDNA-encoded subunits, due to a severe decrease in all mitochondrial mRNAs...
- High-resolution respirometry: OXPHOS protocols for human cells and permeabilized fibers from small biopsies of human muscleDominik Pesta
D Swarovski Research Laboratory, Department of General and Transplant Surgery, Medical University of Innsbruck, Innrain 52, Christoph Probst Platz, 6020 Innsbruck, Austria
Methods Mol Biol 810:25-58. 2012..Multiple substrate-uncoupler-inhibitor titration (SUIT) protocols for analysis of oxidative phosphorylation improve our understanding of mitochondrial respiratory control and the pathophysiology of mitochondrial ..
- Ketones and lactate increase cancer cell "stemness," driving recurrence, metastasis and poor clinical outcome in breast cancer: achieving personalized medicine via Metabolo-GenomicsUbaldo E Martinez-Outschoorn
The Jefferson Stem Cell Biology and Regenerative Medicine Center, Thomas Jefferson University, Philadelphia, PA, USA
Cell Cycle 10:1271-86. 2011..Importantly, this powerful new approach directly links cancer cell metabolism with clinical outcome, and new therapeutic strategies for inhibiting the TCA cycle and mitochondrial oxidative phosphorylation in cancer cells.
- Somatic oxidative bioenergetics transitions into pluripotency-dependent glycolysis to facilitate nuclear reprogrammingClifford D L Folmes
Center for Regenerative Medicine and Marriott Heart Disease Research Program, Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA
Cell Metab 14:264-71. 2011..Functional metamorphosis of somatic oxidative phosphorylation into acquired pluripotent glycolytic metabolism conformed to an embryonic-like archetype...
- Respiratory active mitochondrial supercomplexesRebeca Acín-Pérez
Departamento de Bioquimica, Universidad de Zaragoza, Miguel Servet, 177, 50013 Zaragoza, Spain
Mol Cell 32:529-39. 2008..Therefore, we have demonstrated the existence of a functional respirasome and propose a structural organization model that accommodates these findings...
- The estrogen-related receptor alpha (ERRalpha) functions in PPARgamma coactivator 1alpha (PGC-1alpha)-induced mitochondrial biogenesisSylvia N Schreiber
Biozentrum, University of Basel, CH 4056 Basel, Switzerland
Proc Natl Acad Sci U S A 101:6472-7. 2004..gamma (PPARgamma) coactivator 1alpha], and that it regulates the expression of genes involved in oxidative phosphorylation and mitochondrial biogenesis...
- Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficienciesPaulien Smits
Department of Pediatrics, Nijmegen Center for Mitochondrial Disorders, Radboud University Nijmegen Medical Center, Geert Grooteplein 10, P O Box 9101, 6500 HB Nijmegen, The Netherlands
J Biomed Biotechnol 2010:737385. 2010..These disorders are caused by defects in the oxidative phosphorylation (OXPHOS) system, which comprises five multisubunit enzyme complexes encoded by both the nuclear and the ..
- A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasisJoshua M Baughman
Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA, USA
PLoS Genet 5:e1000590. 2009The human oxidative phosphorylation (OxPhos) system consists of approximately 90 proteins encoded by nuclear and mitochondrial genomes and serves as the primary cellular pathway for ATP biosynthesis...
- Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetesJ P Silva
Department of Molecular Medicine, Karolinska Institutet, Karolinska Hospital, Stockholm, Sweden
Nat Genet 26:336-40. 2000..developed diabetes from the age of approximately 5 weeks and displayed severe mtDNA depletion, deficient oxidative phosphorylation and abnormal appearing mitochondria in islets at the ages of 7-9 weeks...
- Skeletal muscle "mitochondrial deficiency" does not mediate insulin resistanceJohn O Holloszy
Division of Geriatrics and Nutritional Sciences, Washington University School of Medicine, St Louis, MO 63110, USA
Am J Clin Nutr 89:463S-6S. 2009..Furthermore, it was recently shown that skeletal muscle mitochondrial capacity for oxidative phosphorylation in Asian Indians with type 2 diabetes is the same as in nondiabetic Indians and higher than in healthy ..
- Waves of gene regulation suppress and then restore oxidative phosphorylation in cancer cellsKatarina Smolkova
Department of Membrane Transport Biophysics, Institute of Physiology, vvi, Academy of Sciences of the Czech Republic, Prague, Czech Republic
Int J Biochem Cell Biol 43:950-68. 2011..wave" of adaptation stimulates glutaminolysis, which in certain cases partially re-establishes oxidative phosphorylation; this involves the LKB1-AMPK-p53, PI3K-Akt-mTOR axes and MYC dysregulation...
- Attenuation of LDH-A expression uncovers a link between glycolysis, mitochondrial physiology, and tumor maintenanceValeria R Fantin
Department of Genetics, Harvard Medical School and Howard Hughes Medical Institute, Boston, Massachusetts 02115, USA
Cancer Cell 9:425-34. 2006..These results demonstrate that LDH-A plays a key role in tumor maintenance...
- Circumventing the Crabtree effect: replacing media glucose with galactose increases susceptibility of HepG2 cells to mitochondrial toxicantsLisa D Marroquin
Pfizer DSRD, San Diego, CA 92121, USA
Toxicol Sci 97:539-47. 2007..effects in a preclinical cell-based assay, HepG2 cells were forced to rely on mitochondrial oxidative phosphorylation rather than glycolysis by substituting galactose for glucose in the growth media...
- Implications of mitochondrial DNA mutations and mitochondrial dysfunction in tumorigenesisJianxin Lu
Zhejiang Provincial Key Laboratory of Medical Genetics, School of Laboratory Medicine and Life Science, Wenzhou Medical College, Wenzhou, China
Cell Res 19:802-15. 2009Alterations in oxidative phosphorylation resulting from mitochondrial dysfunction have long been hypothesized to be involved in tumorigenesis...
- TEFM (c17orf42) is necessary for transcription of human mtDNAMichal Minczuk
MRC Mitochondrial Biology Unit, Wellcome Trust MRC Building, Hills Road, Cambridge CB2 0XY, UK
Nucleic Acids Res 39:4284-99. 2011..TEFM contains two HhH motifs and a Ribonuclease H fold, similar to the nuclear transcription elongation regulator Spt6. These findings lead us to propose that TEFM is a mitochondrial transcription elongation factor...
- Impaired mitochondrial activity in the insulin-resistant offspring of patients with type 2 diabetesKitt Falk Petersen
Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06510, USA
N Engl J Med 350:664-71. 2004..Insulin resistance appears to be the best predictor of the development of diabetes in the children of patients with type 2 diabetes, but the mechanism responsible is unknown...
- Uncoupling to survive? The role of mitochondrial inefficiency in ageingM D Brand
MRC Dunn Human Nutrition Unit, Hills Road, CB2 2XY, Cambridge, UK
Exp Gerontol 35:811-20. 2000Mitochondria are incompletely coupled, and during oxidative phosphorylation some of the redox energy in substrates is lost as heat. Incomplete coupling is mostly due to a natural leak of protons across the mitochondrial inner membrane...
- Energetic requirements and bioenergetic modulation of mitochondrial morphology and dynamicsCécile Sauvanet
Institut de biochimie et génétique cellulaires IBGC, CNRS UMR 5095, Bordeaux, France
Semin Cell Dev Biol 21:558-65. 2010..Finally, ex vivo studies (in mammals) and in vitro studies (in yeast) confirm the essential role of the inner membrane potential for mitochondrial fusion...
- Impaired oxidative phosphorylation in skeletal muscle of intrauterine growth-retarded ratsMary A Selak
Department of Pediatrics, Children s Hospital of Philadelphia and University of Pennsylvania, 19104, USA
Am J Physiol Endocrinol Metab 285:E130-7. 2003..Such a defect in IUGR mitochondria leads to a chronic reduction in the supply of ATP available from oxidative phosphorylation. Impaired ATP synthesis in muscle compromises energy-dependent GLUT4 recruitment to the cell surface, ..
- Up-regulation of key microRNAs, and inverse down-regulation of their predicted oxidative phosphorylation target genes, during aging in mouse brainNa Li
Gheens Center on Aging, University of Louisville School of Medicine, USA
Neurobiol Aging 32:944-55. 2011..We found unique de-regulated microRNAs shared between aging brain and aging liver, as well as brain- vs. liver-specific microRNAs during normal aging...
- Supramolecular organisation of the mitochondrial respiratory chain: a new challenge for the mechanism and control of oxidative phosphorylationGiorgio Lenaz
Department of Biochemistry, University of Bologna, Bologna, Italy
Adv Exp Med Biol 748:107-44. 2012....
- mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcriptionTina Wenz
Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA
Cell Metab 9:499-511. 2009Regulation of mitochondrial protein expression is crucial for the function of the oxidative phosphorylation (OXPHOS) system...
- CRIF1 is essential for the synthesis and insertion of oxidative phosphorylation polypeptides in the mammalian mitochondrial membraneSoung Jung Kim
Research Center for Endocrine and Metabolic Diseases, Chungnam National University School of Medicine, Daejeon 301 721, Korea
Cell Metab 16:274-83. 2012..factors involved in mitoribosome-mediated synthesis and simultaneous insertion of mitochondrial oxidative phosphorylation (OXPHOS) polypeptides into the inner membrane of mitochondria are still unclear...
- K-ras(G12V) transformation leads to mitochondrial dysfunction and a metabolic switch from oxidative phosphorylation to glycolysisYumin Hu
State Key Laboratory of Oncology in Southern China, Sun Yat sen University Cancer Center, Guangzhou, Guangdong 510275, China
Cell Res 22:399-412. 2012..Our study suggests that induction of mitochondrial dysfunction is an important mechanism by which K-ras(G12V) causes metabolic changes and ROS stress in cancer cells, and promotes tumor development...
- Dual function of a new nuclear gene for oxidative phosphorylation and vegetative growth in yeastT Lisowsky
Botanisches Institut, Heinrich Heine Universitat Dusseldorf, FRG
Mol Gen Genet 232:58-64. 1992..This conditional nuclear mutation selectively affects oxidative phosphorylation at restrictive temperatures...
- Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fissionWencheng Liu
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY 10065, USA
Proc Natl Acad Sci U S A 108:12920-4. 2011..Taken together, these results suggest a unique pathogenic mechanism of PINK1 PD: The loss of PINK1 impairs mitochondrial fission, which causes defective assembly of the ETC complexes, leading to abnormal bioenergetics...
- Phenethyl isothiocyanate inhibits oxidative phosphorylation to trigger reactive oxygen species-mediated death of human prostate cancer cellsDong Xiao
Department of Pharmacology and Chemical Biology and University of Pittsburgh Cancer Institute, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213, USA
J Biol Chem 285:26558-69. 2010..cell death is initiated by production of reactive oxygen species (ROS) resulting from inhibition of oxidative phosphorylation (OXPHOS)...
- Mitochondrial H(+) leak and ROS generation: an odd couplePaul S Brookes
Department of Anesthesiology, University of Rochester, Medical Center Box 604, 601 Elmwood Avenue, Rochester, NY 14642, USA
Free Radic Biol Med 38:12-23. 2005The single-electron chemistry of mitochondrial oxidative phosphorylation (ox-phos) by default generates reactive oxygen species (ROS). These ROS have roles in both physiologic cell signaling and numerous pathologic situations...
- MicroRNA-338 regulates local cytochrome c oxidase IV mRNA levels and oxidative phosphorylation in the axons of sympathetic neuronsArmaz Aschrafi
Laboratory of Molecular Biology, National Institute of Mental Health National Institutes of Health, Bethesda, Maryland 20892 1381, USA
J Neurosci 28:12581-90. 2008..oligonucleotides that inhibit miR-338 increases COXIV levels, and results in a significant increase in oxidative phosphorylation and also norepinephrine uptake in the axons...
- Physiological diversity of mitochondrial oxidative phosphorylationG Benard
INSERM U688, Physiopathologie Mitochondriale, Universite Victor Segalen Bordeaux 2, Bordeaux, France
Am J Physiol Cell Physiol 291:C1172-82. 2006To investigate the physiological diversity in the regulation and control of mitochondrial oxidative phosphorylation, we determined the composition and functional features of the respiratory chain in muscle, heart, liver, kidney, and ..
- Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondriaVamsi K Mootha
MDS Proteomics, Odense 5230, Denmark
Cell 115:629-40. 2003....
- Computer modeling of mitochondrial tricarboxylic acid cycle, oxidative phosphorylation, metabolite transport, and electrophysiologyFan Wu
Biotechnology and Bioengineering Center and Department of Physiology, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA
J Biol Chem 282:24525-37. 2007....
- OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusionClaudia Zanna
Dipartimento di Biologia Evoluzionistica Sperimentale, Universita di Bologna, Via Irnerio 42, 40126 Bologna, Italy
Brain 131:352-67. 2008..The results disclose a novel link between OPA1, apoptosis inducing factor and the respiratory complexes that may shed some light on the pathogenic mechanism of DOA...
- Effects of a high-fat diet on energy metabolism and ROS production in rat liverGuillaume Vial
INSERM, U884, Grenoble, F 38041 France
J Hepatol 54:348-56. 2011..Steatosis is still poorly understood since diverse effects have been reported, depending on the different experimental models used...
- Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylationDaniel J M Fernández-Ayala
Institute of Medical Technology, Tampere University Hospital, University of Tampere, 33014 Tampere, Finland
Cell Metab 9:449-60. 2009..AOX appears to offer promise as a wide-spectrum therapeutic tool in OXPHOS disorders...
- Capacity of oxidative phosphorylation in human skeletal muscle: new perspectives of mitochondrial physiologyErich Gnaiger
Department of General and Transplant Surgery, D Swarovski Research Laboratory, Medical University of Innsbruck, Innrain 66 6, A 6020 Innsbruck, Austria
Int J Biochem Cell Biol 41:1837-45. 2009..50-0.75 of capacity of oxidative phosphorylation (OXPHOS)...
- Mitochondrial p32 protein is a critical regulator of tumor metabolism via maintenance of oxidative phosphorylationValentina Fogal
Cancer Research Center, Burnham Institute for Medical Research, 10901 N Torrey Pines Rd, La Jolla, California 92037, USA
Mol Cell Biol 30:1303-18. 2010..we show that knocking down p32 expression in human cancer cells strongly shifts their metabolism from oxidative phosphorylation (OXPHOS) to glycolysis...
- Warburg, me and Hexokinase 2: Multiple discoveries of key molecular events underlying one of cancers' most common phenotypes, the "Warburg Effect", i.e., elevated glycolysis in the presence of oxygenPeter L Pedersen
Department of Biological Chemistry, Johns Hopkins University, School of Medicine, 725 North Wolfe Street, Baltimore, MD, 21205 2185, USA
J Bioenerg Biomembr 39:211-22. 2007..e., glycolysis and mitochondria) resulting in tumor destruction without harm to the animals...
- Identification of tyrosine-phosphorylated proteins of the mitochondrial oxidative phosphorylation machineryO Augereau
Physiopathologie Mitochondriale, INSERM U688, Universite Victor Segalen Bordeaux 2, 146 rue Leo Saignat, 33076 Bordeaux Cedex, France
Cell Mol Life Sci 62:1478-88. 2005..The tyrosine phosphatase PTP 1B was found in brain but not in muscle, heart or liver mitochondria. Our results suggest that tyrosine kinases and phosphatases are involved in the regulation of oxidative phosphorylation.
- Coevolution predicts direct interactions between mtDNA-encoded and nDNA-encoded subunits of oxidative phosphorylation complex iMoran Gershoni
Department of Life Sciences and the Nation Institute of Biotechnology in the Negev, Ben Gurion University of the Negev, Beer Sheva 84105, Israel
J Mol Biol 404:158-71. 2010Despite years of research, the structure of the largest mammalian oxidative phosphorylation (OXPHOS) complex, NADH-ubiquinone oxidoreductase (complex I), and the interactions among its 45 subunits are not fully understood...
- Mutations in the ND5 subunit of complex I of the mitochondrial DNA are a frequent cause of oxidative phosphorylation diseaseM J Blok
Department of Clinical Genetics, University Hospital, Maastricht, The Netherlands
J Med Genet 44:e74. 2007..However, mutations in other mtDNA regions can be an important cause of oxidative phosphorylation (OXPHOS) disease as well.
- Components of mitochondrial oxidative phosphorylation vary in abundance following exposure to cold and chemical stressesYew Foon Tan
ARC Centre of Excellence in Plant Energy Biology, MCS building M316, The University of Western Australia, 35 Stirling Highway, Crawley WA 6009, Western Australia, Australia
J Proteome Res 11:3860-79. 2012..of plant mitochondria, arguably the site responsible for the most fundamental mitochondrial processes of oxidative phosphorylation, protein import and metabolite transport, remain a technical challenge...
- Mutations in the mitochondrial methionyl-tRNA synthetase cause a neurodegenerative phenotype in flies and a recessive ataxia (ARSAL) in humansVafa Bayat
Program in Developmental Biology, Baylor College of Medicine, Houston, Texas, United States of America
PLoS Biol 10:e1001288. 2012..We further observed that these mutants display defects in oxidative phosphorylation, increased Reactive Oxygen Species (ROS), and an upregulated mitochondrial Unfolded Protein Response...
- Evolution meets disease: penetrance and functional epistasis of mitochondrial tRNA mutationsRaquel Moreno-Loshuertos
Departamento de Bioquimica y Biologia Molecular y Celular, Universidad de Zaragoza, Zaragoza, Spain
PLoS Genet 7:e1001379. 2011..This particular behavior can allow a scenario for the evolution of mitochondrial tRNAs in which the fixation of two alleles that are individually deleterious can proceed in two steps and not require the simultaneous mutation of both...
- Increase in mitochondrial DNA mutations impairs retinal function and renders the retina vulnerable to injuryYu X G Kong
Centre for Eye Research Australia, University of Melbourne, Parkville, 3010 Victoria, Australia
Aging Cell 10:572-83. 2011..mice have increased mtDNA deletions and point mutations and decreased expression of mitochondrial oxidative phosphorylation enzymes...
- Mitochondrial respiration protects against oxygen-associated DNA damageHo Joong Sung
Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
Nat Commun 1:5. 2010Oxygen is not only required for oxidative phosphorylation but also serves as the essential substrate for the formation of reactive oxygen species (ROS), which is implicated in ageing and tumorigenesis...
- Increased mitochondrial oxidative phosphorylation in the liver is associated with obesity and insulin resistanceDavid A Buchner
Department of Genetics, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
Obesity (Silver Spring) 19:917-24. 2011..between 6C1 and 6C2 in pathways related to basal transcription factors, endocytosis, and mitochondrial oxidative phosphorylation (OxPhos)...
- Mitochondrial dysfunction in glaucoma and emerging bioenergetic therapiesShanjean Lee
Centre for Eye Research Australia, University of Melbourne, Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, 32 Gisborne Street East, Melbourne, Victoria 3002, Australia
Exp Eye Res 93:204-12. 2011....
- Subcomplex Ilambda specifically controls integrated mitochondrial functions in Caenorhabditis elegansMarni J Falk
Division of Human Genetics, Department of Pediatrics, The Children s Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA, USA
PLoS ONE 4:e6607. 2009..We demonstrate that functional consequences of complex I deficiency vary with the particular subunit that is defective...
- UVB radiation induces persistent activation of ribosome and oxidative phosphorylation pathwaysMin Lung Tsai
Department of Biomedical Engineering, National Tsing Hua University, Hsinchu, Taiwan
Radiat Res 171:716-24. 2009..we determined that genes participating in two prime cellular processes, the ribosome pathway and the oxidative phosphorylation pathway, were persistently activated after UVB irradiation...
- Tissue-specific remodeling of the mitochondrial proteome in type 1 diabetic akita miceHeiko Bugger
Division of Endocrinology, Metabolism and Diabetes, and Program in Molecular Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA
Diabetes 58:1986-97. 2009..To elucidate the molecular basis for mitochondrial dysfunction, which has been implicated in the pathogenesis of diabetes complications...
- The role of Ca(2+) signaling in the coordination of mitochondrial ATP production with cardiac workRobert S Balaban
Laboratory of Cardiac Energetics, National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
Biochim Biophys Acta 1787:1334-41. 2009..How cytosolic Ca(2+) signaling might regulate oxidative phosphorylation is a focus of this review...
- A disproportionate role for mtDNA in Dobzhansky-Muller incompatibilities?Ronald S Burton
Marine Biology Research Division, Scripps Institution of Oceanography, University of California, San Diego, La Jolla, CA 92093 0202, USA
Mol Ecol 21:4942-57. 2012..All mtDNA-encoded proteins are components of the oxidative phosphorylation (OXPHOS) system and all mtDNA-encoded RNAs are part of the mitochondrial protein synthetic machinery; ..
- Seizure-dependent modulation of mitochondrial oxidative phosphorylation in rat hippocampusAlexei P Kudin
Department of Epileptology, University of Bonn Medical Centre, Sigmund Freud Str 25, D 53105 Bonn, Germany
Eur J Neurosci 15:1105-14. 2002..Here, we demonstrate seizure-dependent changes in mitochondrial oxidative phosphorylation in the epileptic rat hippocampus...
- Mitochondrial expression and function of GAS-1 in Caenorhabditis elegansE B Kayser
Department of Anesthesiology, University Hospitals, Case Western Reserve University, Cleveland, Ohio 44106, USA
J Biol Chem 276:20551-8. 2001..gene profoundly decreases Complex I-dependent metabolism in mitochondria as measured by rates of both oxidative phosphorylation and electron transport...
- Review: Mitochondrial medicine--cardiomyopathy caused by defective oxidative phosphorylationEgil Fosslien
Department of Pathology, College of Medicine, University of Illinois at Chicago, Chicago, Illinois 60612, USA
Ann Clin Lab Sci 33:371-95. 2003..the majority of its requirement for adenosine triphosphate (ATP) from metabolism of fatty acids and oxidative phosphorylation (OXPHOS), to the fetal form, which metabolizes glucose and lactate...
- High membrane potential promotes alkenal-induced mitochondrial uncoupling and influences adenine nucleotide translocase conformationVian Azzu
MRC Dunn Human Nutrition Unit, Wellcome Trust MRC Building, Hills Road, Cambridge, CB2 0XY, UK
Biochem J 413:323-32. 2008..species, whose downstream lipid peroxidation products, such as 4-hydroxynonenal, induce uncoupling of oxidative phosphorylation by increasing proton leak through mitochondrial inner membrane proteins such as the uncoupling proteins ..
- A pivotal role for p53: balancing aerobic respiration and glycolysisWenzhe Ma
Cardiology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
J Bioenerg Biomembr 39:243-6. 2007..The biological significance of this regulation of the two major pathways of energy generation by p53 remains to be seen...
- Mitochondrial disordersMassimo Zeviani
Division of Molecular Neurogenetics, Istituto Nazionale Neurologico C Besta, Via Celoria 11 20133 Milan, Italy
Brain 127:2153-72. 2004..e. oxidative phosphorylation (OXPHOS)...
- Defective oxidative phosphorylation in thyroid oncocytic carcinoma is associated with pathogenic mitochondrial DNA mutations affecting complexes I and IIIElena Bonora
Unità di Genetica Medica, Policlinico Universitario S Orsola Malpighi, Italy
Cancer Res 66:6087-96. 2006..UC1 is due to a combined complex I/III defect associated with mtDNA mutations, as proven by the transfer of the defective energetic phenotype with the mitochondrial genome into the cybrids...
- Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxinsMarina A Schwab
Department of General Pediatrics, Division of Inborn Metabolic Diseases, University Children s Hospital Heidelberg, Im Neuenheimer Feld 150, D 69120 Heidelberg, Germany
Biochem J 398:107-12. 2006..In line with the in vitro results, oxidative phosphorylation was severely compromised in both patients...
- Resveratrol protects against cold ischemia-warm reoxygenation-induced damages to mitochondria and cells in rat liverCatherine Plin
INSERM, U660, Creteil, F 94010 France
Eur J Pharmacol 528:162-8. 2005..cellular damages as attested by the increase in lipid peroxidation of liver membranes, the alteration of oxidative phosphorylation parameters, mitochondrial swelling and the activation of the cellular markers of necrosis and apoptosis, ..
- A Candida albicans petite mutant strain with uncoupled oxidative phosphorylation overexpresses MDR1 and has diminished susceptibility to fluconazole and voriconazoleShaoji Cheng
University of Florida College of Medicine, JHMHC, Gainesville, FL 32610, USA
Antimicrob Agents Chemother 51:1855-8. 2007We showed that a Candida albicans petite mutant in which oxidative phosphorylation is uncoupled was eightfold more resistant to fluconazole and voriconazole than SC5314 but equally susceptible to ketoconazole, itraconazole, and ..
- Hereditary optic neuropathies share a common mitochondrial coupling defectArnaud Chevrollier
Institut National de la Sante et de la Recherche Medicale, U694, Départment de Biochimie et Génétique, Centre Hospitalier Universitaire, Angers, France
Ann Neurol 63:794-8. 2008..We found a common coupling defect of oxidative phosphorylation in fibroblasts of patients affected by autosomal dominant optic atrophy (mutations of OPA1), autosomal ..
- Cardiac mitochondria in heart failure: decrease in respirasomes and oxidative phosphorylationMariana G Rosca
Department of Medicine, School of Medicine, Case Western Reserve University, 10900 Euclid Avenue, Cleveland 44106 4981, OH, USA
Cardiovasc Res 80:30-9. 2008..The aim of this study was to define the defect in the integrative function of cardiac mitochondria in coronary microembolization-induced HF...
- Energy thresholds in brain mitochondria. Potential involvement in neurodegenerationG P Davey
Department of Neurochemistry, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom
J Biol Chem 273:12753-7. 1998..However, the extent to which these decreases cause a disturbance in oxidative phosphorylation and energy homeostasis in the brain is not known...
- Biochemical diagnosis of mitochondrial disordersRichard J T Rodenburg
Nijmegen Center for Mitochondrial Disorders NCMD, 656 Department of Pediatrics, Department of Laboratory Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
J Inherit Metab Dis 34:283-92. 2011..In addition to activity measurements of individual oxidative phosphorylation enzymes, analysis of mitochondrial respiration, substrate oxidation, and ATP production rates is ..
- Thioredoxin-interacting protein (Txnip) gene expression: sensing oxidative phosphorylation status and glycolytic rateFa Xing Yu
Institute of Molecular and Cell Biology, 61 Biopolis Drive, Proteos, Singapore
J Biol Chem 285:25822-30. 2010..Txnip expression is down-regulated at the transcriptional level by diverse inhibitors of mitochondrial oxidative phosphorylation (OXPHOS)...
- Alterations in bioenergetics due to changes in mitochondrial DNA copy numberWei Qian
Department of Pharmacology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, United States
Methods 51:452-7. 2010..of mtDNA due to dysfunctional mitochondrial DNA polymerase gamma (POLG) induce loss of mitochondrial oxidative phosphorylation (OXPHOS) and mitochondrial ATP generation...
- AIF deficiency compromises oxidative phosphorylationNicola Vahsen
CNRS UMR8125, Institut Gustave Roussy, Villejuif, France
EMBO J 23:4679-89. 2004..mice with reduced AIF expression due to a retroviral insertion into the AIF gene also manifest a reduced oxidative phosphorylation (OXPHOS) in the retina and in the brain, correlating with reduced expression of complex I subunits, ..
- Transcriptional profiling of myotubes from patients with type 2 diabetes: no evidence for a primary defect in oxidative phosphorylation genesC M Frederiksen
Department of Endocrinology, Odense University Hospital, Kløvervaenget 6, 5000, Odense C, Denmark
Diabetologia 51:2068-77. 2008..It is unknown whether reduced mitochondrial biogenesis or other transcriptional alterations co-exist with impaired insulin responsiveness in primary human muscle cells from patients with type 2 diabetes...
- The oncogene c-Myc coordinates regulation of metabolic networks to enable rapid cell cycle entryFionnuala Morrish
Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington, USA
Cell Cycle 7:1054-66. 2008..with an inducible c-myc transgene (mycER), we show that the Myc protein programs cells to utilize both oxidative phosphorylation and glycolysis to drive cell cycle progression...
- Measurement of the energy-generating capacity of human muscle mitochondria: diagnostic procedure and application to human pathologyAntoon J M Janssen
Department of Pediatrics and Laboratory of Pediatrics and Neurology, the Nijmegen Centre for Mitochondrial Disorders NCMD, Nijmegen, The Netherlands
Clin Chem 52:860-71. 2006..Diagnosis of mitochondrial disorders usually requires a muscle biopsy to examine mitochondrial function. We describe our diagnostic procedure and results for 29 patients with mitochondrial disorders...
- Age influences DNA methylation and gene expression of COX7A1 in human skeletal muscleT Rönn
Department of Clinical Sciences, CRC Malmö University Hospital, Lund University, 205 02, Malmo, Sweden
Diabetologia 51:1159-68. 2008..Moreover, a set of genes influencing oxidative phosphorylation (OXPHOS) is downregulated in diabetic muscle...
- Threshold effect and tissue specificity. Implication for mitochondrial cytopathiesR Rossignol
INSERM EMI 9929, , , F 33076 Bordeaux cedex, France
J Biol Chem 274:33426-32. 1999..It is based on the metabolic expression of the defect in oxidative phosphorylation (OXPHOS) complexes that can present a biochemical threshold...
- Experimental assessment of bioenergetic differences caused by the common European mitochondrial DNA haplogroups H and TTaku Amo
MRC Dunn Human Nutrition Unit, Hills Road, Cambridge CB2 0XY, UK
Gene 411:69-76. 2008..We found that there were no functionally-important bioenergetic differences between mitochondria bearing these haplogroups, using either isolated mitochondria or mitochondria within cells...
- Loss of p53 causes mitochondrial DNA depletion and altered mitochondrial reactive oxygen species homeostasisMaria A Lebedeva
Department of Pathology, Yale University School of Medicine, New Haven, CT 06520 8023, USA
Biochim Biophys Acta 1787:328-34. 2009..Altogether, these results elucidate additional mitochondria-related functions for p53 and implicate mtDNA depletion and ROS alterations as potentially relevant to cellular transformation, cancer cell phenotypes, and the Warburg Effect...
- Silencing of PINK1 expression affects mitochondrial DNA and oxidative phosphorylation in dopaminergic cellsMatthew E Gegg
Department of Clinical Neurosciences, Institute of Neurology, University College London, Queen Square, London, United Kingdom
PLoS ONE 4:e4756. 2009..The PINK1 protein is a serine/threonine kinase localized in mitochondria and the cytosol. Its precise function is unknown, but it is involved in neuroprotection against a variety of stress signalling pathways...
- Extension of chronological life span by reduced TOR signaling requires down-regulation of Sch9p and involves increased mitochondrial OXPHOS complex densityYong Pan
Department of Pathology, Yale University School of Medicine, New Haven CT 06520, USA
Aging (Albany NY) 1:131-45. 2009..by increasing mitochondrial oxygen consumption, in part, by up-regulating translation of mtDNA-encoded oxidative phosphorylation (OXPHOS) subunits...
- NOA1 is an essential GTPase required for mitochondrial protein synthesisMateusz Kolanczyk
Development and Disease Group, Max Planck Institute for Molecular Genetics, 14195 Berlin, Germany
Mol Biol Cell 22:1-11. 2011..from NOA1 knockout embryos show deficient mitochondrial protein synthesis and a global defect of oxidative phosphorylation (OXPHOS). Additionally, Noa1⁻/⁻ cells are impaired in staurosporine-induced apoptosis...
- Mitochondrial bioenergetic background confers a survival advantage to HepG2 cells in response to chemotherapyDominique Loiseau
INSERM U694, University Hospital Center, Angers, France
Mol Carcinog 48:733-41. 2009..should depend on cellular capability to produce sufficient ATP by upregulating glycolysis or recruiting oxidative phosphorylation (OXPHOS)...
- MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosomeYolanda Camara
Department of Laboratory Medicine, Karolinska Institutet, Stockholm, Sweden
Cell Metab 13:527-39. 2011Precise control of mitochondrial DNA gene expression is critical for regulation of oxidative phosphorylation capacity in mammals...
- Phylogenomics of the oxidative phosphorylation in fungi reveals extensive gene duplication followed by functional divergenceMarina Marcet-Houben
Comparative Genomics, Centre for Genomics Regulation, 08003 Barcelona, Spain
BMC Evol Biol 9:295. 2009b>Oxidative phosphorylation is central to the energy metabolism of the cell. Due to adaptation to different life-styles and environments, fungal species have shaped their respiratory pathways in the course of evolution...
- P/O ratios of mitochondrial oxidative phosphorylationPeter C Hinkle
Department of Molecular Biology and Genetics, Cornell University, Ithaca, NY 14853, USA
Biochim Biophys Acta 1706:1-11. 2005..Stock, A.G.W. Leslie, J.E. Walker, Science 286 (1999) 1700-1705) which suggests that the H+/ATP ratio is 10/3, rather than 3, consistent with P/O ratios of 2.3 with NADH and 1.4 with succinate, values that are also possible...
- Respiration in adipocytes is inhibited by reactive oxygen speciesTong Wang
Obesity Research Center, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA
Obesity (Silver Spring) 18:1493-502. 2010..Maximum O(2) consumption, in vitro, was determined with a chemical uncoupler of oxidative phosphorylation (carbonylcyanide p-trifluoromethoxyphenylhydrazone (FCCP))...
- The striatum is highly susceptible to mitochondrial oxidative phosphorylation dysfunctionsAlicia M Pickrell
Neuroscience Graduate Program, University of Miami Miller School of Medicine, Miami, Florida 33133, USA
J Neurosci 31:9895-904. 2011Neuronal oxidative phosphorylation (OXPHOS) deficiency has been associated with a variety of neurodegenerative diseases, including Parkinson's disease and Huntington's disease...
- Control of oxidative phosphorylation by vitamin A illuminates a fundamental role in mitochondrial energy homoeostasisRebeca Acín-Pérez
Department of Neurology and Neurobiology Weill Cornell Medical School, New York, New York, USA
FASEB J 24:627-36. 2010..The data provide a mechanistic explanation to the nearly 100-yr-old question of why vitamin A deficiency causes so many pathologies that are independent of retinoic acid action...
- Transcriptional profiles in CD8+ T cells from HIV+ progressors on HAART are characterized by coordinated up-regulation of oxidative phosphorylation enzymes and interferon responsesJing Qin Wu
Retroviral Genetics Division, Center for Virus Research, Westmead Millennium Institute, University of Sydney, Westmead, NSW, Australia
Virology 380:124-35. 2008..By GSEA, the coordinated up-regulation of oxidative phosphorylation enzymes and interferon responses were detected as fingerprints in HIV progressors on HAART, whereas LTNP ..
- Association of 3-methylglutaconic aciduria with sensori-neural deafness, encephalopathy, and Leigh-like syndrome (MEGDEL association) in four patients with a disorder of the oxidative phosphorylationS Wortmann
Radboud University Nijmegen Medical Centre, Nijmegen Centre for Mitochondrial Disorders, Department of Pediatrics, The Netherlands
Mol Genet Metab 88:47-52. 2006..3-methylglutaconyl-CoA hydratase activity, cardiolipin levels, OPA3 gene analysis and measurement of the oxidative phosphorylation in four female patients with 3-methylglutaconic aciduria...
- Genetic variation in ATP5O is associated with skeletal muscle ATP50 mRNA expression and glucose uptake in young twinsTina Rönn
Department of Clinical Sciences, Diabetes and Endocrinology Research Unit, CRC Malmö University Hospital, Lund University, Malmo, Sweden
PLoS ONE 4:e4793. 2009..Furthermore, mRNA expression of genes involved in oxidative phosphorylation, including ATP5O, is reduced in skeletal muscle from T2D patients...
- SIRT1 Overexpression in Cellular Mitochondrial Metabolism and FunctionJames Russell; Fiscal Year: 2009..Several lines of evidence indicate that a common link in these diseases is diminished mitochondrial oxidative phosphorylation and response to oxidative injury...
- OPTIC NEUROPATHY FROM MITOCHONDRIAL DERANGEMENTAlfredo Sadun; Fiscal Year: 1999..In each case, there is evidence that singular or combined insults to mitochondrial oxidative phosphorylation impair ATP production, however, this does not explain why other metabolically active tissues such as ..
- STRUCTURE AND FUNCTION OF THE TRANSFERRIN RECEPTORCAROLINE ENNS; Fiscal Year: 2002..for the enzymatic activity of key proteins in the DNA synthetic pathway, electron transport in the oxidative phosphorylation pathway in mitochondria, the activity of enzymes in the citric acid cycle, and the activity of proteins ..
- Modulation of Mitochondrial Function by Pro-OxidantsLUKE IGNATIUS SZWEDA; Fiscal Year: 2010..of cardiac energy status and function, mitochondria exhibit declines in the rate of respiration and oxidative phosphorylation during ischemia, with further age-dependent deficits evident upon reperfusion...
- ELECTRON-TRANSFER PROTEINSFRANCIS S contact MILLETT; Fiscal Year: 2010..reactions play essential roles in numerous biological processes important to human health, including oxidative phosphorylation in mitochondria...
- PROTON TRANSLOCATING SECTOR OF E COLI H+ ATPASEROBERT FILLINGAME; Fiscal Year: 2003A reversible, H+-transporting ATPase catalyzes ATP synthesis during oxidative phosphorylation. The enzyme is made up of two sectors. F1 lies at the membrane surface, and on isolation catalyzes ATP hydrolysis...
- Molecular Adaptation of Hypoxia in Oxygen Sensing CellsDavid Millhorn; Fiscal Year: 2007Mammalian cells require a constant supply of oxygen to maintain energy balance. Hypoxia leads to reduced oxidative phosphorylation and depletion of cellular ATP, which can result in cell death...
- DIAPHRAGM ADAPTATIONS TO CHRONIC RESISTIVE LOADINGDavid Prezant; Fiscal Year: 2001..ATP turnover, determined largely by myosin heavy chain (MHC) isoform composition, and b) mitochondrial oxidative phosphorylation capacity. This grant focuses on how these 2 factors are altered as the diaphragm adapts to CRRL...
- OXIDATIVE PHOSPHORYLATION IN CARDIAC HYPERTROPHYTERRENCE O BRIEN; Fiscal Year: 2000..is an up regulation of several components of the nuclear and mitochondrially derived mitochondrial oxidative phosphorylation system at both the mRNA and protein level...
- Mitochondrial dysfunction in HAART: Point of care testsMichael F Marusich; Fiscal Year: 2010..Mitochondrial Oxidative Phosphorylation (OXPHOS) protein levels are good candidates for early biomarkers of HAART toxicity as OXPHOS normally ..
- Mouse Modeling of Leigh Disease and Complex I AssemblyCarl Pinkert; Fiscal Year: 2007unreadable] DESCRIPTION (provided by applicant): Mitochondria provide energy for cells via oxidative phosphorylation (OXPHOS)...
- Proton-Translocating Sector of E. coli H+ATP SynthaseRobert H Fillingame; Fiscal Year: 2010The H+-transporting F1Fo ATP synthases of oxidative phosphorylation in mitochondria and bacteria are very similar...