OXPHOS mutations and neurodegeneration

[1]  F. Fontanesi,et al.  Redox and reactive oxygen species regulation of mitochondrial cytochrome C oxidase biogenesis. , 2013, Antioxidants & redox signaling.

[2]  P. Berger,et al.  Adaptative capacity of mitochondrial biogenesis and of mitochondrial dynamics in response to pathogenic respiratory chain dysfunction. , 2013, Antioxidants & redox signaling.

[3]  S. Strack,et al.  Cell signaling and mitochondrial dynamics: Implications for neuronal function and neurodegenerative disease , 2013, Neurobiology of Disease.

[4]  Jolein Gloerich,et al.  BOLA1 is an aerobic protein that prevents mitochondrial morphology changes induced by glutathione depletion. , 2013, Antioxidants & redox signaling.

[5]  L. Scorrano,et al.  Mitochondrial morphology in mitophagy and macroautophagy. , 2013, Biochimica et biophysica acta.

[6]  Xinnan Wang,et al.  The meaning of mitochondrial movement to a neuron's life. , 2013, Biochimica et biophysica acta.

[7]  R. Rodenburg,et al.  Trolox-sensitive reactive oxygen species regulate mitochondrial morphology, oxidative phosphorylation and cytosolic calcium handling in healthy cells. , 2012, Antioxidants & redox signaling.

[8]  F. Fontanesi,et al.  Mitochondrial complex I plays an essential role in human respirasome assembly , 2012 .

[9]  R. Rodenburg,et al.  Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts. , 2012, Biochimica et biophysica acta.

[10]  B. Westermann.  Bioenergetic role of mitochondrial fusion and fission. , 2012, Biochimica et biophysica acta.

[11]  H. McBride,et al.  The intracellular redox state is a core determinant of mitochondrial fusion , 2012, EMBO reports.

[12]  Jochen Buck,et al.  Metabolic Communication between Astrocytes and Neurons via Bicarbonate-Responsive Soluble Adenylyl Cyclase , 2012, Neuron.

[13]  D. Attwell,et al.  Synaptic Energy Use and Supply , 2012, Neuron.

[14]  J. Enríquez,et al.  NDUFA4 is a subunit of complex IV of the mammalian electron transport chain. , 2012, Cell metabolism.

[15]  M. Harper,et al.  Mitochondrial proticity and ROS signaling: lessons from the uncoupling proteins , 2012, Trends in Endocrinology & Metabolism.

[16]  R. Scarpulla.  Nucleus-encoded regulators of mitochondrial function: integration of respiratory chain expression, nutrient sensing and metabolic stress. , 2012, Biochimica et biophysica acta.

[17]  E. Koc,et al.  Regulation of mammalian mitochondrial translation by post-translational modifications. , 2012, Biochimica et biophysica acta.

[18]  P. Bénit,et al.  Therapies in inborn errors of oxidative metabolism , 2012, Trends in Endocrinology & Metabolism.

[19]  C. Moraes,et al.  Mitochondrial transcription: lessons from mouse models. , 2012, Biochimica et biophysica acta.

[20]  R. Palmiter,et al.  Altered Anesthetic Sensitivity of Mice Lacking Ndufs4, a Subunit of Mitochondrial Complex I , 2012, PloS one.

[21]  J. Arenas,et al.  Mitochondrial respiratory chain dysfunction: implications in neurodegeneration. , 2012, Free radical biology & medicine.

[22]  I. Novak,et al.  Mitophagy: a complex mechanism of mitochondrial removal. , 2012, Antioxidants & redox signaling.

[23]  G. Dienel,et al.  Brain Lactate Metabolism: The Discoveries and the Controversies , 2012, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[24]  K. Hejzlarová,et al.  Compensatory upregulation of respiratory chain complexes III and IV in isolated deficiency of ATP synthase due to TMEM70 mutation. , 2012, Biochimica et biophysica acta.

[25]  S. Kmoch,et al.  Adaptation of respiratory chain biogenesis to cytochrome c oxidase deficiency caused by SURF1 gene mutations. , 2012, Biochimica et biophysica acta.

[26]  Amanda R. Burnham-Marusich,et al.  Multiple proteins with essential mitochondrial functions have glycosylated isoforms. , 2012, Mitochondrion.

[27]  R. Swerdlow.  Mitochondria and cell bioenergetics: increasingly recognized components and a possible etiologic cause of Alzheimer's disease. , 2012, Antioxidants & redox signaling.

[28]  R. Palmiter,et al.  Mitochondrion-derived reactive oxygen species lead to enhanced amyloid beta formation. , 2012, Antioxidants & redox signaling.

[29]  A. Orr,et al.  Mitochondrial Complex II Can Generate Reactive Oxygen Species at High Rates in Both the Forward and Reverse Reactions* , 2012, The Journal of Biological Chemistry.

[30]  Joseph Loscalzo,et al.  Redox regulation of mitochondrial function. , 2012, Antioxidants & redox signaling.

[31]  D. Turnbull,et al.  Mitochondrial changes within axons in multiple sclerosis: an update. , 2012, Current opinion in neurology.

[32]  D. Winge,et al.  Reprint of: Biogenesis of the cytochrome bc(1) complex and role of assembly factors. , 2012, Biochimica et biophysica acta.

[33]  F. Court,et al.  Mitochondria as a central sensor for axonal degenerative stimuli , 2012, Trends in Neurosciences.

[34]  Brian O'Rourke,et al.  Glutathione/thioredoxin systems modulate mitochondrial H2O2 emission: An experimental-computational study , 2012, The Journal of general physiology.

[35]  D. Winge.  Sealing the Mitochondrial Respirasome , 2012, Molecular and Cellular Biology.

[36]  P. Reddy,et al.  Mitochondrial division ensures the survival of postmitotic neurons by suppressing oxidative damage , 2012, The Journal of cell biology.

[37]  P. Ray,et al.  Reactive oxygen species (ROS) homeostasis and redox regulation in cellular signaling. , 2012, Cellular signalling.

[38]  R. Skoumal,et al.  Physiological regulation of cardiac contractility by endogenous reactive oxygen species , 2012, Acta physiologica.

[39]  C. Rocher,et al.  Preservation of NADH ubiquinone-oxidoreductase activity by Src kinase-mediated phosphorylation of NDUFB10. , 2012, Biochimica et biophysica acta.

[40]  D. S. St. Clair,et al.  Manganese superoxide dismutase, MnSOD and its mimics. , 2012, Biochimica et biophysica acta.

[41]  M. Ankarcrona,et al.  Strategic role for mitochondria in Alzheimer's disease and cancer. , 2012, Antioxidants & redox signaling.

[42]  M. Krams,et al.  Impaired mitochondrial function in psychiatric disorders , 2012, Nature Reviews Neuroscience.

[43]  L. Scorrano,et al.  Shaping the role of mitochondria in the pathogenesis of Huntington's disease , 2012, The EMBO journal.

[44]  M. Tarnopolsky,et al.  The psychiatric manifestations of mitochondrial disorders: a case and review of the literature. , 2012, The Journal of clinical psychiatry.

[45]  R. Balaban,et al.  Role of mitochondrial Ca2+ in the regulation of cellular energetics. , 2012, Biochemistry.

[46]  M. Hüttemann,et al.  Multiple phosphorylations of cytochrome c oxidase and their functions , 2012, Proteomics.

[47]  C. Hiser,et al.  Gating and regulation of the cytochrome c oxidase proton pump. , 2012, Biochimica et biophysica acta.

[48]  J. Walker,et al.  Mitochondrial nucleoid interacting proteins support mitochondrial protein synthesis , 2012, Nucleic acids research.

[49]  Werner J H Koopman,et al.  Monogenic mitochondrial disorders. , 2012, The New England journal of medicine.

[50]  E. Rugarli,et al.  Mitochondrial quality control: a matter of life and death for neurons , 2012, The EMBO journal.

[51]  J. Nunnari,et al.  Mitochondria: In Sickness and in Health , 2012, Cell.

[52]  Antonio Gnoni,et al.  Respiratory chain complex I, a main regulatory target of the cAMP/PKA pathway is defective in different human diseases , 2012, FEBS letters.

[53]  E. Shoubridge.  Supersizing the mitochondrial respiratory chain. , 2012, Cell metabolism.

[54]  S. Gygi,et al.  Identification of a protein mediating respiratory supercomplex stability. , 2012, Cell metabolism.

[55]  M. Hellmich,et al.  Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly , 2012, The EMBO journal.

[56]  S. Jakobs,et al.  Rcf1 mediates cytochrome oxidase assembly and respirasome formation, revealing heterogeneity of the enzyme complex. , 2012, Cell metabolism.

[57]  R. von Bernhardi,et al.  Alzheimer's disease: redox dysregulation as a common denominator for diverse pathogenic mechanisms. , 2012, Antioxidants & redox signaling.

[58]  N. Pfanner,et al.  Mitochondrial protein import: from transport pathways to an integrated network. , 2012, Trends in biochemical sciences.

[59]  M. Cambron,et al.  White-Matter Astrocytes, Axonal Energy Metabolism, and Axonal Degeneration in Multiple Sclerosis , 2012, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[60]  M. Chakravarty,et al.  Brain Energy Metabolism and Blood Flow Differences in Healthy Aging , 2012, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[61]  Y. Harada,et al.  Intracellular temperature mapping with a fluorescent polymeric thermometer and fluorescence lifetime imaging microscopy , 2012, Nature Communications.

[62]  H. M. Cochemé,et al.  Mitochondrial redox signalling at a glance , 2012, Journal of Cell Science.

[63]  R. Stuart,et al.  Rcf1 and Rcf2, Members of the Hypoxia-Induced Gene 1 Protein Family, Are Critical Components of the Mitochondrial Cytochrome bc1-Cytochrome c Oxidase Supercomplex , 2012, Molecular and Cellular Biology.

[64]  M. Murphy,et al.  Mitochondrial thiols in antioxidant protection and redox signaling: distinct roles for glutathionylation and other thiol modifications. , 2012, Antioxidants & redox signaling.

[65]  M. Mattson,et al.  Recruiting adaptive cellular stress responses for successful brain ageing , 2012, Nature Reviews Neuroscience.

[66]  O. Kann,et al.  The Energy Demand of Fast Neuronal Network Oscillations: Insights from Brain Slice Preparations , 2012, Front. Pharmacol..

[67]  Jianhua Zhang,et al.  Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling , 2011, The Biochemical journal.

[68]  Quan Chen,et al.  Caspase cleavage of cytochrome c1 disrupts mitochondrial function and enhances cytochrome c release , 2011, Cell Research.

[69]  V. Borutaite,et al.  There is no evidence that mitochondria are the main source of reactive oxygen species in mammalian cells. , 2012, Mitochondrion.

[70]  J. Smeitink,et al.  Cellular Consequences of mtDNA-Encoded Mutations in NADH:Ubiquinone Oxidoreductase , 2012 .

[71]  Xiongwei Zhu,et al.  Mitochondrial importance in Alzheimer's, Huntington's and Parkinson's diseases. , 2012, Advances in experimental medicine and biology.

[72]  J. Smeitink,et al.  Pharmacological targeting of mitochondrial complex I deficiency: the cellular level and beyond. , 2012, Mitochondrion.

[73]  G. Castello,et al.  Oxidative stress and mitochondrial dysfunction in Down syndrome. , 2012, Advances in experimental medicine and biology.

[74]  C. Koehler,et al.  The complexity of cardiolipin in health and disease. , 2012, Trends in biochemical sciences.

[75]  S. Arnold.  The power of life--cytochrome c oxidase takes center stage in metabolic control, cell signalling and survival. , 2012, Mitochondrion.

[76]  Rutger O. Vogel,et al.  Assembly factors as a new class of disease genes for mitochondrial complex I deficiency: cause, pathology and treatment options. , 2012, Brain : a journal of neurology.

[77]  Sung-jin Kim,et al.  Reactive oxygen/nitrogen species and their functional correlations in neurodegenerative diseases , 2012, Journal of Neural Transmission.

[78]  Pierre J Magistretti,et al.  Brain energy metabolism: focus on astrocyte-neuron metabolic cooperation. , 2011, Cell metabolism.

[79]  G. Doherty.  Nitric oxide in neurodegeneration: potential benefits of non-steroidal anti-inflammatories , 2011, Neuroscience Bulletin.

[80]  J. Fransen,et al.  Defective mitochondrial translation differently affects the live cell dynamics of complex I subunits. , 2011, Biochimica et biophysica acta.

[81]  N. Fineberg,et al.  Differentiation of SH-SY5Y cells to a neuronal phenotype changes cellular bioenergetics and the response to oxidative stress. , 2011, Free radical biology & medicine.

[82]  M. Gustafsson,et al.  Super-resolution 3D microscopy of live whole cells using structured illumination , 2011, Nature Methods.

[83]  L. Martin.  Mitochondrial pathobiology in ALS , 2011, Journal of bioenergetics and biomembranes.

[84]  M. Huynen,et al.  Restoration of complex V deficiency caused by a novel deletion in the human TMEM70 gene normalizes mitochondrial morphology. , 2011, Mitochondrion.

[85]  C. Sue,et al.  The genetics of mitochondrial disease. , 2011, Seminars in neurology.

[86]  B. Korzeniewski.  Computer-aided studies on the regulation of oxidative phosphorylation during work transitions. , 2011, Progress in biophysics and molecular biology.

[87]  G. Shadel,et al.  Revisiting the TCA cycle: signaling to tumor formation. , 2011, Trends in molecular medicine.

[88]  M. Whelan,et al.  Autofluorescence microscopy: a non-destructive tool to monitor mitochondrial toxicity. , 2011, Toxicology letters.

[89]  J. V. van Dongen,et al.  The Composition of Plant Mitochondrial Supercomplexes Changes with Oxygen Availability* , 2011, The Journal of Biological Chemistry.

[90]  Harald F Hess,et al.  Superresolution Fluorescence Imaging of Mitochondrial Nucleoids Reveals Their Spatial Range, Limits, and Membrane Interaction , 2011, Molecular and Cellular Biology.

[91]  M. Zeviani,et al.  Epilepsy in Mitochondrial Disorders , 2011 .

[92]  M. V. Vander Heiden,et al.  Aerobic glycolysis: meeting the metabolic requirements of cell proliferation. , 2011, Annual review of cell and developmental biology.

[93]  E. Shoubridge,et al.  Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. , 2011, American journal of human genetics.

[94]  Brian O'Rourke,et al.  Redox Regulation of Mitochondrial ATP Synthase: Implications for Cardiac Resynchronization Therapy , 2011, Circulation Research.

[95]  W. Kühlbrandt,et al.  Arrangement of electron transport chain components in bovine mitochondrial supercomplex I1III2IV1 , 2011, The EMBO journal.

[96]  S. Girardin,et al.  Mitochondria in innate immunity , 2011, EMBO reports.

[97]  H. Stahlberg,et al.  Interaction of complexes I, III, and IV within the bovine respirasome by single particle cryoelectron tomography , 2011, Proceedings of the National Academy of Sciences.

[98]  R. Rodenburg,et al.  Mitochondrial ATP synthase: architecture, function and pathology , 2011, Journal of Inherited Metabolic Disease.

[99]  R. Efremov,et al.  Structure of the membrane domain of respiratory complex I , 2011, Nature.

[100]  W. Kühlbrandt,et al.  Macromolecular organization of ATP synthase and complex I in whole mitochondria , 2011, Proceedings of the National Academy of Sciences.

[101]  P. Bénit,et al.  Developmental aspects of respiratory chain from fetus to infancy. , 2011, Seminars in fetal & neonatal medicine.

[102]  R. Efremov,et al.  Respiratory complex I: 'steam engine' of the cell? , 2011, Current opinion in structural biology.

[103]  Thomas Wild,et al.  Machine Learning Improves the Precision and Robustness of High-Content Screens , 2011, Journal of biomolecular screening.

[104]  J. Hirst.  Why does mitochondrial complex I have so many subunits? , 2011, The Biochemical journal.

[105]  M. Radermacher,et al.  A scaffold of accessory subunits links the peripheral arm and the distal proton-pumping module of mitochondrial complex I. , 2011, The Biochemical journal.

[106]  Nektarios Tavernarakis,et al.  Cellular stress response pathways and ageing: intricate molecular relationships , 2011, The EMBO journal.

[107]  M. Brand,et al.  The Mechanism of Superoxide Production by the Antimycin-inhibited Mitochondrial Q-cycle* , 2011, The Journal of Biological Chemistry.

[108]  E. Schon,et al.  Mitochondria: The Next (Neurode)Generation , 2011, Neuron.

[109]  J. Smeitink,et al.  Quantitative Glucose and ATP Sensing in Mammalian Cells , 2011, Pharmaceutical Research.

[110]  X. Xu,et al.  Iron-sulfur clusters: biogenesis, molecular mechanisms, and their functional significance. , 2011, Antioxidants & redox signaling.

[111]  M. Brand,et al.  Evidence for Two Sites of Superoxide Production by Mitochondrial NADH-Ubiquinone Oxidoreductase (Complex I)* , 2011, The Journal of Biological Chemistry.

[112]  Keshav K. Singh,et al.  Genetic insights into OXPHOS defect and its role in cancer. , 2011, Biochimica et biophysica acta.

[113]  Hiroyuki Noji,et al.  Rotation and structure of FoF1-ATP synthase. , 2011, Journal of biochemistry.

[114]  Ajit S. Divakaruni,et al.  The regulation and physiology of mitochondrial proton leak. , 2011, Physiology.

[115]  Roland Brock,et al.  Solute diffusion is hindered in the mitochondrial matrix , 2011, Proceedings of the National Academy of Sciences.

[116]  Ashim Malhotra,et al.  Cardiolipin affects the supramolecular organization of ATP synthase in mitochondria. , 2011, Biophysical journal.

[117]  C. Konradi,et al.  Mitochondrial dysfunction and pathology in bipolar disorder and schizophrenia , 2011, International Journal of Developmental Neuroscience.

[118]  C. Mammucari,et al.  Molecules and roles of mitochondrial calcium signaling. , 2011, BioFactors.

[119]  M. Schuelke,et al.  Region-Specific Expression of Mitochondrial Complex I Genes during Murine Brain Development , 2011, PloS one.

[120]  Linda Partridge,et al.  Unraveling the biological roles of reactive oxygen species. , 2011, Cell metabolism.

[121]  Enrico Gratton,et al.  Lessons in fluctuation correlation spectroscopy. , 2011, Annual review of physical chemistry.

[122]  J. Hirst,et al.  Superoxide Is Produced by the Reduced Flavin in Mitochondrial Complex I , 2011, The Journal of Biological Chemistry.

[123]  Amy E Palmer,et al.  Design and application of genetically encoded biosensors. , 2011, Trends in biotechnology.

[124]  R. Touyz,et al.  NOX isoforms and reactive oxygen species in vascular health. , 2011, Molecular interventions.

[125]  Kun-Liang Guan,et al.  Regulation of intermediary metabolism by protein acetylation. , 2011, Trends in biochemical sciences.

[126]  T. Mak,et al.  Regulation of cancer cell metabolism , 2011, Nature Reviews Cancer.

[127]  Jason M. Held,et al.  Quantitative Mapping of Reversible Mitochondrial Complex I Cysteine Oxidation in a Parkinson Disease Mouse Model* , 2011, The Journal of Biological Chemistry.

[128]  J. Andersen,et al.  Nitrosylation and nitration of mitochondrial complex I in Parkinson's disease , 2011, Free radical research.

[129]  C. Ackerley,et al.  Complex V TMEM70 deficiency results in mitochondrial nucleoid disorganization. , 2011, Mitochondrion.

[130]  Masasuke Yoshida,et al.  Mechanical modulation of catalytic power on F1-ATPase. , 2011, Nature chemical biology.

[131]  Guy C. Brown,et al.  Nitric oxide and neuronal death. , 2010, Nitric oxide : biology and chemistry.

[132]  A. Stuchebrukhov,et al.  Electron tunneling in respiratory complex I , 2010, Proceedings of the National Academy of Sciences.

[133]  A. Munnich,et al.  A common pattern of brain MRI imaging in mitochondrial diseases with complex I deficiency , 2010, Journal of Medical Genetics.

[134]  F. Jiménez-Jiménez,et al.  Oxidative stress in skin fibroblasts cultures from patients with Parkinson's disease , 2010, BMC neurology.

[135]  Srinivas C. Turaga,et al.  Machines that learn to segment images: a crucial technology for connectomics , 2010, Current Opinion in Neurobiology.

[136]  A. Leslie,et al.  Bioenergetic cost of making an adenosine triphosphate molecule in animal mitochondria , 2010, Proceedings of the National Academy of Sciences.

[137]  Martin D. Brand,et al.  The sites and topology of mitochondrial superoxide production , 2010, Experimental Gerontology.

[138]  N. Dencher,et al.  Ageing alters the supramolecular architecture of OxPhos complexes in rat brain cortex , 2010, Experimental Gerontology.

[139]  A. Godzik,et al.  S-nitrosylation of Drp1 links excessive mitochondrial fission to neuronal injury in neurodegeneration. , 2010, Mitochondrion.

[140]  A. Schapira.  Complex I: Inhibitors, inhibition and neurodegeneration , 2010, Experimental Neurology.

[141]  Robert W. Taylor,et al.  A neurological perspective on mitochondrial disease , 2010, The Lancet Neurology.

[142]  C. Hunte,et al.  Functional Modules and Structural Basis of Conformational Coupling in Mitochondrial Complex I , 2010, Science.

[143]  M. Ryan,et al.  Assembly factors of human mitochondrial complex I and their defects in disease , 2010, IUBMB life.

[144]  T. Matsuishi,et al.  MELAS and l‐arginine therapy: pathophysiology of stroke‐like episodes , 2010, Annals of the New York Academy of Sciences.

[145]  Hans-Peter Braun,et al.  Structure and function of mitochondrial supercomplexes. , 2010, Biochimica et biophysica acta.

[146]  F. Striggow,et al.  The regulation of OXPHOS by extramitochondrial calcium. , 2010, Biochimica et biophysica acta.

[147]  S. Friedman,et al.  The use of neuroimaging in the diagnosis of mitochondrial disease. , 2010, Developmental disabilities research reviews.

[148]  J. Smeitink,et al.  Detection and manipulation of mitochondrial reactive oxygen species in mammalian cells. , 2010, Biochimica et biophysica acta.

[149]  L. Kadaja,et al.  Structure-function relationships in feedback regulation of energy fluxes in vivo in health and disease: mitochondrial interactosome. , 2010, Biochimica et biophysica acta.

[150]  R. Llinás,et al.  Calcium clearance and its energy requirements in cerebellar neurons. , 2010, Cell calcium.

[151]  B. Cohen.  Pharmacologic effects on mitochondrial function. , 2010, Developmental disabilities research reviews.

[152]  D. Winge,et al.  Succinate dehydrogenase - Assembly, regulation and role in human disease. , 2010, Mitochondrion.

[153]  Aabid Shariff,et al.  Automated Image Analysis for High-Content Screening and Analysis , 2010, Journal of biomolecular screening.

[154]  Cindy E J Dieteren,et al.  Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation. , 2010, Antioxidants & redox signaling.

[155]  P. Bénit,et al.  Genetic background influences mitochondrial function: modeling mitochondrial disease for therapeutic development. , 2010, Trends in molecular medicine.

[156]  T. Ohnishi.  Structural biology: Piston drives a proton pump , 2010, Nature.

[157]  Rouslan G. Efremov,et al.  The architecture of respiratory complex I , 2010, Nature.

[158]  J. Enríquez,et al.  Five Entry Points of the Mitochondrially Encoded Subunits in Mammalian Complex I Assembly , 2010, Molecular and Cellular Biology.

[159]  S. Moncada,et al.  Nitric oxide, cytochrome C oxidase, and the cellular response to hypoxia. , 2010, Arteriosclerosis, thrombosis, and vascular biology.

[160]  J. Finsterer,et al.  Drugs interfering with mitochondrial disorders , 2010, Drug and chemical toxicology.

[161]  Eun Bo Shim,et al.  Mitochondrial dysfunction and metabolic syndrome-looking for environmental factors. , 2010, Biochimica et biophysica acta.

[162]  M. Whittington,et al.  Mitochondrial DNA mutations affect calcium handling in differentiated neurons , 2010, Brain : a journal of neurology.

[163]  J. Altschmied,et al.  Nuclear redox signaling. , 2010, Antioxidants & redox signaling.

[164]  C. Conrad,et al.  Automated microscopy for high-content RNAi screening , 2010, The Journal of cell biology.

[165]  D. Turnbull,et al.  Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations , 2010, Brain : a journal of neurology.

[166]  Bryan C Dickinson,et al.  Mitochondrial-targeted fluorescent probes for reactive oxygen species. , 2010, Current opinion in chemical biology.

[167]  J. N. Spelbrink.  Functional organization of mammalian mitochondrial DNA in nucleoids: History, recent developments, and future challenges , 2009, IUBMB life.

[168]  F. Sakai,et al.  Pathophysiology of stroke-like episodes in MELAS: neuron–astrocyte uncoupling in neuronal hyperexcitability , 2010 .

[169]  Todd A. Clason,et al.  The structure of eukaryotic and prokaryotic complex I. , 2010, Journal of structural biology.

[170]  C. Chinopoulos,et al.  Mitochondria as ATP consumers in cellular pathology. , 2010, Biochimica et biophysica acta.

[171]  Thomas D. Fox,et al.  Inventory control: cytochrome c oxidase assembly regulates mitochondrial translation , 2010, Nature Reviews Molecular Cell Biology.

[172]  V. Mootha,et al.  The mitochondrial proteome and human disease. , 2010, Annual review of genomics and human genetics.

[173]  S. Dimauro,et al.  A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases. , 2009, Biochimica et biophysica acta.

[174]  Anne E Carpenter,et al.  Introduction to the Quantitative Analysis of Two-Dimensional Fluorescence Microscopy Images for Cell-Based Screening , 2009, PLoS Comput. Biol..

[175]  Günther Deuschl,et al.  Essential tremor — Neurodegenerative or nondegenerative disease towards a working definition of ET , 2009, Movement disorders : official journal of the Movement Disorder Society.

[176]  D. Butterfield,et al.  Nitric oxide in cell survival: a janus molecule. , 2009, Antioxidants & redox signaling.

[177]  J. Smeitink,et al.  Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency. , 2009, The international journal of biochemistry & cell biology.

[178]  P. Magistretti,et al.  The role of astroglia in neuroprotection , 2009, Dialogues in clinical neuroscience.

[179]  M. Whittington,et al.  Transmitochondrial embryonic stem cells containing pathogenic mtDNA mutations are compromised in neuronal differentiation , 2009, Cell proliferation.

[180]  H. Schägger,et al.  Supramolecular organization of ATP synthase and respiratory chain in mitochondrial membranes. , 2009, Biochimica et biophysica acta.

[181]  L. A. Kane,et al.  Post-translational modifications of ATP synthase in the heart: biology and function , 2009, Journal of bioenergetics and biomembranes.

[182]  N. Dencher,et al.  Respiratory complexes III and IV are not essential for the assembly/stability of complex I in fungi. , 2009, Journal of molecular biology.

[183]  P. Morgan,et al.  Complex I Function Is Defective in Complex IV-deficient Caenorhabditis elegans* , 2009, Journal of Biological Chemistry.

[184]  Xianlin Han,et al.  Lack of Cytochrome c in Mouse Fibroblasts Disrupts Assembly/Stability of Respiratory Complexes I and IV* , 2009, Journal of Biological Chemistry.

[185]  Michael P. Murphy,et al.  How mitochondria produce reactive oxygen species , 2008, The Biochemical journal.

[186]  R. Rodenburg,et al.  Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease. , 2008, Brain : a journal of neurology.

[187]  Rutger O. Vogel,et al.  Subunits of Mitochondrial Complex I Exist as Part of Matrix- and Membrane-associated Subcomplexes in Living Cells* , 2008, Journal of Biological Chemistry.

[188]  Werner J H Koopman,et al.  Computer-assisted live cell analysis of mitochondrial membrane potential, morphology and calcium handling. , 2008, Methods.

[189]  A. Starkov.  The Role of Mitochondria in Reactive Oxygen Species Metabolism and Signaling , 2008, Annals of the New York Academy of Sciences.

[190]  Carmen A Mannella,et al.  Structural Diversity of Mitochondria , 2008, Annals of the New York Academy of Sciences.

[191]  Antonio Feliciello,et al.  Control of mitochondria dynamics and oxidative metabolism by cAMP, AKAPs and the proteasome. , 2008, Trends in cell biology.

[192]  J. Enríquez,et al.  Respiratory active mitochondrial supercomplexes. , 2008, Molecular cell.

[193]  R. Wanders,et al.  Cardiolipin provides an essential activating platform for caspase-8 on mitochondria , 2008, The Journal of cell biology.

[194]  J. Andersen,et al.  Redox imbalance in Parkinson's disease. , 2008, Biochimica et biophysica acta.

[195]  J. Zweier,et al.  Protein Tyrosine Nitration of the Flavin Subunit Is Associated with Oxidative Modification of Mitochondrial Complex II in the Post-ischemic Myocardium* , 2008, Journal of Biological Chemistry.

[196]  M. Klingenberg.  The ADP and ATP transport in mitochondria and its carrier. , 2008, Biochimica et biophysica acta.

[197]  P. Kaufmann,et al.  Mitochondrial Encephalopathy, Lactic Acidosis, and Strokelike Episodes , 2008, Annals of the New York Academy of Sciences.

[198]  T. Hurd,et al.  Complex I within Oxidatively Stressed Bovine Heart Mitochondria Is Glutathionylated on Cys-531 and Cys-704 of the 75-kDa Subunit , 2008, Journal of Biological Chemistry.

[199]  U. Brandt,et al.  The Mechanism of Mitochondrial Superoxide Production by the Cytochrome bc1 Complex* , 2008, Journal of Biological Chemistry.

[200]  S. Carr,et al.  A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology , 2008, Cell.

[201]  J. Smeitink,et al.  Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency. , 2008, Cell calcium.

[202]  K. Wallace.  Mitochondrial off targets of drug therapy. , 2008, Trends in pharmacological sciences.

[203]  Ferdinando Palmieri,et al.  Diseases caused by defects of mitochondrial carriers: a review. , 2008, Biochimica et biophysica acta.

[204]  G. Perkins,et al.  Mitochondrial fragmentation in neurodegeneration , 2008, Nature Reviews Neuroscience.

[205]  R. Pathak,et al.  Complex I and energy thresholds in the brain. , 2008, Biochimica et biophysica acta.

[206]  S. Dimauro,et al.  Mitochondrial disorders in the nervous system. , 2008, Annual review of neuroscience.

[207]  G. Lenaz,et al.  Generation of Reactive Oxygen Species by Mitochondrial Complex I: Implications in Neurodegeneration , 2008, Neurochemical Research.

[208]  R. Rossignol,et al.  Ultrastructure of the mitochondrion and its bearing on function and bioenergetics. , 2008, Antioxidants & redox signaling.

[209]  J. Lieberman,et al.  Granzyme A Cleaves a Mitochondrial Complex I Protein to Initiate Caspase-Independent Cell Death , 2008, Cell.

[210]  H. Fukui,et al.  The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis? , 2008, Trends in Neurosciences.

[211]  W. Watt,et al.  Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy. , 2008, Cell metabolism.

[212]  R. Scarpulla.  Transcriptional paradigms in mammalian mitochondrial biogenesis and function. , 2008, Physiological reviews.

[213]  O Orwar,et al.  Controlling the rates of biochemical reactions and signaling networks by shape and volume changes , 2008, Proceedings of the National Academy of Sciences.

[214]  F. Sterky,et al.  Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration , 2008, Human molecular genetics.

[215]  Seon-Yong Jeong,et al.  The role of mitochondria in apoptosis. , 2008, BMB reports.

[216]  K. Smolková,et al.  Functional dynamic compartmentalization of respiratory chain intermediate substrates: implications for the control of energy production and mitochondrial diseases. , 2008, The international journal of biochemistry & cell biology.

[217]  N. Yadava,et al.  Investigations of the potential effects of phosphorylation of the MWFE and ESSS subunits on complex I activity and assembly. , 2008, The international journal of biochemistry & cell biology.

[218]  S. Moncada,et al.  S-Nitrosation of Mitochondrial Complex I Depends on Its Structural Conformation* , 2007, Journal of Biological Chemistry.

[219]  A. Starkov,et al.  The mitochondrial respiratory chain is a modulator of apoptosis , 2007, The Journal of cell biology.

[220]  J. Smeitink,et al.  Mitochondrial and cytosolic thiol redox state are not detectably altered in isolated human NADH:ubiquinone oxidoreductase deficiency. , 2007, Biochimica et biophysica acta.

[221]  J. Smeitink,et al.  Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology? , 2007, American journal of physiology. Cell physiology.

[222]  S. Vannucci,et al.  Supply and Demand in Cerebral Energy Metabolism: The Role of Nutrient Transporters , 2007, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[223]  Yvonne Will,et al.  Circumventing the Crabtree effect: replacing media glucose with galactose increases susceptibility of HepG2 cells to mitochondrial toxicants. , 2007, Toxicological sciences : an official journal of the Society of Toxicology.

[224]  W. Dröge,et al.  Oxidative stress and aberrant signaling in aging and cognitive decline , 2007, Aging cell.

[225]  M. L. Genova,et al.  Kinetics of integrated electron transfer in the mitochondrial respiratory chain: random collisions vs. solid state electron channeling. , 2007, American journal of physiology. Cell physiology.

[226]  Rutger O. Vogel,et al.  Identification of Mitochondrial Complex I Assembly Intermediates by Tracing Tagged NDUFS3 Demonstrates the Entry Point of Mitochondrial Subunits* , 2007, Journal of Biological Chemistry.

[227]  J. Smeitink,et al.  Superoxide production is inversely related to complex I activity in inherited complex I deficiency. , 2007, Biochimica et biophysica acta.

[228]  Massimo Zeviani,et al.  Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice. , 2007, Human molecular genetics.

[229]  Liang Peng,et al.  Energy Metabolism in Astrocytes: High Rate of Oxidative Metabolism and Spatiotemporal Dependence on Glycolysis/Glycogenolysis , 2007, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[230]  L. Szweda,et al.  Inhibition of complex I by Ca2+ reduces electron transport activity and the rate of superoxide anion production in cardiac submitochondrial particles. , 2007, Biochemistry.

[231]  Hans-Peter Braun,et al.  Supramolecular Structure of the Mitochondrial Oxidative Phosphorylation System* , 2007, Journal of Biological Chemistry.

[232]  A. Kaasik,et al.  Regulation of mitochondrial matrix volume. , 2007, American journal of physiology. Cell physiology.

[233]  C. Chinopoulos,et al.  Bioenergetics and the formation of mitochondrial reactive oxygen species. , 2006, Trends in pharmacological sciences.

[234]  A. Galinier,et al.  Physiological diversity of mitochondrial oxidative phosphorylation. , 2006, American journal of physiology. Cell physiology.

[235]  J. Finsterer.  Central nervous system manifestations of mitochondrial disorders , 2006, Acta neurologica Scandinavica.

[236]  M. Lazarou,et al.  Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients. , 2006, Journal of molecular biology.

[237]  B. Spiegelman,et al.  Peroxisome proliferator-activated receptor gamma coactivator 1 coactivators, energy homeostasis, and metabolism. , 2006, Endocrine reviews.

[238]  Philip Hinchliffe,et al.  Structure of the Hydrophilic Domain of Respiratory Complex I from Thermus thermophilus , 2006, Science.

[239]  C. Sevier,et al.  Generating disulfides enzymatically: reaction products and electron acceptors of the endoplasmic reticulum thiol oxidase Ero1p. , 2006, Proceedings of the National Academy of Sciences of the United States of America.

[240]  J. Dixon,et al.  Mitochondrial modulation: reversible phosphorylation takes center stage? , 2006, Trends in biochemical sciences.

[241]  A. Szewczyk,et al.  [Mitochondrial ion channels]. , 2002, Postepy higieny i medycyny doswiadczalnej.

[242]  A. Boneh,et al.  Regulation of mitochondrial oxidative phosphorylation by second messenger-mediated signal transduction mechanisms , 2006, Cellular and Molecular Life Sciences CMLS.

[243]  L. Tretter,et al.  Alpha-ketoglutarate dehydrogenase: a target and generator of oxidative stress , 2005, Philosophical Transactions of the Royal Society B: Biological Sciences.

[244]  T. Horvath,et al.  Mitochondrial uncoupling proteins in the cns: in support of function and survival , 2005, Nature Reviews Neuroscience.

[245]  J. Smeitink,et al.  Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency. , 2005, American journal of physiology. Cell physiology.

[246]  P. Bénit,et al.  Succinate dehydrogenase deficiency in human , 2005, Cellular and Molecular Life Sciences CMLS.

[247]  N. Newman.  Hereditary optic neuropathies: from the mitochondria to the optic nerve. , 2005, American journal of ophthalmology.

[248]  J. Meldolesi,et al.  Astrocytes, from brain glue to communication elements: the revolution continues , 2005, Nature Reviews Neuroscience.

[249]  L. Sazanov,et al.  Organization of Iron-Sulfur Clusters in Respiratory Complex I , 2005, Science.

[250]  S. Minucci,et al.  Electron Transfer between Cytochrome c and p66Shc Generates Reactive Oxygen Species that Trigger Mitochondrial Apoptosis , 2005, Cell.

[251]  D. Butterfield,et al.  Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia , 2005, Journal of the Neurological Sciences.

[252]  Robert S. Balaban,et al.  Mitochondria, Oxidants, and Aging , 2005, Cell.

[253]  S. Flanagan,et al.  Mitochondrial O2*- and H2O2 mediate glucose deprivation-induced stress in human cancer cells. , 2005, The Journal of biological chemistry.

[254]  T. Zimmermann.  Spectral imaging and linear unmixing in light microscopy. , 2005, Advances in biochemical engineering/biotechnology.

[255]  F. Muller,et al.  Complex III Releases Superoxide to Both Sides of the Inner Mitochondrial Membrane* , 2004, Journal of Biological Chemistry.

[256]  M. Bauer,et al.  Significance of Respirasomes for the Assembly/Stability of Human Respiratory Chain Complex I* , 2004, Journal of Biological Chemistry.

[257]  Mark H Ellisman,et al.  Disruption of Mitochondrial Function during Apoptosis Is Mediated by Caspase Cleavage of the p75 Subunit of Complex I of the Electron Transport Chain , 2004, Cell.

[258]  C. Bruno,et al.  Respiratory complex III is required to maintain complex I in mammalian mitochondria. , 2004, Molecular cell.

[259]  J. Rabek,et al.  Oxidatively damaged proteins of heart mitochondrial electron transport complexes. , 2004, Biochimica et biophysica acta.

[260]  Rodrigue Rossignol,et al.  Energy Substrate Modulates Mitochondrial Structure and Oxidative Capacity in Cancer Cells , 2004, Cancer Research.

[261]  P. Rich.  The molecular machinery of Keilin's respiratory chain. , 2003, Biochemical Society transactions.

[262]  Don P Giddens,et al.  Role of xanthine oxidoreductase and NAD(P)H oxidase in endothelial superoxide production in response to oscillatory shear stress. , 2003, American journal of physiology. Heart and circulatory physiology.

[263]  Marjan S. Bolouri,et al.  Integrated Analysis of Protein Composition, Tissue Diversity, and Gene Regulation in Mouse Mitochondria , 2003, Cell.

[264]  Keith D Garlid,et al.  Mitochondrial potassium transport: the K(+) cycle. , 2003, Biochimica et biophysica acta.

[265]  Steven W. Taylor,et al.  Oxidative Damage to Mitochondrial Complex I Due to Peroxynitrite , 2003, Journal of Biological Chemistry.

[266]  C. Bruno,et al.  Leigh Syndrome with COX deficiency and SURF1 gene mutations: MR imaging findings. , 2003, AJNR. American journal of neuroradiology.

[267]  W. Hauswirth,et al.  Suppression of complex I gene expression induces optic neuropathy , 2003, Annals of neurology.

[268]  M. Vila,et al.  Neurodegeneration: what is it and where are we? , 2003, The Journal of clinical investigation.

[269]  G. Paradies,et al.  Reactive oxygen species affect mitochondrial electron transport complex I activity through oxidative cardiolipin damage. , 2002, Gene.

[270]  K. Pfeiffer,et al.  The ratio of oxidative phosphorylation complexes I-V in bovine heart mitochondria and the composition of respiratory chain supercomplexes. , 2001, The Journal of biological chemistry.

[271]  S. Dimauro,et al.  The genetics and pathology of oxidative phosphorylation , 2001, Nature Reviews Genetics.

[272]  B. Fanburg,et al.  Reactive oxygen species in cell signaling. , 2000, American journal of physiology. Lung cellular and molecular physiology.

[273]  E. Kandel,et al.  Essentials of Neural Science and Behavior , 1996 .

[274]  A. Azzi,et al.  High rates of extracellular superoxide generation by cultured human fibroblasts: involvement of a lipid-metabolizing enzyme. , 1996, The Biochemical journal.

[275]  S. Roy,et al.  Superoxide generation by lipoxygenase in the presence of NADH and NADPH. , 1994, Biochimica et biophysica acta.

[276]  A. Davison,et al.  Mitochondrial mutations may increase oxidative stress: implications for carcinogenesis and aging? , 1990, Free radical biology & medicine.

[277]  M. King,et al.  Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation. , 1989, Science.

[278]  S. Dimauro,et al.  Mitochondrial diseases. , 1989, Neurologic clinics.

[279]  D. Wallace,et al.  Mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy. , 1988, Science.

[280]  Michael P. King,et al.  Injection of mitochondria into human cells leads to a rapid replacement of the endogenous mitochondrial DNA , 1988, Cell.

[281]  M. Hess,et al.  PGH Synthase and Lipoxygenase Generate Superoxide in the Presence of NADH or NADPH , 1986, Circulation research.

[282]  S. Dimauro,et al.  Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndrome , 1984, Annals of neurology.

[283]  B Chance,et al.  The cellular production of hydrogen peroxide. , 1972, The Biochemical journal.

[284]  C. Hackenbrock,et al.  OXIDATIVE PHOSPHORYLATION AND ULTRASTRUCTURAL TRANSFORMATION IN MITOCHONDRIA IN THE INTACT ASCITES TUMOR CELL , 1971, The Journal of cell biology.

[285]  D. Leigh.  SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY IN AN INFANT , 1951, Journal of neurology, neurosurgery, and psychiatry.