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| mitochondrial proteinsSummarySummary: Proteins encoded by the mitochondrial genome or proteins encoded by the nuclear genome that are imported to and resident in the MITOCHONDRIA. Top Publications
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Publications
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...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....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...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...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...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...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...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...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....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...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...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...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...Importing mitochondrial proteins: machineries and mechanisms
Agnieszka Chacinska
Institut fur Biochemie und Molekularbiologie, ZBMZ, Universitat Freiburg, 79104 Freiburg, Germany
Cell 138:628-44. 2009Most mitochondrial proteins are synthesized on cytosolic ribosomes and must be imported across one or both mitochondrial membranes...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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....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...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...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...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....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...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)...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....Translocation of proteins into mitochondria
Walter Neupert
Institut fur Physiologische Chemie, Universitat Munchen, 81377 Munchen, Germany
Annu Rev Biochem 76:723-49. 2007About 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...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...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...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...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...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...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. 2004Substrates 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....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.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...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...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....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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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...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....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...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...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...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...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....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 ..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...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...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...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...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...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...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....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...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...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...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...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...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...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...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...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...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 Grants
- THE ROLE OF ACETYLATION IN MITOCHONDRIAL FUNCTIONGary 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...
- Functional Genomics of the Mitochondrial ProteomeDennis 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...
- Mitochondrial Dysfunction and Adipose Insulin ResistanceDavid 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...
- Regulation of epithelial cell homeostasis by actin microfilamentsSeema Khurana; Fiscal Year: 2010....
- Protein Nitration in the PlacentaLeslie Myatt; Fiscal Year: 2012..Nitration of mitochondrial proteins is a reversible process dependent on oxygen concentration hence the hypoxic environment or ischemia/..
- Iron in the pathogenesis of Friedreich's ataxiaARNULF 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. ..
- Genetic Studies of Optic AtrophyTaosheng 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. ..
- Mitochondrial Dysfunction and Adipose Insulin ResistanceDAVID 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...
- Impaired mitochondrial fusion in pulmonary arterial hypertensionStephen 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...
- Protein alterations in skeletal muscle cells with improved mtDNA repairLyudmila 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,..
- Nanoelectrode arrays for study of the molecular mechanisms, triggers, and inhibitPeter 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 ..
- DRUG RESISTANCE AND PTERIDINE METABOLISM IN LEISHMANIAStephen 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] ..
- FASEB SRC on Mitochondrial Assembly &Dynamics in Health, Disease &AgingAntoni Barrientos; Fiscal Year: 2011..Experts will also learn about involvement of mitochondrial function/dysfunction in common age-related neurodegenerative disorders and the aging process. ..
- Deacetylation of mitochondrial proteins protect neurons from ischemic injuryConrad Alano; Fiscal Year: 2013..In particular Sirt3 regulates acetylation level of mitochondrial proteins, and we will study how Sirt3 deacetylase activity reduces oxidative injury...
- Genetic Vulnerability to Alcohol Withdrawal and Genetically Correlated BehaviorsKari 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...
- AAA ATPases linking mitochondria with microtubule processing in flies and yeastKaren 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 ..
- Protein Kinase C in the Repair of Cellular FunctionsGrazyna 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...
- Experimental Optic Neuritis: Gene TherapyJohn 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 ..
- Function of mRNA Editing in TrypanosomesStephen 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 ..
- 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 ..
- Oxygen/Nitrogen Stress in Acetaminophen HepatotoxicityJack A Hinson; Fiscal Year: 2013..The ultimate goal is to develop new approaches to treat acetaminophen poisoning in humans. ..
- Anti-Aging Mitochondrial Protein Therapy for Alzheimer?s Disease and SarcopeniaJAMES 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. ..
- ASS, NOS2, AND ALCOHOLIC LIVER DISEASENatalia 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...
- MYOCARDIAL PERFUSION IN THE HYPERTROPHIED HEARTROBERT 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. ..
- REGULATION OF MYOCARDIAL GROWTH AND DEATH BY THE HIPPO PATHWAYJunichi Sadoshima; Fiscal Year: 2013..K-Ras, Rassf1A and Mst1 form a complex and phosphorylate mitochondrial proteins, including Bcl-xL, thereby stimulating apoptosis. 2...
- Exercise Induced Biochemical and Anatomical AdaptationsJohn 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...
- PGC-1 &Mitichondrial Dysfunction in DiabetesLawrence 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...
- Metabolic &Developmental Aspects of Intellectual DisabilityMary 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.
- Mitochondrial Proteins in Parkinson's DiseaseJ Timothy Greenamyre; Fiscal Year: 2013....
- Functional Characterization of CCDC130 GeneDeepak 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. ..
- Mechanisms of Gene Silencing in Friedreich's AtaxiaJoel M Gottesfeld; Fiscal Year: 2013..By studying the mechanisms whereby these repeats silence frataxin gene expression, new therapeutic strategies will come from these studies. ..
- Indicators of early neurodegeneration: gene-toxin interactions at the syanpseFelix 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...
- Maintenance of intestinal epithelial cell homeostasis by prohibitinArianne 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. ..
- Stimulation of Mitochondrial Biogenesis with PDE3 InhibitorsRyan 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...
- Redox Control of Apoptosis SignalingMARGARET 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 ..
- Cellular Functions of ParkinJian Feng; Fiscal Year: 2013..Estrogen-Related Receptor 1 (ERR1), which plays a significant role in transcription regulation of many mitochondrial proteins including MAO...
- Molecular and Epigenetic Mitochondriomics of Air Particles, Lead and CognitionAndrea 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 ..
- MOLECULAR REGULATION OF MUSCLE GLUCOSE METABOLISM IN MANLawrence 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...
- A Mouse model of Barth syndrome, a mitochondrial cardiolipin disorderZaza Khuchua; Fiscal Year: 2013....
- Physical and Functional Systems Analysis of Mammalian MitochondriaJODI 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. ..
- The New Family of NEET Proteins: 2Fe-2S Protein Mediated Health and DiseasePatricia 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. ..
- Mechanisms of Action of C. perfringens EnterotoxinBRUCE 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 ..
- High throughput screening assays to identify small molecules that target the ClpXCarolyn 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. ..
- Defining Molecular Determinants of Type 2 Diabetes Using Quantitative ProteomicsPaul 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. ..
- Mechanisms of Hypothermic Protection from Ischemia/Reperfusion Cardiac InjuryKimm 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...
- MECHANISMS OF HUMAN INSULIN RESISTANCEW 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...
- Adipocyte Mithochondrial Dysfunction in Insulin ResistanceChristian 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 ..
- Discovery of Biomakers in Human Mitochondrial DisordersRONALD 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...
- Mitochondrial Genetic Susceptibility to Breast and Prostate CancersIona 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...
- Substrates and Binding Partners in the PINK1/Parkin PathwayDerek 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 ..
- Mechanisms of glucose mediated cardiac mitochondrial dysfunctionADAM 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)...
- Mechanisms of glucose mediated cardiac mitochondrial dysfunctionADAM 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)...
- High Fat Diet and Deficient Antioxidant Status as Contributing Factors to MitochoCARIN 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...
- MAVS protein: A role of mitochondria in innate immunity and cardioprotectionKirk Peterson; Fiscal Year: 2013..abstract_text> ..
- Dendritic Cell Activation by Mitochondrial Transcription Factor AElliott 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. ..
- Modulation of Oxidative phosphorylation by mitochondrial soluble adenylyl cyclaseGiovanni Manfredi; Fiscal Year: 2013DESCRIPTION (provided by applicant): Phosphorylation of mitochondrial proteins is a rapid and efficient way to regulate oxidative phosphorylation (OXPHOS) and maintain energy homeostasis...
- Titanium-gold-based chemotherapeutics for prostate and kidney cancerMaria Contel; Fiscal Year: 2013..of these compounds with different biomolecular targets, including protein kinases, DNA, transport and mitochondrial proteins, and cell death pathways...
- NEUROTROPHIC FACTOR DEPRIVATION AND NEURONAL CELL DEATHLloyd A Greene; Fiscal Year: 2013..Induction of Bim promotes release of mitochondrial proteins and activation of caspases, resulting in neuron death...
- Control of Yeast Life SpanKurt 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. ..
- Inducers of Mitochondrial BiogenesisCraig Cano Beeson; Fiscal Year: 2011..We identified 7 hits that were confirmed with secondary assays of increased expression of mitochondrial proteins, the "gold standard"...
- REVERSIBLE MITOCHONDRIAL PROTEIN ACETYLATION AND METABOLIC REGULATIONERIC M VERDIN; Fiscal Year: 2013..abstract_text> ..
- SECRETION FROM INDIVIDUAL VESICLESROBERT 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. ..
- CYTOCHROME B5--A CASE STUDY IN MOLECULAR RECOGNITIONMario Rivera; Fiscal Year: 1999....
- HUMAN N FORMYL PEPTIDE RECEPTOR--REGULATION AND GENETICSDavid 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...
- Diaphragm Mitochondrial Alterations in SepsisLeigh 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 ..
- Small molecule modulators for mitochondrial protein importCarla Koehler; Fiscal Year: 2009..Long-term, this project may lead to the development of therapeutics that modulates mitochondrial function in these diseases. ..
- MOLECULAR MECHANISMS OF APOPTOSIS IN MYOCARDIAL ISCHEMIASeigo Izumo; Fiscal Year: 2002....
- A New Toxicity Screen to Assess Mitochondrial DNA Content and Protein SynthesisROSS JOHN WHITTAKER; Fiscal Year: 2010....
- Functional Proteomic Analysis of Cardiac MitochondriaPeipei 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 ..
- Functional Proteogenomics of MitochondriaPeter 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...