mitochondrial proteins

Summary

Summary: Proteins encoded by the mitochondrial genome or proteins encoded by the nuclear genome that are imported to and resident in the MITOCHONDRIA.

Top Publications

  1. pmc A mitochondrial protein compendium elucidates complex I disease biology
    David J Pagliarini
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Cell 134:112-23. 2008
  2. ncbi Functional brown adipose tissue in healthy adults
    Kirsi A Virtanen
    Turku PET Center, University of Turku, Finland
    N Engl J Med 360:1518-25. 2009
  3. pmc Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin
    Atsushi Tanaka
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 191:1367-80. 2010
  4. ncbi Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1
    Z Wu
    Dana Farber Cancer Institute, and Department of Cell Biology, Harvard Medical School, Boston, Massachusetts 02115, USA
    Cell 98:115-24. 1999
  5. pmc Fission and selective fusion govern mitochondrial segregation and elimination by autophagy
    Gilad Twig
    Department of Pharmacology and Experimental Therapeutics, Tufts University School of Medicine, Boston, MA 02111, USA
    EMBO J 27:433-46. 2008
  6. pmc PINK1 stabilized by mitochondrial depolarization recruits Parkin to damaged mitochondria and activates latent Parkin for mitophagy
    Noriyuki Matsuda
    Laboratory of Frontier Science, Tokyo Metropolitan Institute of Medical Science, Setagaya Ku, Tokyo 156 8506, Japan
    J Cell Biol 189:211-21. 2010
  7. ncbi Mitofusin 2 tethers endoplasmic reticulum to mitochondria
    Olga Martins de Brito
    Dulbecco Telethon Institute, Venetian Institute of Molecular Medicine, Via Orus 2, 35129 Padova, Italy
    Nature 456:605-10. 2008
  8. ncbi Mitochondrial dysfunction and type 2 diabetes
    Bradford 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
  9. pmc Chronic peroxisome proliferator-activated receptor gamma (PPARgamma) activation of epididymally derived white adipocyte cultures reveals a population of thermogenically competent, UCP1-containing adipocytes molecularly distinct from classic brown adipocyt
    Natasa Petrovic
    Wenner Gren Institute, The Arrhenius Laboratories, Stockholm University, SE 106 91 Stockholm, Sweden
    J Biol Chem 285:7153-64. 2010
  10. pmc Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development
    Hsiuchen Chen
    Division of Biology, Beckman Institute, California Institute of Technology, Pasadena, CA 91125, USA
    J Cell Biol 160:189-200. 2003

Research Grants

  1. THE ROLE OF ACETYLATION IN MITOCHONDRIAL FUNCTION
    Gary Cecchini; Fiscal Year: 2013
  2. Functional Genomics of the Mitochondrial Proteome
    Dennis R Winge; Fiscal Year: 2011
  3. Mitochondrial Dysfunction and Adipose Insulin Resistance
    David A Bernlohr; Fiscal Year: 2013
  4. Regulation of epithelial cell homeostasis by actin microfilaments
    Seema Khurana; Fiscal Year: 2010
  5. Protein Nitration in the Placenta
    Leslie Myatt; Fiscal Year: 2012
  6. Iron in the pathogenesis of Friedreich's ataxia
    ARNULF HANS WERNER KOEPPEN; Fiscal Year: 2013
  7. Genetic Studies of Optic Atrophy
    Taosheng Huang; Fiscal Year: 2012
  8. Mitochondrial Dysfunction and Adipose Insulin Resistance
    DAVID BERNLOHR; Fiscal Year: 2009
  9. Impaired mitochondrial fusion in pulmonary arterial hypertension
    Stephen L Archer; Fiscal Year: 2010
  10. Protein alterations in skeletal muscle cells with improved mtDNA repair
    Lyudmila I Rachek; Fiscal Year: 2010

Detail Information

Publications305 found, 100 shown here

  1. pmc A mitochondrial protein compendium elucidates complex I disease biology
    David J Pagliarini
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Cell 134:112-23. 2008
    ..Our results have important implications for understanding CI function and pathogenesis and, more generally, illustrate how our compendium can serve as a foundation for systematic investigations of mitochondria...
  2. ncbi Functional brown adipose tissue in healthy adults
    Kirsi A Virtanen
    Turku PET Center, University of Turku, Finland
    N Engl J Med 360:1518-25. 2009
    ....
  3. pmc Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin
    Atsushi Tanaka
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 191:1367-80. 2010
    ..Inhibition of Drp1-mediated mitochondrial fission, the proteasome, or p97 prevents Parkin-induced mitophagy...
  4. ncbi Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1
    Z Wu
    Dana Farber Cancer Institute, and Department of Cell Biology, Harvard Medical School, Boston, Massachusetts 02115, USA
    Cell 98:115-24. 1999
    ..These data elucidate a pathway that directly links external physiological stimuli to the regulation of mitochondrial biogenesis and function...
  5. pmc Fission and selective fusion govern mitochondrial segregation and elimination by autophagy
    Gilad Twig
    Department of Pharmacology and Experimental Therapeutics, Tufts University School of Medicine, Boston, MA 02111, USA
    EMBO J 27:433-46. 2008
    ..DRP1(K38A) or FIS1 RNAi decreased mitochondrial autophagy and resulted in the accumulation of oxidized mitochondrial proteins, reduced respiration and impaired insulin secretion...
  6. pmc PINK1 stabilized by mitochondrial depolarization recruits Parkin to damaged mitochondria and activates latent Parkin for mitophagy
    Noriyuki Matsuda
    Laboratory of Frontier Science, Tokyo Metropolitan Institute of Medical Science, Setagaya Ku, Tokyo 156 8506, Japan
    J Cell Biol 189:211-21. 2010
    ..Some pathogenic mutations of PINK1 and Parkin interfere with the aforementioned events, suggesting an etiological importance. These results provide crucial insight into the pathogenic mechanisms of PD...
  7. ncbi Mitofusin 2 tethers endoplasmic reticulum to mitochondria
    Olga Martins de Brito
    Dulbecco Telethon Institute, Venetian Institute of Molecular Medicine, Via Orus 2, 35129 Padova, Italy
    Nature 456:605-10. 2008
    ..Thus, mitofusin 2 tethers ER to mitochondria, a juxtaposition required for efficient mitochondrial Ca(2+) uptake...
  8. ncbi Mitochondrial dysfunction and type 2 diabetes
    Bradford 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...
  9. pmc Chronic peroxisome proliferator-activated receptor gamma (PPARgamma) activation of epididymally derived white adipocyte cultures reveals a population of thermogenically competent, UCP1-containing adipocytes molecularly distinct from classic brown adipocyt
    Natasa Petrovic
    Wenner Gren Institute, The Arrhenius Laboratories, Stockholm University, SE 106 91 Stockholm, Sweden
    J Biol Chem 285:7153-64. 2010
    ....
  10. pmc Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development
    Hsiuchen Chen
    Division of Biology, Beckman Institute, California Institute of Technology, Pasadena, CA 91125, USA
    J Cell Biol 160:189-200. 2003
    ..Therefore, mitochondrial fusion is essential for embryonic development, and by enabling cooperation between mitochondria, has protective effects on the mitochondrial population...
  11. pmc Dynamin-related protein Drp1 is required for mitochondrial division in mammalian cells
    E Smirnova
    Department of Biological Chemistry, UCLA School of Medicine, Los Angeles, California 90095 1737, USA
    Mol Biol Cell 12:2245-56. 2001
    ..We conclude that Drp1 contributes to mitochondrial division in mammalian cells...
  12. pmc Mitochondrial nucleoid interacting proteins support mitochondrial protein synthesis
    J He
    MRC Mitochondrial Biology Unit, Wellcome Trust MRC Building, Hills Road Cambridge, CB2 0XY, UK
    Nucleic Acids Res 40:6109-21. 2012
    ..ATAD3 and prohibitin are tightly associated with the mitochondrial membranes and so we propose that they support nucleic acid complexes at the inner membrane of the mitochondrion...
  13. pmc ER tubules mark sites of mitochondrial division
    Jonathan R Friedman
    Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, CO 80309, USA
    Science 334:358-62. 2011
    ..Thus, ER tubules may play an active role in defining the position of mitochondrial division sites...
  14. pmc Importing mitochondrial proteins: machineries and mechanisms
    Agnieszka Chacinska
    Institut fur Biochemie und Molekularbiologie, ZBMZ, Universitat Freiburg, 79104 Freiburg, Germany
    Cell 138:628-44. 2009
    Most mitochondrial proteins are synthesized on cytosolic ribosomes and must be imported across one or both mitochondrial membranes...
  15. ncbi Smac, a mitochondrial protein that promotes cytochrome c-dependent caspase activation by eliminating IAP inhibition
    C Du
    Howard Hughes Medical Institute and Department of Biochemistry, University of Texas Southwestern Medical Center at Dallas, 75235, USA
    Cell 102:33-42. 2000
    ..Overexpression of Smac increases cells' sensitivity to apoptotic stimuli. Smac is the second mitochondrial protein, along with cytochrome c, that promotes apoptosis by activating caspases...
  16. ncbi The role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis
    S Frank
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Behesda, Maryland 20892, USA
    Dev Cell 1:515-25. 2001
    ..Remarkably, inhibition of Drp1 blocks cell death, implicating mitochondrial fission as an important step in apoptosis...
  17. pmc Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy
    Matthew E Gegg
    Department of Clinical Neurosciences, Institute of Neurology, University College London, London, UK
    Hum Mol Genet 19:4861-70. 2010
    ..The ubiquitination of several mitochondrial proteins, including mitofusin 1 and mitofusin 2, were detected within 3 h of CCCP treatment...
  18. pmc Transcriptional control of brown fat determination by PRDM16
    Patrick Seale
    Dana Farber Cancer Institute and the Department of Cell Biology, Harvard Medical School, 1 Jimmy Fund Way, Boston, MA 02115, USA
    Cell Metab 6:38-54. 2007
    ..PRDM16 activates brown fat cell identity at least in part by simultaneously activating PGC-1alpha and PGC-1beta through direct protein binding. These data indicate that PRDM16 can control the determination of brown fat fate...
  19. pmc Mff is an essential factor for mitochondrial recruitment of Drp1 during mitochondrial fission in mammalian cells
    Hidenori Otera
    Department of Molecular Biology, Graduate School of Medical Science, Kyushu University, Fukuoka 812 8582, Japan
    J Cell Biol 191:1141-58. 2010
    ..Thus, Mff functions as an essential factor in mitochondrial recruitment of Drp1...
  20. pmc Conformational changes in Dnm1 support a contractile mechanism for mitochondrial fission
    Jason A Mears
    Laboratory of Cell Biochemistry and Biology, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
    Nat Struct Mol Biol 18:20-6. 2011
    ..Our structural studies support the idea that Dnm1 has a mechanochemical role during mitochondrial division...
  21. pmc UCP2 mediates ghrelin's action on NPY/AgRP neurons by lowering free radicals
    Zane B Andrews
    Section of Comparative Medicine, Department of Obstetrics, Gynecology and Reproductive Sciences, Howard Hughes Medical Institute, New York, New York 10021, USA
    Nature 454:846-51. 2008
    ..These results reveal a signalling modality connecting mitochondria-mediated effects of G-protein-coupled receptors on neuronal function and associated behaviour...
  22. pmc Selective degradation of mitochondria by mitophagy
    Insil Kim
    Center for Cell Death, Injury and Regeneration, Departments of Pharmaceutical Sciences and Biochemistry and Molecular Biology, Medical University of South Carolina, Charleston, SC 29425, USA
    Arch Biochem Biophys 462:245-53. 2007
    ..This review provides an overview of the process of mitophagy, the possible role of the mitochondrial permeability transition in mitophagy and the importance of mitophagy in turnover of dysfunctional mitochondria...
  23. ncbi p53 regulates mitochondrial respiration
    Satoaki Matoba
    Cardiology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Science 312:1650-3. 2006
    ..That SCO2 couples p53 to mitochondrial respiration provides a possible explanation for the Warburg effect and offers new clues as to how p53 might affect aging and metabolism...
  24. ncbi Mitochondria: more than just a powerhouse
    Heidi M McBride
    University of Ottawa Heart Institute, Canada
    Curr Biol 16:R551-60. 2006
    ..In this review we will highlight the emerging evidence that provides molecular definition to mitochondria as a central platform in the execution of diverse cellular events...
  25. pmc Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA
    Christian Kukat
    Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, D 50931 Cologne, Germany
    Proc Natl Acad Sci U S A 108:13534-9. 2011
    ..These fundamental insights into the organization of mtDNA have broad implications for understanding mitochondrial dysfunction in disease and aging...
  26. pmc Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL
    Seok Min Jin
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 191:933-42. 2010
    ..Thus, differential localization to the inner and outer mitochondrial membranes appears to regulate PINK1 stability and function...
  27. ncbi Mitochondrial dynamics in mammalian health and disease
    Marc Liesa
    Institute for Research in Biomedicine IRB Barcelona, CIBER de Diabetes y Enfermedades Metabólicas Asociadas, and Departament de Bioquímica i Biologia Molecular, Universitat de Barcelona, Barcelona 08028, Spain
    Physiol Rev 89:799-845. 2009
    ..Here we review the most significant findings in the field of mitochondrial dynamics in mammalian cells and their implication in human pathologies...
  28. ncbi Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice
    N G Larsson
    Department of Molecular Medicine, Center for Molecular Medicine L8 02, Stockholm, Sweden
    Nat Genet 18:231-6. 1998
    ..5. Thus, Tfam is the first mammalian protein demonstrated to regulate mtDNA copy number in vivo and is essential for mitochondrial biogenesis and embryonic development...
  29. ncbi Mitochondrial protein import: from proteomics to functional mechanisms
    Oliver Schmidt
    Institut fur Biochemie und Molekularbiologie, ZBMZ, Universitat Freiburg, Stefan Meier Strabetae 17, 79104 Freiburg, Germany
    Nat Rev Mol Cell Biol 11:655-67. 2010
    ..Protein translocases do not function as independent units but are integrated into dynamic networks and are connected to machineries that function in bioenergetics, mitochondrial morphology and coupling to the endoplasmic reticulum...
  30. pmc Mitochondrial dynamics and apoptosis
    Der Fen Suen
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, MD 20892, USA
    Genes Dev 22:1577-90. 2008
    ..This review will cover the recent advances and presents competing models on how the mitochondrial fission and fusion machinery may intersect apoptosis pathways...
  31. ncbi Mitochondrial DNA mutations in disease and aging
    Douglas C Wallace
    ORU for Molecular and Mitochondrial Medicine and Genetics, University of California, Irvine, CA, USA
    Environ Mol Mutagen 51:440-50. 2010
    ..This unique genetic system provides a flexible method for generating genetic variation in cellular and organismal energetics that permits species to adapt to alterations in their regional energetic environment...
  32. ncbi The mitochondrial calcium uniporter is a highly selective ion channel
    Yuriy Kirichok
    Howard Hughes Medical Institute, Department of Cardiovascular Research, Children s Hospital, andHarvard Medical School, 320 Longwood Avenue, Boston, Massachusetts 02115, USA
    Nature 427:360-4. 2004
    ..The channel is inwardly rectifying, making it especially effective for Ca2+ uptake into energized mitochondria. Thus, we conclude that the properties of the current mediated by this novel channel are those of the MCU...
  33. pmc The mechanism of Ca2+ -dependent regulation of kinesin-mediated mitochondrial motility
    Xinnan Wang
    F M Kirby Neurobiology Center, Children s Hospital Boston, Boston, MA 02115, USA
    Cell 136:163-74. 2009
    ..Disrupting Ca(2+)-dependent regulation diminishes neuronal resistance to excitotoxicity...
  34. pmc Dephosphorylation by calcineurin regulates translocation of Drp1 to mitochondria
    G M Cereghetti
    Dulbecco Telethon Institute, Padua, Italy
    Proc Natl Acad Sci U S A 105:15803-8. 2008
    ..Thus, fragmentation of depolarized mitochondria depends on a loop involving sustained Ca(2+) rise, activation of calcineurin, and dephosphorylation of Drp1 and its translocation to the organelle...
  35. ncbi Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice
    Naotada Ishihara
    Department of Physiology and Cell Biology, Tokyo Medical and Dental University, Tokyo 113 8519, Japan
    Nat Cell Biol 11:958-66. 2009
    ....
  36. pmc Mitochondrial fusion is required for mtDNA stability in skeletal muscle and tolerance of mtDNA mutations
    Hsiuchen Chen
    Division of Biology, California Institute of Technology, Pasadena, CA 91125, USA
    Cell 141:280-9. 2010
    ..With its dual function in safeguarding mtDNA integrity and preserving mtDNA function in the face of mutations, mitochondrial fusion is likely to be a protective factor in human disorders associated with mtDNA mutations...
  37. pmc A role for the mitochondrial deacetylase Sirt3 in regulating energy homeostasis
    Bong Hyun Ahn
    Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 105:14447-52. 2008
    ..These results implicate protein acetylation as an important regulator of Complex I activity and demonstrate that Sirt3 functions in vivo to regulate and maintain basal ATP levels...
  38. pmc MiD49 and MiD51, new components of the mitochondrial fission machinery
    Catherine S Palmer
    La Trobe Institute for Molecular Science, La Trobe University, Plenty Road, Melbourne 3086, Victoria, Australia
    EMBO Rep 12:565-73. 2011
    ..Overexpression of MiD49/51 seems to sequester Drp1 from functioning at mitochondria and cause fused tubules to associate with actin. Thus, MiD49/51 are new mediators of mitochondrial division affecting Drp1 action at mitochondria...
  39. ncbi UCP1 ablation induces obesity and abolishes diet-induced thermogenesis in mice exempt from thermal stress by living at thermoneutrality
    Helena M Feldmann
    The Wenner Gren Institute, The Arrhenius Laboratories F3, Stockholm University, Stockholm, Sweden
    Cell Metab 9:203-9. 2009
    ....
  40. pmc A mitochondrial-focused genetic interaction map reveals a scaffold-like complex required for inner membrane organization in mitochondria
    Suzanne Hoppins
    Department of Molecular and Cellular Biology, University of California, Davis, Davis, CA 95616, USA
    J Cell Biol 195:323-40. 2011
    ..a comprehensive view of mitochondrial function including insights into the activity of uncharacterized mitochondrial proteins and the functional connection between mitochondria and the ER...
  41. pmc The human mitochondrial transcriptome
    Tim R Mercer
    Institute for Molecular Bioscience, The University of Queensland, Brisbane QLD 4072, Australia
    Cell 146:645-58. 2011
    ..matticklab.com)...
  42. ncbi The presence of UCP1 demonstrates that metabolically active adipose tissue in the neck of adult humans truly represents brown adipose tissue
    Maria Cristina Zingaretti
    Department of Molecular Pathology and Innovative Therapies, Faculty of Medicine, Ospedali Riuniti Ancona, University of Ancona Politecnica delle Marche, Ancona, Italy
    FASEB J 23:3113-20. 2009
    ....
  43. ncbi Translocation of proteins into mitochondria
    Walter Neupert
    Institut fur Physiologische Chemie, Universitat Munchen, 81377 Munchen, Germany
    Annu Rev Biochem 76:723-49. 2007
    About 10% to 15% of the nuclear genes of eukaryotic organisms encode mitochondrial proteins. These proteins are synthesized in the cytosol and recognized by receptors on the surface of mitochondria...
  44. ncbi Identification of DIABLO, a mammalian protein that promotes apoptosis by binding to and antagonizing IAP proteins
    A M Verhagen
    The Walter and Eliza Hall Institute of Medical Research, Post Office Royal Melbourne Hospital, Victoria, Australia
    Cell 102:43-53. 2000
    ..As transfection of cells with DIABLO was able to counter the protection afforded by MIHA against UV irradiation, DIABLO may promote apoptosis by binding to IAPs and preventing them from inhibiting caspases...
  45. pmc The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster
    Ana Bratic
    Department of Laboratory Medicine, Karolinska Institutet, Solna, Sweden
    PLoS Genet 7:e1002324. 2011
    ..We thus report a novel function of BSF in flies and demonstrate that it has an important intra-mitochondrial role, which is essential for maintaining mtDNA gene expression and oxidative phosphorylation...
  46. pmc Fine-tuning of Drp1/Fis1 availability by AKAP121/Siah2 regulates mitochondrial adaptation to hypoxia
    Hyungsoo Kim
    Signal Transduction Program, Sanford Burnham Medical Research Institute, La Jolla, CA 92037, USA
    Mol Cell 44:532-44. 2011
    ..Through modulating Fis1/Drp1 complex availability, our studies identify Siah2 as a key regulator of hypoxia-induced mitochondrial fission and its physiological significance in ischemic injury and nematode life span...
  47. ncbi DNA replication and transcription in mammalian mitochondria
    Maria Falkenberg
    Division of Metabolic Diseases, Karolinska Institutet, Novum, SE 141 86 Stockholm
    Annu Rev Biochem 76:679-99. 2007
    ..Recent years have seen a rapid development in our molecular understanding of these machineries, but many aspects still remain unknown...
  48. pmc MICU1 encodes a mitochondrial EF hand protein required for Ca(2+) uptake
    Fabiana Perocchi
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114, USA
    Nature 467:291-6. 2010
    ..Its discovery may lead to the complete molecular characterization of mitochondrial calcium uptake pathways, and offers genetic strategies for understanding their contribution to normal physiology and disease...
  49. ncbi Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A
    Stephan Zuchner
    Department of Neuropathology, University Hospital, RWTH Aachen, Pauwelsstrasse 30, 52074 Aachen, Germany
    Nat Genet 36:449-51. 2004
  50. ncbi Substrates and regulation mechanisms for the human mitochondrial sirtuins Sirt3 and Sirt5
    Christine Schlicker
    Laboratory of Biochemistry, Department of Physiological Chemistry, Ruhr University Bochum, Universitaetsstrasse 150, 44801 Bochum, Germany
    J Mol Biol 382:790-801. 2008
    ....
  51. pmc Mammalian Sir2 homolog SIRT3 regulates global mitochondrial lysine acetylation
    David B Lombard
    Howard Hughes Medical Institute, The Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Mol Cell Biol 27:8807-14. 2007
    ..Overall, our results extend the recent finding of lysine acetylation of mitochondrial proteins and demonstrate that SIRT3 has evolved to control reversible lysine acetylation in this organelle.
  52. ncbi Human mitochondrial DNA deletions associated with mutations in the gene encoding Twinkle, a phage T7 gene 4-like protein localized in mitochondria
    J N Spelbrink
    Institute of Medical Technology and Tampere University Hospital, Tampere, Finland
    Nat Genet 28:223-31. 2001
    ..The mutations cluster in a region of the protein proposed to be involved in subunit interactions. The function of Twinkle is inferred to be critical for lifetime maintenance of human mtDNA integrity...
  53. pmc Reversible phosphorylation of Drp1 by cyclic AMP-dependent protein kinase and calcineurin regulates mitochondrial fission and cell death
    J Thomas Cribbs
    Department of Pharmacology, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242, USA
    EMBO Rep 8:939-44. 2007
    ..Thus, Drp1 phosphorylation at Ser 656 provides a mechanism for the integration of cAMP and calcium signals in the control of mitochondrial shape, apoptosis and other aspects of mitochondrial function...
  54. pmc Loss of PINK1 causes mitochondrial functional defects and increased sensitivity to oxidative stress
    Clement A Gautier
    Center for Neurologic Diseases, Brigham and Women s Hospital, Program in Neuroscience, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 105:11364-9. 2008
    ....
  55. ncbi Superoxide activates mitochondrial uncoupling proteins
    Karim S Echtay
    Medical Research Council Dunn Human Nutrition Unit, Hills Road, Cambridge CB2 2XY, UK
    Nature 415:96-9. 2002
    ..Our findings indicate that the interaction of superoxide with UCPs may be a mechanism for decreasing the concentrations of reactive oxygen species inside mitochondria...
  56. pmc Mitochondrial fragmentation in neurodegeneration
    Andrew B Knott
    Burnett School of Biomedical Sciences, College of Medicine, University of Central Florida, 4000 Central Florida Boulevard, Orlando, Florida 32816, USA
    Nat Rev Neurosci 9:505-18. 2008
    ..This results in impaired bioenergetics and mitochondrial migration, and can trigger neurodegeneration. These findings suggest potential new treatment avenues for neurodegenerative diseases...
  57. ncbi TNF-alpha induces two distinct caspase-8 activation pathways
    Lai Wang
    Howard Hughes Medical Institute and Department of Biochemistry, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390, USA
    Cell 133:693-703. 2008
    ..These findings revealed that TNF-alpha is able to induce apoptosis via two distinct caspase-8 activation pathways that are differentially regulated by cIAP1/2 and c-FLIP...
  58. ncbi A lethal defect of mitochondrial and peroxisomal fission
    Hans R Waterham
    Department of Pediatrics, Academic Medical Center, Amsterdam, The Netherlands
    N Engl J Med 356:1736-41. 2007
    ..Overexpression of the mutant DLP1 in control cells reproduced the fission defect. Our findings are representative of a class of disease characterized by defects in both mitochondria and peroxisomes...
  59. ncbi Recent advances into the understanding of mitochondrial fission
    Kirstin Elgass
    Department of Biochemistry, La Trobe Institute for Molecular Science, La Trobe University, Melbourne, Australia
    Biochim Biophys Acta 1833:150-61. 2013
    ..This article is part of a Special Issue entitled: Mitochondrial dynamics and physiology...
  60. pmc Mechanism of protein biosynthesis in mammalian mitochondria
    Brooke E Christian
    Department of Chemistry, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA
    Biochim Biophys Acta 1819:1035-54. 2012
    ..This article is part of a Special Issue entitled: Mitochondrial Gene Expression...
  61. pmc Atg32 is a mitochondrial protein that confers selectivity during mitophagy
    Tomotake Kanki
    Life Sciences Institute, University of Michigan, Ann Arbor, MI 48109, USA
    Dev Cell 17:98-109. 2009
    ..Therefore, Atg32 confers selectivity for mitochondrial sequestration as a cargo and is necessary for recruitment of this organelle by the autophagy machinery for mitophagy...
  62. ncbi The role of mitochondria in apoptosis*
    Chunxin Wang
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892, USA
    Annu Rev Genet 43:95-118. 2009
    ..We also compare and contrast apoptosis pathways in Caenorhabditis elegans, Drosophila melanogaster, and mammals that indicate major mysteries remaining to be solved...
  63. pmc Mammalian transcription factor A is a core component of the mitochondrial transcription machinery
    Yonghong Shi
    Department of Medical Biochemistry and Cell Biology, University of Gothenburg, Gothenburg, Sweden
    Proc Natl Acad Sci U S A 109:16510-5. 2012
    ..Instead, our findings support a model in which TFAM is absolutely required to recruit the transcription machinery during initiation of transcription...
  64. ncbi Energy substrate modulates mitochondrial structure and oxidative capacity in cancer cells
    Rodrigue Rossignol
    Institute of Molecular Biology, University of Oregon, Eugene, Oregon, USA
    Cancer Res 64:985-93. 2004
    ..We compared our finding on HeLa cells with those for nontransformed fibroblasts to help distinguish the regulatory pathways...
  65. ncbi Mitotic phosphorylation of dynamin-related GTPase Drp1 participates in mitochondrial fission
    Naoko Taguchi
    Department of Molecular Biology, Graduate School of Medical Science, Kyushu University, Fukuoka 812 8582, Japan
    J Biol Chem 282:11521-9. 2007
    ..Exogenous expression of unphosphorylated mutant Drp1S585A led to reduced mitotic mitochondrial fragmentation. These results suggest that phosphorylation of Drp1 on Ser-585 promotes mitochondrial fission in mitotic cells...
  66. ncbi Dynamics of mitochondrial morphology in healthy cells and during apoptosis
    M Karbowski
    The Biochemistry Section, SNB, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Cell Death Differ 10:870-80. 2003
    ..In this review the recent progress in understanding the mechanisms governing mitochondrial morphology and the latest advances connecting the regulation of mitochondrial morphology with programmed cell death are discussed...
  67. pmc ERAL1 is associated with mitochondrial ribosome and elimination of ERAL1 leads to mitochondrial dysfunction and growth retardation
    Takeshi Uchiumi
    Department of Clinical Chemistry and Laboratory Medicine, Kyushu University Graduate School of Medical Sciences, 3 1 1, Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Nucleic Acids Res 38:5554-68. 2010
    ..These results suggest that ERAL1 is localized in a small subunit of the mitochondrial ribosome, plays an important role in the small ribosomal constitution, and is also involved in cell viability...
  68. pmc Human MIEF1 recruits Drp1 to mitochondrial outer membranes and promotes mitochondrial fusion rather than fission
    Jian Zhao
    Department of Oncology Pathology, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden
    EMBO J 30:2762-78. 2011
    ..As MIEF1 is vertebrate-specific, these data also reveal important differences between yeast and vertebrates in the regulation of mitochondrial dynamics...
  69. pmc Human ERAL1 is a mitochondrial RNA chaperone involved in the assembly of the 28S small mitochondrial ribosomal subunit
    Sven Dennerlein
    Institute for Ageing and Health, Newcastle University, UK
    Biochem J 430:551-8. 2010
    ..Finally, whereas depletion of ERAL1 leads to apoptosis, cell death occurs prior to any appreciable loss of mitochondrial protein synthesis or reduction in the stability of mitochondrial mRNA...
  70. ncbi Physiological functions of the mitochondrial uncoupling proteins UCP2 and UCP3
    Martin D Brand
    MRC Dunn Human Nutrition Unit, Hills Road, Cambridge, United Kingdom
    Cell Metab 2:85-93. 2005
    ..Evidence that they export fatty acids physiologically is weak. UCP2 and UCP3 are important potential targets for treatment of aging, degenerative diseases, diabetes, and perhaps obesity...
  71. pmc Membrane remodeling induced by the dynamin-related protein Drp1 stimulates Bax oligomerization
    Sylvie Montessuit
    Department of Cell Biology, University of Geneva, Sciences III, 30 Quai Ernest Ansermet, 1211 Geneva 4, Switzerland
    Cell 142:889-901. 2010
    ..In cells, overexpression of Drp1 R247A/E delays Bax oligomerization and cell death. Our findings uncover a function of Drp1 and provide insight into the mechanism of Bax oligomerization...
  72. ncbi Mitochondrial bioenergetics and structural network organization
    Giovanni Benard
    Institut National de la Sante et de la Recherche Medicale INSERM, U688 Physiopathologie Mitochondriale, Universite Victor Segalen Bordeaux 2, 146 rue Leo Saignat, F 33076 Bordeaux cedex, France
    J Cell Sci 120:838-48. 2007
    ..Our data demonstrate that the relationship between mitochondrial network organization and bioenergetics is bidirectional, and we provide a model for analyzing the metabolic signals involved in this crosstalk...
  73. pmc A human dynamin-related protein controls the distribution of mitochondria
    E Smirnova
    Department of Biological Chemistry, UCLA School of Medicine, Los Angeles, California 90095 1737, USA
    J Cell Biol 143:351-8. 1998
    ..The function of this new dynamin-related protein in organelle morphology represents a novel role for a member of the dynamin family of proteins...
  74. pmc An ER-mitochondria tethering complex revealed by a synthetic biology screen
    Benoît Kornmann
    Department of Biochemistry and Biophysics, University of California at San Francisco, San Francisco, CA 94158, USA
    Science 325:477-81. 2009
    ..The tethering complex localized to discrete foci, suggesting that discrete sites of close apposition between ER and mitochondria facilitate interorganelle calcium and phospholipid exchange...
  75. ncbi The emergence of cold-induced brown adipocytes in mouse white fat depots is determined predominantly by white to brown adipocyte transdifferentiation
    G Barbatelli
    Department of Molecular Pathology and Innovative Therapies, Faculty of Medicine, University of Ancona Politecnica delle Marche, Ancona, Italy
    Am J Physiol Endocrinol Metab 298:E1244-53. 2010
    ..Overall, our data strongly suggest that the cold-induced emergence of brown adipocytes in WAT predominantly reflects beta(3)-adrenoceptor-mediated transdifferentiation...
  76. pmc Genome-wide RNAi screen identifies Letm1 as a mitochondrial Ca2+/H+ antiporter
    Dawei Jiang
    Department of Cardiology, Howard Hughes Medical Institute, Children s Hospital Boston, Manton Center for Orphan Disease, and Department of Neurobiology, Harvard Medical School, Enders Building 1309, 320 Longwood Avenue, Boston, MA 02115, USA
    Science 326:144-7. 2009
    ..RNAi knockdown, overexpression, and liposome reconstitution of the purified Letm1 protein demonstrate that Letm1 is a mitochondrial Ca2+/H+ antiporter...
  77. ncbi Uncoupling proteins in human heart
    Andrew J Murray
    University Laboratory of Physiology, University of Oxford, Oxford, UK
    Lancet 364:1786-8. 2004
    ..New treatment to correct these energy defects would be to simultaneously lower plasma free fatty acids and provide an alternative energy source...
  78. ncbi Mitofusin-2 determines mitochondrial network architecture and mitochondrial metabolism. A novel regulatory mechanism altered in obesity
    Daniel Bach
    Parc Cientific de Barcelona, Facultat de Biologia, Universitat de Barcelona, Barcelona 08028, Spain
    J Biol Chem 278:17190-7. 2003
    ....
  79. pmc Essential role for Nix in autophagic maturation of erythroid cells
    Hector Sandoval
    Department of Immunology, Baylor College of Medicine, Houston, Texas 77030, USA
    Nature 454:232-5. 2008
    ..Our study may also provide insights into molecular mechanisms underlying mitochondrial quality control involving mitochondrial autophagy...
  80. ncbi The layered structure of human mitochondrial DNA nucleoids
    Daniel F Bogenhagen
    Department of Pharmacological Sciences, State University of New York at Stony Brook, Stony Brook, New York 11794 8651, USA
    J Biol Chem 283:3665-75. 2008
    ..These results are discussed in relation to a model for a layered structure of mtDNA nucleoids in which replication and transcription occur in the central core, whereas translation and complex assembly may occur in the peripheral region...
  81. pmc Mitochondrial fusion in human cells is efficient, requires the inner membrane potential, and is mediated by mitofusins
    Frédéric Legros
    Institut National de la Santé et de la Recharche Médicale U523, Institut de Myologie, Groupe Hospitalier Pitie Salpetriere, 75651 Paris, France
    Mol Biol Cell 13:4343-54. 2002
    ..These results show that human mitochondria represent a single cellular compartment whose exchanges and interconnectivity are dynamically regulated by the balance between continuous fusion and fission reactions...
  82. ncbi Dynamic regulation of mitochondrial fission through modification of the dynamin-related protein Drp1
    Chuang Rung Chang
    Institute of Biotechnology and Department of Life Sciences, National Tsing Hua University, Hsinchu, Taiwan, ROC
    Ann N Y Acad Sci 1201:34-9. 2010
    ..We discuss the dynamic nature of Drp1 modifications and how they contribute not only to the normal regulation of mitochondrial division, but also to neuropathologic processes...
  83. ncbi Uncoupling proteins and the control of mitochondrial reactive oxygen species production
    Ryan J Mailloux
    Department of Biochemistry, Microbiology, and Immunology, University of Ottawa Faculty of Medicine, Ottawa, ON, Canada K1G8M5
    Free Radic Biol Med 51:1106-15. 2011
    ....
  84. pmc ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function
    Manjula Darshi
    Howard Hughes Medical Institute, University of California San Diego, La Jolla, California 92093 0654, USA
    J Biol Chem 286:2918-32. 2011
    ..of ChChd3 led to almost complete loss of both mitofilin and Sam50 proteins and alterations in several mitochondrial proteins, suggesting that ChChd3 is a scaffolding protein that stabilizes protein complexes involved in maintaining ..
  85. pmc When a theory of aging ages badly
    Jerome Lapointe
    Department of Biology, McGill University, Montreal H3A 1B1, Canada
    Cell Mol Life Sci 67:1-8. 2010
    ..Thus, we believe that it is reasonable to now consider the MFRTA as refuted and that it is time to use the insight gained by many years of testing this theory to develop new views as to the physiological causes of aging...
  86. ncbi Dual targeting of mitochondrial proteins: mechanism, regulation and function
    Ohad Yogev
    Department of Microbiology and Molecular Genetics, IMRIC, Faculty of Medicine, Hebrew University, Jerusalem 91120, Israel
    Biochim Biophys Acta 1808:1012-20. 2011
    ..In this review we ask how, when and why mitochondrial proteins are dual localized in the cell...
  87. pmc Parkin ubiquitinates Drp1 for proteasome-dependent degradation: implication of dysregulated mitochondrial dynamics in Parkinson disease
    Hongxia Wang
    Department of Genetics and Cell Biology, College of Life Sciences, Nankai University, Tianjin, China
    J Biol Chem 286:11649-58. 2011
    ..These results identify Drp1 as a novel substrate of Parkin and suggest a potential mechanism linking abnormal Parkin expression to mitochondrial dysfunction in the pathogenesis of Parkinson disease...
  88. pmc Reversible lysine acetylation controls the activity of the mitochondrial enzyme acetyl-CoA synthetase 2
    Bjoern Schwer
    Gladstone Institute of Virology and Immunology, University of California, San Francisco, CA 94158, USA
    Proc Natl Acad Sci U S A 103:10224-9. 2006
    ..Because the activity of a bacterial ortholog of AceCS2, called ACS, is controlled via deacetylation by a bacterial sirtuin protein, our observation highlights the conservation of a metabolic regulatory pathway from bacteria to humans...
  89. pmc Quality control of mitochondria: protection against neurodegeneration and ageing
    Takashi Tatsuta
    Institute for Genetics and Centre for Molecular Medicine, University of Cologne, Cologne, Germany
    EMBO J 27:306-14. 2008
    ..Here, we will summarize the current knowledge on these surveillance strategies and their role in human disease...
  90. pmc Role of BNIP3 and NIX in cell death, autophagy, and mitophagy
    J Zhang
    Department of Biochemistry, St Jude Children s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38117 3678, USA
    Cell Death Differ 16:939-46. 2009
    ..In this review, we will discuss possible mechanisms by which BNIP3 and NIX induce cell death and mitophagy. We will also consider the potential relationship between cell death pathways and autophagy in development and homeostasis...
  91. ncbi Mitochondrial dysfunction and oxidative damage in parkin-deficient mice
    James J Palacino
    Center for Neurologic Diseases, Brigham and Women s Hospital, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 279:18614-22. 2004
    ....
  92. ncbi Mitochondrial transcription factor A (TFAM): roles in maintenance of mtDNA and cellular functions
    Dongchon Kang
    Department of Clinical Chemistry and Laboratory Medicine, Kyushu University Graduate School of Medical Sciences, Fukuoka 812 8582, Japan
    Mitochondrion 7:39-44. 2007
    ..TFAM may play a crucial role in maintaining mitochondrial DNA as a main component of the nucleoid...
  93. ncbi Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease
    Karsten M Strauss
    Center of Neurology and Hertie Institute for Clinical Brain Research, Leicester, UK
    Hum Mol Genet 14:2099-111. 2005
    ..On the basis of functional genomics, our results provide a novel link between mitochondrial dysfunction and neurodegeneration in PD...
  94. ncbi SIRT3, a mitochondrial sirtuin deacetylase, regulates mitochondrial function and thermogenesis in brown adipocytes
    Tong Shi
    United States Department of Agriculture Agricultural Research Service Children s Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 280:13560-7. 2005
    ..In summary, our results demonstrate that SIRT3 activates mitochondria functions and plays an important role in adaptive thermogenesis in brown adipose...
  95. ncbi Mitochondrial uncoupling protein 2 (UCP2) in glucose and lipid metabolism
    Sabrina Diano
    Program in Integrative Cell Signaling and Neurobiology of Metabolism, Yale University School of Medicine, New Haven, CT 06520, USA
    Trends Mol Med 18:52-8. 2012
    ..The goal is to provide a framework for discussion of future therapeutic strategies for metabolism-related chronic diseases...
  96. pmc Uncoupling proteins 2 and 3 are fundamental for mitochondrial Ca2+ uniport
    Michael Trenker
    Institute of Molecular Biology and Biochemistry, Centre of Molecular Medicine, Medical University of Graz, Harrachgasse 21 III, 8010 Graz, Austria
    Nat Cell Biol 9:445-52. 2007
    ..Moreover, the identification of proteins fundemental for mitochondrial Ca(2+) uptake expands our knowledge of the physiological role for mitochondrial Ca(2+) sequestration...
  97. pmc Disrupted-in-schizophrenia 1 (DISC1) plays essential roles in mitochondria in collaboration with Mitofilin
    Young Un Park
    Division of Molecular and Life Science, Department of Life Science, Biotechnology Research Center, Pohang University of Science and Technology, Pohang 790 784, Korea
    Proc Natl Acad Sci U S A 107:17785-90. 2010
    ..According to these results, we propose that DISC1 plays essential roles for mitochondrial function in collaboration with a mitochondrial interacting partner, Mitofilin...
  98. ncbi Human mitochondrial transcription factor A induces a U-turn structure in the light strand promoter
    Anna Rubio-Cosials
    Department of Structural Biology, Molecular Biology Institute of Barcelona CSIC, Barcelona, Spain
    Nat Struct Mol Biol 18:1281-9. 2011
    ..Our data suggest that TFAM bends LSP to create an optimal DNA arrangement for transcriptional initiation while facilitating DNA compaction elsewhere in the genome...
  99. ncbi The changing shape of mitochondrial apoptosis
    Michał Wasilewski
    Dulbecco Telethon Institute, Venetian Institute of Molecular Medicine, 35129 Padova, Italy
    Trends Endocrinol Metab 20:287-94. 2009
    ..Here, we discuss the recent advancements in our knowledge about the machinery that regulates mitochondrial shape and on the role of molecular mechanisms controlling mitochondrial morphology during cell death...
  100. ncbi Oral administration of vitamin C decreases muscle mitochondrial biogenesis and hampers training-induced adaptations in endurance performance
    Mari Carmen Gomez-Cabrera
    Department of Physiology, Faculty of Medicine, University of Valencia, Valencia, Spain
    Am J Clin Nutr 87:142-9. 2008
    ..There is, however, considerable debate regarding the beneficial health effects of vitamin C supplementation...
  101. pmc Mitophagy in yeast occurs through a selective mechanism
    Tomotake Kanki
    Life Sciences Institute and Department of Molecular, Cellular, and Developmental Biology, University of Michigan, Ann Arbor, Michigan 48109 2216, USA
    J Biol Chem 283:32386-93. 2008
    ..autophagy (mitophagy) by fusing the green fluorescent protein at the C terminus of two endogenous mitochondrial proteins and monitored vacuolar release of green fluorescent protein...

Research Grants70

  1. THE ROLE OF ACETYLATION IN MITOCHONDRIAL FUNCTION
    Gary Cecchini; Fiscal Year: 2013
    ..suggested that acetylation affects both the activity of these complexes and their interaction with other mitochondrial proteins which affects overall mitochondrial efficiency and potentially contributes to ROS formation...
  2. Functional Genomics of the Mitochondrial Proteome
    Dennis R Winge; Fiscal Year: 2011
    ..Using bioinformatics tools, we have identified a subset of mitochondrial proteins of unknown function that are conserved from yeast to mammals...
  3. Mitochondrial Dysfunction and Adipose Insulin Resistance
    David A Bernlohr; Fiscal Year: 2013
    ..from genomic, proteomic, metabolomic and molecular analyses that point toward the covalent modification of mitochondrial proteins with bioactive lipids and the oxidation of mitochondrial thioredoxin as central to the process...
  4. Regulation of epithelial cell homeostasis by actin microfilaments
    Seema Khurana; Fiscal Year: 2010
    ....
  5. Protein Nitration in the Placenta
    Leslie Myatt; Fiscal Year: 2012
    ..Nitration of mitochondrial proteins is a reversible process dependent on oxygen concentration hence the hypoxic environment or ischemia/..
  6. Iron in the pathogenesis of Friedreich's ataxia
    ARNULF HANS WERNER KOEPPEN; Fiscal Year: 2013
    ..The work is clinically relevant because it will resolve questions about iron in the formal pathogenesis and natural history of FRDA, and the potential value of iron chelation. ..
  7. Genetic Studies of Optic Atrophy
    Taosheng Huang; Fiscal Year: 2012
    ..This study will allow us to understand how mutations of OPA1 cause optic atrophy and explore its novel function in lifespan and provide us a potential opportunity to develop treatments for the disease. ..
  8. Mitochondrial Dysfunction and Adipose Insulin Resistance
    DAVID BERNLOHR; Fiscal Year: 2009
    ..from genomic, proteomic, metabolomic and molecular analyses that point toward the covalent modification of mitochondrial proteins with bioactive lipids and the oxidation of mitochondrial thioredoxin as central to the process...
  9. Impaired mitochondrial fusion in pulmonary arterial hypertension
    Stephen L Archer; Fiscal Year: 2010
    ..Fusion is regulated by SNARE-like proteins called mitofusin-1 and mitofusin-2. Fusion redistributes mitochondrial proteins/genes, protecting the cell from oxidant stress, apoptosis and mitochondrial DNA mutations...
  10. Protein alterations in skeletal muscle cells with improved mtDNA repair
    Lyudmila I Rachek; Fiscal Year: 2010
    ..the generation of secondary ROS and thus reduce the proportion of oxidative modifications of specific mitochondrial proteins. This will increase mitochondrial protein function, and ultimately lead to enhanced mitochondrial function,..
  11. Nanoelectrode arrays for study of the molecular mechanisms, triggers, and inhibit
    Peter J Burke; Fiscal Year: 2012
    ..by the activation of the mitochondrial permeability transition pore (mtPTP), an aggregate of several mitochondrial proteins. When this pore opens, the critical membrane polarization of the mitochondrial inner membrane disappears ..
  12. DRUG RESISTANCE AND PTERIDINE METABOLISM IN LEISHMANIA
    Stephen M Beverley; Fiscal Year: 2010
    ..These studies have yielded new opportunities for chemotherapeutic attack and new directions for future study of pteridine metabolism and biology. [unreadable] ..
  13. FASEB SRC on Mitochondrial Assembly &Dynamics in Health, Disease &Aging
    Antoni Barrientos; Fiscal Year: 2011
    ..Experts will also learn about involvement of mitochondrial function/dysfunction in common age-related neurodegenerative disorders and the aging process. ..
  14. Deacetylation of mitochondrial proteins protect neurons from ischemic injury
    Conrad Alano; Fiscal Year: 2013
    ..In particular Sirt3 regulates acetylation level of mitochondrial proteins, and we will study how Sirt3 deacetylase activity reduces oxidative injury...
  15. Genetic Vulnerability to Alcohol Withdrawal and Genetically Correlated Behaviors
    Kari J Buck; Fiscal Year: 2012
    ..A major strength of this proposal is that we have identified three Alcdp1/Alcw1 candidates that encode mitochondrial proteins with primary effects on oxidative stress...
  16. AAA ATPases linking mitochondria with microtubule processing in flies and yeast
    Karen G Hales; Fiscal Year: 2012
    ..Elucidation of molecular mechanisms by which mitochondrial shaping is mediated by fly and yeast mitochondrial proteins related to those implicated in hereditary spastic paraplegia will enable characterization of related ..
  17. Protein Kinase C in the Repair of Cellular Functions
    Grazyna Nowak; Fiscal Year: 2013
    ..PKC-?, PKC-??are present in RPTC mitochondria and that PKC phosphorylates a number of yet unidentified mitochondrial proteins. We demonstrated that PKC-??activation mediates mitochondrial dysfunction following injury...
  18. Experimental Optic Neuritis: Gene Therapy
    John Guy; Fiscal Year: 2011
    ..We have found that peroxynitrite mediates nitration of key mitochondrial proteins. This commences during the earliest phase of the EAE animal model of MS, well before infiltration of ..
  19. Function of mRNA Editing in Trypanosomes
    Stephen L Hajduk; Fiscal Year: 2010
    ..we recently discovered that primary mRNAs are differentially edited and translated to produce novel mitochondrial proteins. Our initial studies focused on the mRNA for cytochrome c oxidase III (COIII), which we discovered was ..
  20. Mitochondrial chaperones mortalin and Tid1 in protein degradation
    Carolyn K Suzuki; Fiscal Year: 2012
    ..b>Mitochondrial proteins are at increased risk of protein misfolding and aggregation as they are located in proximity to the ..
  21. Oxygen/Nitrogen Stress in Acetaminophen Hepatotoxicity
    Jack A Hinson; Fiscal Year: 2013
    ..The ultimate goal is to develop new approaches to treat acetaminophen poisoning in humans. ..
  22. Anti-Aging Mitochondrial Protein Therapy for Alzheimer?s Disease and Sarcopenia
    JAMES PEPPER BENNETT; Fiscal Year: 2010
    ..As a degenerative disease, AD places a significant burden on caregivers and is therefore, one of the most economically costly diseases to society. The potential impact on public health is significant. ..
  23. ASS, NOS2, AND ALCOHOLIC LIVER DISEASE
    Natalia Nieto; Fiscal Year: 2012
    ..a combination of a cutting edge proteomics technique along with a Systems Biology approach to elucidate mitochondrial proteins involved in ALD that could impact NO synthesis...
  24. MYOCARDIAL PERFUSION IN THE HYPERTROPHIED HEART
    ROBERT JAMES BACHE; Fiscal Year: 2010
    ..These studies will characterize responses of myocardial KATP channels that may contribute to the development of CHF in the chronically overloaded heart. ..
  25. REGULATION OF MYOCARDIAL GROWTH AND DEATH BY THE HIPPO PATHWAY
    Junichi Sadoshima; Fiscal Year: 2013
    ..K-Ras, Rassf1A and Mst1 form a complex and phosphorylate mitochondrial proteins, including Bcl-xL, thereby stimulating apoptosis. 2...
  26. Exercise Induced Biochemical and Anatomical Adaptations
    John O Holloszy; Fiscal Year: 2013
    ..A third goal is to determine whether the initial, very rapid, increase in some mitochondrial proteins is mediated by increased translation of preexisting mRNAs...
  27. PGC-1 &Mitichondrial Dysfunction in Diabetes
    Lawrence J Mandarino; Fiscal Year: 2012
    ..2. To determine how insulin resistance alters the pattern of abundance of mitochondrial proteins. 3. To determine how insulin resistance alters phosphorylation of proteins in the ETC...
  28. Metabolic &Developmental Aspects of Intellectual Disability
    Mary C McKenna; Fiscal Year: 2013
    ..effects of gender on mechanisms and outcome, as well as optimization of neurologic outcome by protection against cell death, protecting mitochondrial proteins, preserving signal transduction, or promotion of neurogenesis.
  29. Mitochondrial Proteins in Parkinson's Disease
    J Timothy Greenamyre; Fiscal Year: 2013
    ....
  30. Functional Characterization of CCDC130 Gene
    Deepak Kumar; Fiscal Year: 2012
    ..The proposal may lead to development of a novel cancer biomarker and/or a drug target. The proposal will catalyze PIs transition to non-SCORE funding mechanism and enhance biomedical research at a minority serving institution like UDC. ..
  31. Mechanisms of Gene Silencing in Friedreich's Ataxia
    Joel M Gottesfeld; Fiscal Year: 2013
    ..By studying the mechanisms whereby these repeats silence frataxin gene expression, new therapeutic strategies will come from these studies. ..
  32. Indicators of early neurodegeneration: gene-toxin interactions at the syanpse
    Felix E Schweizer; Fiscal Year: 2010
    ..Interestingly, some PD-linked genes code for mitochondrial proteins and some pesticides linked to PD inhibit the mitochondrial electron transport chain...
  33. Maintenance of intestinal epithelial cell homeostasis by prohibitin
    Arianne L Theiss; Fiscal Year: 2013
    ..We believe our findings will identify novel molecular mechanisms that act to preserve epithelial barrier integrity basally and in the context of tissue injury and will provide new therapeutic targets. ..
  34. Stimulation of Mitochondrial Biogenesis with PDE3 Inhibitors
    Ryan M Whitaker; Fiscal Year: 2013
    ..Our laboratory has demonstrated persistent mitochondrial dysfunction and depletion of mitochondrial proteins following renal I/R injury that correlates with sustained renal tubular dysfunction in these animals...
  35. Redox Control of Apoptosis Signaling
    MARGARET BRIEHL; Fiscal Year: 2009
    ..2) To use immunoblots and 2D gels with proteomic analyses to identify key differences in mitochondrial proteins between glucocorticoid-sensitive and glucocorticoid-resistant lymphoma-derived cell lines;2D gels will ..
  36. Cellular Functions of Parkin
    Jian Feng; Fiscal Year: 2013
    ..Estrogen-Related Receptor 1 (ERR1), which plays a significant role in transcription regulation of many mitochondrial proteins including MAO...
  37. Molecular and Epigenetic Mitochondriomics of Air Particles, Lead and Cognition
    Andrea Baccarelli; Fiscal Year: 2013
    ..damage, heteroplasmy and mtDNA abundance;and 2) methylation of nuclear DNA (nDNA) genes coding for mitochondrial proteins (for which we will have the unique opportunity to time- and cost-effectively abstract information from ..
  38. MOLECULAR REGULATION OF MUSCLE GLUCOSE METABOLISM IN MAN
    Lawrence J Mandarino; Fiscal Year: 2013
    ..Preliminary data indicate that acetylation of mitochondrial proteins in humans is regulated by muscle contraction and is dysregulated in insulin resistance...
  39. A Mouse model of Barth syndrome, a mitochondrial cardiolipin disorder
    Zaza Khuchua; Fiscal Year: 2013
    ....
  40. Physical and Functional Systems Analysis of Mammalian Mitochondria
    JODI M NUNNARI; Fiscal Year: 2013
    ..Collectively, these objectives are designed to provide new insights into the complex etiologies of diseases and have the potential to identify novel therapeutic avenues. ..
  41. The New Family of NEET Proteins: 2Fe-2S Protein Mediated Health and Disease
    Patricia A Jennings; Fiscal Year: 2013
    ..The proposed studies are of both fundamental significance in understanding this protein-drug recognition as well as the identification of novel therapeutics. ..
  42. Mechanisms of Action of C. perfringens Enterotoxin
    BRUCE A MC CLANE; Fiscal Year: 2013
    ..transmission electron microscopy to evaluate complex mass and homogeneity, investigate the role of mitochondrial proteins in CPE-induced cell death, evaluate CPE complex formation in vivo, and determine if CPE induces intestinal ..
  43. High throughput screening assays to identify small molecules that target the ClpX
    Carolyn K Suzuki; Fiscal Year: 2010
    ..In addition, activators of ClpXP and/or Lon may also be exploited in the treatment of neurodegenerative disorders linked to mitochondrial protein aggregation. ..
  44. Defining Molecular Determinants of Type 2 Diabetes Using Quantitative Proteomics
    Paul A Grimsrud; Fiscal Year: 2013
    ..A parallel hypothesis I will test is that induction of aldehyde dehydrogenase 3A2 (Aldh3a2) expression and phosphorylation is a compensatory response in obesity for detoxifying reactive lipid-peroxidation products. ..
  45. Mechanisms of Hypothermic Protection from Ischemia/Reperfusion Cardiac Injury
    Kimm J Hamann; Fiscal Year: 2010
    ..through which hypothermia-induced NO generation provides protection and prevents release of pro-apoptotic mitochondrial proteins cyt c and activation of caspases...
  46. MECHANISMS OF HUMAN INSULIN RESISTANCE
    W Timothy Garvey; Fiscal Year: 2010
    ..differential gene expression in human muscle using cDNA microarrays and found that multiple genes encoding mitochondrial proteins are down-regulated in insulin resistance...
  47. Adipocyte Mithochondrial Dysfunction in Insulin Resistance
    Christian Meyer; Fiscal Year: 2010
    ..2) To use HPLC-ESI-MS/MS proteomics to determine the relative abundance of mitochondrial proteins in adipocytes from subjects that are resistant to suppression of FFA release by insulin and subjects that ..
  48. Discovery of Biomakers in Human Mitochondrial Disorders
    RONALD WAYNE DAVIS; Fiscal Year: 2011
    ..and redox balance, while subcellular localization of candidate genes is performed to identify new mitochondrial proteins and expand proteome annotations...
  49. Mitochondrial Genetic Susceptibility to Breast and Prostate Cancers
    Iona Cheng; Fiscal Year: 2013
    ..genes and nuclear regulators of mitochondrial activity will be identified from a comprehensive catalog of mitochondrial proteins based on computational genomics, protein biochemistry, and imaging...
  50. Substrates and Binding Partners in the PINK1/Parkin Pathway
    Derek P Narendra; Fiscal Year: 2011
    ..of Parkin to mitochondria and its promotion of mitochondrial degradation require binding to specific mitochondrial proteins. To address these hypotheses, the proposed research will use radiolabeling in cell culture and high ..
  51. Mechanisms of glucose mediated cardiac mitochondrial dysfunction
    ADAM RAYMOND WENDE; Fiscal Year: 2013
    ..mitochondrial regulation by examining glucose-delivery regulated post-translational modification of mitochondrial proteins (Aim 1) and epigenetic control of oxidative phosphorylation (OXPHOS) gene expression (Aim 2)...
  52. Mechanisms of glucose mediated cardiac mitochondrial dysfunction
    ADAM RAYMOND WENDE; Fiscal Year: 2013
    ..mitochondrial regulation by examining glucose-delivery regulated post-translational modification of mitochondrial proteins (Aim 1) and epigenetic control of oxidative phosphorylation (OXPHOS) gene expression (Aim 2)...
  53. High Fat Diet and Deficient Antioxidant Status as Contributing Factors to Mitocho
    CARIN contact THOMAS; Fiscal Year: 2011
    ..Exactly how high-fat diets or intracellular lipid accumulation lead to mitochondrial dysfunction or how mitochondrial proteins contribute to this process is unclear...
  54. MAVS protein: A role of mitochondria in innate immunity and cardioprotection
    Kirk Peterson; Fiscal Year: 2013
    ..abstract_text> ..
  55. Dendritic Cell Activation by Mitochondrial Transcription Factor A
    Elliott D Crouser; Fiscal Year: 2010
    ..This project has important implications for understanding how cell and tissue damage, such as commonly occurs in many acute illnesses, can induce activation of the immune system, which is potentially harmful to the host. ..
  56. Modulation of Oxidative phosphorylation by mitochondrial soluble adenylyl cyclase
    Giovanni Manfredi; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Phosphorylation of mitochondrial proteins is a rapid and efficient way to regulate oxidative phosphorylation (OXPHOS) and maintain energy homeostasis...
  57. Titanium-gold-based chemotherapeutics for prostate and kidney cancer
    Maria Contel; Fiscal Year: 2013
    ..of these compounds with different biomolecular targets, including protein kinases, DNA, transport and mitochondrial proteins, and cell death pathways...
  58. NEUROTROPHIC FACTOR DEPRIVATION AND NEURONAL CELL DEATH
    Lloyd A Greene; Fiscal Year: 2013
    ..Induction of Bim promotes release of mitochondrial proteins and activation of caspases, resulting in neuron death...
  59. Control of Yeast Life Span
    Kurt W Runge; Fiscal Year: 2013
    ..We have also discovered a way to find many new mutations that prolong life in yeast that can reveal similar ways to prolong life in human cells. ..
  60. Inducers of Mitochondrial Biogenesis
    Craig Cano Beeson; Fiscal Year: 2011
    ..We identified 7 hits that were confirmed with secondary assays of increased expression of mitochondrial proteins, the "gold standard"...
  61. REVERSIBLE MITOCHONDRIAL PROTEIN ACETYLATION AND METABOLIC REGULATION
    ERIC M VERDIN; Fiscal Year: 2013
    ..abstract_text> ..
  62. SECRETION FROM INDIVIDUAL VESICLES
    ROBERT MARK WIGHTMAN; Fiscal Year: 2012
    ..In the proposed research, these processes will be investigated with chemical sensing microelectrodes placed adjacent to isolated cells or implanted in slices of brain tissue. ..
  63. CYTOCHROME B5--A CASE STUDY IN MOLECULAR RECOGNITION
    Mario Rivera; Fiscal Year: 1999
    ....
  64. HUMAN N FORMYL PEPTIDE RECEPTOR--REGULATION AND GENETICS
    David Haviland; Fiscal Year: 2000
    ..that bind the fMLF-R are thought to be derived from bacterial protein degradation and possibly from mitochondrial proteins upon tissue damage...
  65. Diaphragm Mitochondrial Alterations in Sepsis
    Leigh Callahan; Fiscal Year: 2004
    ..kinase, (b) these physiologic changes are due, in turn, to alterations in the content and composition of mitochondrial proteins, and (c) protein changes are due, in part, to free radical-mediated decrements in mitochondrial gene ..
  66. Small molecule modulators for mitochondrial protein import
    Carla Koehler; Fiscal Year: 2009
    ..Long-term, this project may lead to the development of therapeutics that modulates mitochondrial function in these diseases. ..
  67. MOLECULAR MECHANISMS OF APOPTOSIS IN MYOCARDIAL ISCHEMIA
    Seigo Izumo; Fiscal Year: 2002
    ....
  68. A New Toxicity Screen to Assess Mitochondrial DNA Content and Protein Synthesis
    ROSS JOHN WHITTAKER; Fiscal Year: 2010
    ....
  69. Functional Proteomic Analysis of Cardiac Mitochondria
    Peipei Ping; Fiscal Year: 2005
    ..correlate, or a partial catalog of proteins without information regarding the manner in which the mitochondrial proteins are organized to perform their cellular tasks, will be insufficient to generate a thorough understanding ..
  70. Functional Proteogenomics of Mitochondria
    Peter Oefner; Fiscal Year: 2005
    ..phenotypes have been associated with mutations in a quarter of the known 406 nuclear-encoded human mitochondrial proteins. Estimated 500-600 nuclear encoded mitochondrial proteins await identification...