mitochondrial membrane transport proteins

Summary

Summary: Proteins involved in the transport of specific substances across the membranes of the MITOCHONDRIA.

Top Publications

  1. 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
  2. ncbi Effect of cyclosporine on reperfusion injury in acute myocardial infarction
    Christophe Piot
    Hopital Arnaud de Villeneuve, Montpellier, France
    N Engl J Med 359:473-81. 2008
  3. 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
  4. pmc PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death
    Sonia Gandhi
    Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, UK
    Mol Cell 33:627-38. 2009
  5. ncbi Mitochondrial permeability transition in Ca(2+)-dependent apoptosis and necrosis
    Andrea Rasola
    Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
    Cell Calcium 50:222-33. 2011
  6. ncbi The mitochondrial permeability transition pore and its involvement in cell death and in disease pathogenesis
    Andrea Rasola
    CNR Institute of Neuroscience and Department of Biomedical Sciences, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padua, Italy
    Apoptosis 12:815-33. 2007
  7. 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
  8. ncbi Mitochondrial permeability transition pore opening during myocardial reperfusion--a target for cardioprotection
    Andrew P Halestrap
    Department of Biochemistry and The Bristol Heart Institute, University of Bristol, Bristol BS8 1TD, UK
    Cardiovasc Res 61:372-85. 2004
  9. ncbi What is the mitochondrial permeability transition pore?
    Andrew P Halestrap
    Department of Biochemistry and Bristol Heart Institute, University of Bristol, School of Medical Sciences, University Walk, Bristol, UK
    J Mol Cell Cardiol 46:821-31. 2009
  10. pmc Mitochondrial calcium and the permeability transition in cell death
    John J Lemasters
    Center for Cell Death, Injury and Regeneration, Medical University of South Carolina, Charleston, SC 29425, USA
    Biochim Biophys Acta 1787:1395-401. 2009

Research Grants

  1. DNA Damage/Repair and Cell Death
    Lee Martin; Fiscal Year: 2009
  2. Mechanisms of Motor Neuron Death
    Lee Martin; Fiscal Year: 2006
  3. MECHANISMS OF NEURONAL APOPTOSIS IN VIVO
    Lee Martin; Fiscal Year: 2009
  4. PHOSPHOLIPASE C AND CARDIAC HYPERTROPHY
    GERALD DORN; Fiscal Year: 2005
  5. Calpains as Mediators of Cardiac Ischemic Injury
    Gerald W Dorn; Fiscal Year: 2010
  6. Effect of Drugs on Myocardial Hypoxia
    Garrett Gross; Fiscal Year: 2009
  7. PHOSPHATE TRANSPORT PATHS WITHIN HOMODIMERIC PTP
    Hartmut Wohlrab; Fiscal Year: 2001
  8. CATECHOLS AND ADENOSINE IN MYOCARDIAL PRECONDITIONING
    Michael Cohen; Fiscal Year: 2006
  9. Mitochondrial control of neuronal excitotoxicity
    David Nicholls; Fiscal Year: 2005
  10. Aging-Related Models and Mitochondrial Bioenergetics
    David Nicholls; Fiscal Year: 2006

Detail Information

Publications286 found, 100 shown here

  1. 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...
  2. ncbi Effect of cyclosporine on reperfusion injury in acute myocardial infarction
    Christophe Piot
    Hopital Arnaud de Villeneuve, Montpellier, France
    N Engl J Med 359:473-81. 2008
    ..In this pilot trial, we sought to determine whether the administration of cyclosporine at the time of percutaneous coronary intervention (PCI) would limit the size of the infarct during acute myocardial infarction...
  3. 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...
  4. pmc PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death
    Sonia Gandhi
    Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, UK
    Mol Cell 33:627-38. 2009
    ..Our findings propose a mechanism by which PINK1 dysfunction renders neurons vulnerable to cell death...
  5. ncbi Mitochondrial permeability transition in Ca(2+)-dependent apoptosis and necrosis
    Andrea Rasola
    Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
    Cell Calcium 50:222-33. 2011
    ....
  6. ncbi The mitochondrial permeability transition pore and its involvement in cell death and in disease pathogenesis
    Andrea Rasola
    CNR Institute of Neuroscience and Department of Biomedical Sciences, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padua, Italy
    Apoptosis 12:815-33. 2007
    ..Here we review basic concepts about PTP structure, function and regulation within the framework of intracellular death signalling, and its role in disease pathogenesis...
  7. 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...
  8. ncbi Mitochondrial permeability transition pore opening during myocardial reperfusion--a target for cardioprotection
    Andrew P Halestrap
    Department of Biochemistry and The Bristol Heart Institute, University of Bristol, Bristol BS8 1TD, UK
    Cardiovasc Res 61:372-85. 2004
    ..However, only if subsequent MPTP closure occurs will ATP levels be maintained, ensuring that cell death continues down an apoptotic, rather than a necrotic, pathway...
  9. ncbi What is the mitochondrial permeability transition pore?
    Andrew P Halestrap
    Department of Biochemistry and Bristol Heart Institute, University of Bristol, School of Medical Sciences, University Walk, Bristol, UK
    J Mol Cell Cardiol 46:821-31. 2009
    ..Agents that modulate pore opening may act on either or both the PiC and the ANT. However, knockdown and reconstitution studies are awaited to confirm or refute this model...
  10. pmc Mitochondrial calcium and the permeability transition in cell death
    John J Lemasters
    Center for Cell Death, Injury and Regeneration, Medical University of South Carolina, Charleston, SC 29425, USA
    Biochim Biophys Acta 1787:1395-401. 2009
    ..Thus, the exact role of Ca(2+) for inducing the MPT and cell death depends on the particular biologic setting...
  11. ncbi The role of the mitochondrial permeability transition pore in heart disease
    Andrew P Halestrap
    Department of Biochemistry and Bristol Heart Institute, University of Bristol, School of Medical Sciences, University Walk, Bristol BS8 1TD, UK
    Biochim Biophys Acta 1787:1402-15. 2009
    ..This confirms the mPTP as a promising drug target in human cardiovascular disease. Indeed, the first clinical trials have shown CsA treatment improves recovery after treatment of a coronary thrombosis with angioplasty...
  12. pmc The ADP/ATP translocator is not essential for the mitochondrial permeability transition pore
    Jason E Kokoszka
    Center for Molecular and Mitochondrial Medicine and Genetics, University of California, Irvine, California 92697, USA
    Nature 427:461-5. 2004
    ..Moreover, hepatocytes without ANT remained competent to respond to various initiators of cell death. Therefore, ANTs are non-essential structural components of the mtPTP, although they do contribute to its regulation...
  13. ncbi Dual role of mitofilin in mitochondrial membrane organization and protein biogenesis
    Karina von der Malsburg
    Institut fur Biochemie und Molekularbiologie, ZBMZ, Universitat Freiburg, 79104 Freiburg, Germany
    Dev Cell 21:694-707. 2011
    ..Our findings indicate that mitofilin is a central component of MINOS and functions as a multifunctional regulator of mitochondrial architecture and protein biogenesis...
  14. ncbi Mitochondrial ROS-induced ROS release: an update and review
    Dmitry B Zorov
    Laboratory of Cardiovascular Science, Gerontology Research Center, National Institute on Aging, National Institutes of Health, Baltimore, MD 21224, USA
    Biochim Biophys Acta 1757:509-17. 2006
    ..RIRR could be a general cell biology phenomenon relevant to the processes of programmed mitochondrial destruction and cell death, and may contribute to other mechanisms of post-ischemic pathologies, including arrhythmias...
  15. pmc Glycogen synthase kinase-3beta mediates convergence of protection signaling to inhibit the mitochondrial permeability transition pore
    Magdalena Juhaszova
    Laboratory of Cardiovascular Science, Gerontology Research Center, Intramural Research Program, National Institute on Aging, NIH, Baltimore, Maryland 21224 6825, USA
    J Clin Invest 113:1535-49. 2004
    ..The convergence of these pathways via inhibition of GSK-3beta on the end effector, the permeability transition pore complex, to limit MPT induction is the general mechanism of cardiomyocyte protection...
  16. pmc Glutathione redox potential in the mitochondrial intermembrane space is linked to the cytosol and impacts the Mia40 redox state
    Kerstin Kojer
    Cellular Biochemistry, Department of Biology, University of Kaiserslautern, Germany
    EMBO J 31:3169-82. 2012
    ..Taken together, we provide a comprehensive mechanistic picture of the IMS redox milieu and define the redox influences on Mia40 in living cells...
  17. pmc Activation of mitochondrial ERK protects cancer cells from death through inhibition of the permeability transition
    Andrea Rasola
    Department of Biomedical Sciences, National Research Council Institute of Neuroscience and Venetian Institute of Molecular Medicine, University of Padua, 35121 Padua, Italy
    Proc Natl Acad Sci U S A 107:726-31. 2010
    ..Thus, in tumor cells mitochondrial ERK activation desensitizes the PTP through a signaling axis that involves GSK-3 and cyclophilin D, a finding that provides a mechanistic basis for increased resistance to apoptosis of neoplastic cells...
  18. pmc Signal transduction to the permeability transition pore
    Andrea Rasola
    Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
    FEBS Lett 584:1989-96. 2010
    ..Recent progress on PTP modulation by kinase/phosphatase signal transduction is discussed, with specific emphasis on hexokinase and on the Akt-ERK-GSK3 axis, which might modulate the PTP through CyP-D phosphorylation...
  19. ncbi Translocation of proteins into mitochondria
    Walter Neupert
    Institut fur Physiologische Chemie, Universitat Munchen, 81377 Munchen, Germany
    Annu Rev Biochem 76:723-49. 2007
    ..This review summarizes the present knowledge on the import and sorting of mitochondrial precursor proteins, with a special emphasis on unresolved questions and topics of current research...
  20. pmc Mitochondrial calcium signalling and cell death: approaches for assessing the role of mitochondrial Ca2+ uptake in apoptosis
    Gyorgy Hajnoczky
    Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Cell Calcium 40:553-60. 2006
    ..We also present a systematic evaluation of the effect of ruthenium red and Ru360, two inhibitors of mitochondrial Ca(2+) uptake on cytosolic [Ca(2+)] and [Ca(2+)](m) in intact cultured cells...
  21. ncbi The mitochondrial permeability transition from in vitro artifact to disease target
    Paolo Bernardi
    Department of Biomedical Sciences and CNR Institute of Neurosciences, University of Padova, Italy
    FEBS J 273:2077-99. 2006
    ..Finally, we discuss evidence indicating that the permeability transition pore plays a role in pathophysiology, with specific emphasis on in vivo models of disease...
  22. ncbi The mitochondrial permeability transition pore as a target for preconditioning and postconditioning
    Derek J Hausenloy
    The Hatter Institute and Center for Cardiology, University College London Hospitals and Medical School, Grafton Way, London, UK
    Basic Res Cardiol 104:189-202. 2009
    ..The objectve of this review article will be to explore the potential mechanisms which link IPC and IPost to mPTP inhibition in the reperfused heart...
  23. ncbi Frequency and distribution in East Asia of 12 mutations identified in the SLC25A13 gene of Japanese patients with citrin deficiency
    Yao Bang Lu
    Department of Molecular Metabolism and Biochemical Genetics, Kagoshima University Graduate School of Medical and Dental Sciences, 8 35 1 Sakuragaoka, Kagoshima 890 8544, Japan
    J Hum Genet 50:338-46. 2005
    ..We detected many carriers in Chinese (64/4169 = 1/65), Japanese (20/1372 = 1/69) and Korean (22/2455 = 1/112) populations, suggesting that over 80,000 East Asians are homozygotes with two mutated SLC25A13 alleles...
  24. pmc The role of mitochondria in protection of the heart by preconditioning
    Andrew P Halestrap
    Department of Biochemistry and Bristol Heart Institute, University of Bristol, School of Medical Sciences, University Walk, Bristol BS8 1TD, UK
    Biochim Biophys Acta 1767:1007-31. 2007
    ..This phase of protection may involve survival kinase pathways such as Akt and glycogen synthase kinase 3 (GSK3) either increasing ROS removal or reducing mitochondrial ROS production...
  25. ncbi Clinical pictures of 75 patients with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD)
    T Ohura
    Depatment of Paediatrics, Tohoku University School of Medicine, Sendai, Japan
    J Inherit Metab Dis 30:139-44. 2007
    ..Another patient developed citrullinaemia type II (CTLN2) at age 16 years. It is important to recognize that NICCD is not always a benign condition...
  26. pmc Cytosolic thioredoxin system facilitates the import of mitochondrial small Tim proteins
    Romina Durigon
    Manchester Institute of Biotechnology, Faculty of Life Sciences, University of Manchester, Manchester, UK
    EMBO Rep 13:916-22. 2012
    ..Here we provide the first evidence that the cytosolic thioredoxin system is required to maintain the IMS small Tim proteins in reduced forms and facilitate their mitochondrial import during respiratory growth...
  27. pmc Cyclophilin D controls mitochondrial pore-dependent Ca(2+) exchange, metabolic flexibility, and propensity for heart failure in mice
    John W Elrod
    Department of Pediatrics, University of Cincinnati, Cincinnati Children s Hospital Medical Center, Howard Hughes Medical Institute, Cincinnati, Ohio 45229, USA
    J Clin Invest 120:3680-7. 2010
    ..These findings suggest that the MPTP maintains homeostatic mitochondrial Ca(2+) levels to match metabolism with alterations in myocardial workload, thereby suggesting a physiologic function for the MPTP...
  28. ncbi Disulfide formation in the ER and mitochondria: two solutions to a common process
    Jan Riemer
    Cell Biology, University of Kaiserslautern, Erwin Schrödinger Strasse 13, 67663 Kaiserslautern, Germany
    Science 324:1284-7. 2009
    ..Here, we summarize the current knowledge of these oxidation systems and discuss their functional similarities and differences...
  29. ncbi Genetic dissection of the permeability transition pore
    Michael Forte
    Vollum Institute, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA
    J Bioenerg Biomembr 37:121-8. 2005
    ....
  30. ncbi Mitofusin-1 protein is a generally expressed mediator of mitochondrial fusion in mammalian cells
    Ansgar Santel
    Department of Developmental Biology, Stanford University School of Medicine, Stanford, CA 94305, USA
    J Cell Sci 116:2763-74. 2003
    ..Thus, Mfn1 appears to be a key player in mediating mitochondrial fusion and morphology in mammalian cells...
  31. ncbi Mitochondrial permeability transition pore opening as an endpoint to initiate cell death and as a putative target for cardioprotection
    Sabzali Javadov
    Department of Physiology and Pharmacology, Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada
    Cell Physiol Biochem 20:1-22. 2007
    ....
  32. ncbi Dual function of Sdh3 in the respiratory chain and TIM22 protein translocase of the mitochondrial inner membrane
    Natalia Gebert
    Institut fur Biochemie und Molekularbiologie, ZBMZ, Universitat Freiburg, 79104 Freiburg, Germany
    Mol Cell 44:811-8. 2011
    ..We conclude that the assembly of Sdh3 with different partner proteins, Sdh4 and Tim18, recruits it to two different mitochondrial membrane complexes with functions in bioenergetics and protein biogenesis, respectively...
  33. ncbi A pore way to die: the role of mitochondria in reperfusion injury and cardioprotection
    Andrew P Halestrap
    Department of Biochemistry and The Bristol Heart Institute, University of Bristol, School of Medical Sciences, University Walk, Bristol BS8 1TD, UK
    Biochem Soc Trans 38:841-60. 2010
    ..MPTP inhibition also protects against congestive heart failure in hypertensive animal models. Thus the MPTP is a very promising pharmacological target for clinical practice, especially once more specific drugs are developed...
  34. pmc High- and low-calcium-dependent mechanisms of mitochondrial calcium signalling
    András Spät
    Department of Physiology, Faculty of Medicine, Semmelweis University, Budapest, Hungary
    Cell Calcium 44:51-63. 2008
    ....
  35. ncbi The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein
    K Kobayashi
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Japan
    Nat Genet 22:159-63. 1999
    ....
  36. ncbi Hydrogen sulfide protects cardiomyocytes from hypoxia/reoxygenation-induced apoptosis by preventing GSK-3beta-dependent opening of mPTP
    Ling Ling Yao
    Dept of Physiology and Pathophysiology, Fudan University Shanghai Medical College, 138 Yi Xue Yuan Rd, Shanghai, China
    Am J Physiol Heart Circ Physiol 298:H1310-9. 2010
    ..In conclusion, the H(2)S donor prevents cardiomyocyte apoptosis by inducing phosphorylation of GSK-3beta (Ser9) and subsequent inhibition of mPTP opening...
  37. pmc Temperature preconditioning of isolated rat hearts--a potent cardioprotective mechanism involving a reduction in oxidative stress and inhibition of the mitochondrial permeability transition pore
    Igor Khaliulin
    Department of Biochemistry, Bristol Heart Institute, University of Bristol, UK
    J Physiol 581:1147-61. 2007
    ..The presence of N-(2-mercaptopropionyl) glycine during TP also abolished cardioprotection, indicating an involvement of free radicals in the signalling mechanism...
  38. ncbi Inhibition of GSK3beta by postconditioning is required to prevent opening of the mitochondrial permeability transition pore during reperfusion
    Ludovic Gomez
    INSERM U886, Universite Claude Bernard Lyon, 69373 Lyon, France
    Circulation 117:2761-8. 2008
    ..We investigated whether phosphorylated GSK3beta may protect the heart via the inhibition of mPTP opening during postconditioning...
  39. ncbi A disulfide relay system in the intermembrane space of mitochondria that mediates protein import
    Nikola Mesecke
    Institute für Physiologische Chemie, Universitat Munchen, Germany
    Cell 121:1059-69. 2005
    ..We suggest that this process reflects the evolutionary origin of the IMS from the periplasmic space of the prokaryotic ancestors of mitochondria...
  40. ncbi Mitochondria and ischemia-reperfusion injury of the heart: fixing a hole
    Fabio Di Lisa
    Dipartimento di Chimica Biologica, Universita di Padova, Viale G Colombo 3, 35121 Padova
    Cardiovasc Res 70:191-9. 2006
    ..Here, we discuss our current understanding of the structure and function of PTP in the context of heart injury caused by ischemia and reperfusion...
  41. pmc Mitochondrial permeability transition pore in Alzheimer's disease: cyclophilin D and amyloid beta
    Heng Du
    Departments of Pathology and Cell Biology, Surgery, and the Taub Institute for Research on Alzheimer s Disease and the Aging Brain, College of Physicians and Surgeons of Columbia University, 630 W 168th Street, New York, NY 10032, USA
    Biochim Biophys Acta 1802:198-204. 2010
    ..In this review, the role of Abeta in the formation of mPTP and the potential of mPTP inhibition as a therapeutic strategy in AD treatment are examined...
  42. ncbi Local control of mitochondrial membrane potential, permeability transition pore and reactive oxygen species by calcium and calmodulin in rat ventricular myocytes
    Keiichi Odagiri
    Division of Cardiology, Internal Medicine III, Hamamatsu University School of Medicine, Higashi Ward, Hamamatsu, Japan
    J Mol Cell Cardiol 46:989-97. 2009
    ..In addition, CaM was involved in the regulation of mitochondrial Ca(2+) homeostasis...
  43. ncbi The adenine nucleotide translocase: a central component of the mitochondrial permeability transition pore and key player in cell death
    Andrew P Halestrap
    Department of Biochemistry, School of Medical Sciences, University of Bristol, Bristol BS8 1TD, UK
    Curr Med Chem 10:1507-25. 2003
    ..Thus the ANT may provide a molecular link between physiopathological mechanisms of infection and the regulation of MPTP function and so represents a potential therapeutic target...
  44. pmc MICU2, a paralog of MICU1, resides within the mitochondrial uniporter complex to regulate calcium handling
    Molly Plovanich
    Departments of Molecular Biology and Medicine, Massachusetts General Hospital, and Department of Systems Biology, Harvard Medical School, Boston, Massachusetts, USA
    PLoS ONE 8:e55785. 2013
    ..The results identify MICU2 as a new component of the uniporter complex that may contribute to the tissue-specific regulation of this channel...
  45. ncbi Mitochondrial disulfide bond formation is driven by intersubunit electron transfer in Erv1 and proofread by glutathione
    Melanie Bien
    Institut fur Zellbiologie, Universitat Kaiserslautern, Erwin Schrödinger Strasse 13, 67663 Kaiserslautern, Germany
    Mol Cell 37:516-28. 2010
    ..The generation of these side products is efficiently counteracted by reduced glutathione. Thus, our findings suggest a role for a glutathione-dependent proofreading during oxidative protein folding by the mitochondrial disulfide relay...
  46. pmc Loss of MARCH5 mitochondrial E3 ubiquitin ligase induces cellular senescence through dynamin-related protein 1 and mitofusin 1
    Yong Yea Park
    Department of Biochemistry, Ajou University School of Medicine, Yeongtong Gu, Suwon 443 721, Korea
    J Cell Sci 123:619-26. 2010
    ..Collectively, our data indicate that the lack of MARCH5 results in mitochondrial elongation, which promotes cellular senescence by blocking Drp1 activity and/or promoting accumulation of Mfn1 at the mitochondria...
  47. ncbi High resolution melting analysis for the detection of SLC25A13 gene mutations in Taiwan
    Jing Ting Lin
    Department of Medical Laboratory Science and Biotechnology, National Cheng Kung University, Tainan, Taiwan
    Clin Chim Acta 412:460-5. 2011
    ..This study aimed to develop a high resolution melting (HRM) analysis for SLC25A13 mutation scanning and determine the carrier rate in Taiwan...
  48. ncbi Mitochondrial permeability transition pore as a target for cardioprotection in the human heart
    Selvaraj Shanmuganathan
    Hatter Institute and Center for Cardiology, University College London Hospitals and Medical School, Grafton Way, London WC1E 6DB, UK
    Am J Physiol Heart Circ Physiol 289:H237-42. 2005
    ..01). We report that suppressing mPTP opening at the onset of reoxygenation protects human myocardium against lethal hypoxia-reoxygenation injury. This suggests that, in the human heart, the mPTP is a viable target for cardioprotection...
  49. ncbi Adult-onset type II citrullinemia and idiopathic neonatal hepatitis caused by citrin deficiency: involvement of the aspartate glutamate carrier for urea synthesis and maintenance of the urea cycle
    Takeyori Saheki
    Department of Molecular Metabolism and Biochemical Genetics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
    Mol Genet Metab 81:S20-6. 2004
    ....
  50. ncbi Inhibition of mitochondrial permeability transition to prevent the post-cardiac arrest syndrome: a pre-clinical study
    Martin Cour
    INSERM U886 Cardioprotection, Lyon, France
    Eur Heart J 32:226-35. 2011
    ..Opening of the mitochondrial permeability transition pore (mPTP) appears to be a pivotal event in ischaemia-reperfusion injury. We hypothesized that pharmacological inhibition of mPTP opening may prevent the post-CA syndrome...
  51. ncbi The mitochondrial permeability transition pore and cyclophilin D in cardioprotection
    Fabio Di Lisa
    Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, 35121 Padova, Italy
    Biochim Biophys Acta 1813:1316-22. 2011
    ..This article is part of a Special Issue entitled: Mitochondria and Cardioprotection...
  52. pmc Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR
    Lucia Banci
    Magnetic Resonance Center CERM, University of Florence, Via Luigi Sacconi 6, Sesto Fiorentino, 50019 Florence, Italy
    Proc Natl Acad Sci U S A 108:4811-6. 2011
    ..The hydrophobicity-driven binding of this region ensures precise protein-protein recognition needed for an efficient electron transfer process...
  53. ncbi Genotypic and phenotypic features of citrin deficiency: five-year experience in a Chinese pediatric center
    Yuan Zong Song
    Department of Pediatrics, The First Affiliated Hospital, Jinan University, No 613 Huangpu Dadao Xi, Guangzhou 510630, Guangdong, PR China
    Int J Mol Med 28:33-40. 2011
    ....
  54. pmc Flurbiprofen, a cyclooxygenase inhibitor, protects mice from hepatic ischemia/reperfusion injury by inhibiting GSK-3β signaling and mitochondrial permeability transition
    Hailong Fu
    Departments of Anesthesiology, Changzheng Hospital, Second Military Medical University, Shanghai, China
    Mol Med 18:1128-35. 2012
    ....
  55. pmc In vivo evidence for cooperation of Mia40 and Erv1 in the oxidation of mitochondrial proteins
    Lena Böttinger
    Institut fur Biochemie und Molekularbiologie, Zentrum für Biochemie und Molekulare Zellforschung, 79104 Freiburg, Germany
    Mol Biol Cell 23:3957-69. 2012
    ..Thus Mia40 in cooperation with Erv1 promotes the formation of two disulfide bonds in the substrate protein, ensuring the efficiency of oxidative folding in the intermembrane space of mitochondria...
  56. pmc Characteristics and possible functions of mitochondrial Ca(2+) transport mechanisms
    Thomas E Gunter
    Department of Biochemistry and Biophysics and Mitochondrial Research and Innovation Group, University of Rochester School of Medicine and Dentistry, 601 Elmwood Avenue, Rochester, NY 14642, USA
    Biochim Biophys Acta 1787:1291-308. 2009
    ..The characteristics of these mechanisms of Ca(2+) transport and a discussion of how they might function are described in this paper...
  57. ncbi Screening assays for the mitochondrial permeability transition using a fluorescence multiwell plate reader
    J R Blattner
    Department of Cell Biology and Anatomy, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7090, USA
    Anal Biochem 295:220-6. 2001
    ..This high-throughput multiwell assay is amenable for screening panels of compounds for their ability to promote or block the MPT...
  58. ncbi Molecular analysis of SLC25A13 gene in human peripheral blood lymphocytes: Marked transcript diversity, and the feasibility of cDNA cloning as a diagnostic tool for citrin deficiency
    Zhan Hui Zhang
    Department of Pediatrics, The First Affiliated Hospital, Jinan University, Guangzhou 510630, China
    Gene 511:227-34. 2012
    ....
  59. ncbi Citrin deficiency, a perplexing global disorder
    David Dimmock
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, BCM225, Houston, TX 77030, USA
    Mol Genet Metab 96:44-9. 2009
    ..The finding of citrin mutations in patients of Arabic, Pakistani, French Canadian and Northern European origins supports the concept that citrin deficiency is a panethnic disease...
  60. ncbi Protein translocation into mitochondria: the role of TIM complexes
    M F Bauer
    Institut fur Klinische Chemie, Molekulare Diagnostik und Institut für Klinische Chemie, Akad Krankenhaus München Schwabing, D 80804 München, Germany
    Trends Cell Biol 10:25-31. 2000
    ....
  61. pmc Mitochondrial biogenesis, switching the sorting pathway of the intermembrane space receptor Mia40
    Agnieszka Chacinska
    Institut fur Biochemie und Molekularbiologie, ZBMZ, Universitat Freiburg, 79104 Freiburg, Germany
    J Biol Chem 283:29723-9. 2008
    ....
  62. ncbi Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease
    Michiharu Komatsu
    Department of Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan
    J Hepatol 49:810-20. 2008
    ..As its clinical features remain unclear, we aimed to explore the characteristics of fatty liver disease associated with citrin deficiency...
  63. ncbi Neonatal intrahepatic cholestasis caused by citrin deficiency: clinical and laboratory investigation of 13 subjects in mainland of China
    Y Z Song
    Department of Pediatrics, First Affiliated Hospital, Jinan University, Guangzhou 510630, China
    Dig Liver Dis 41:683-9. 2009
    ..Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is a novel inborn error of metabolism due to dysfunction of citrin protein, and much more information about this new disease is still needed for its clinical management...
  64. ncbi Novel diagnostic approach to citrin deficiency: analysis of citrin protein in lymphocytes
    Daisuke Tokuhara
    Department of Pediatrics, Osaka City University Graduate School of Medicine, 1 4 3 Asahimachi, Abenoku, Osaka 545 8585, Japan
    Mol Genet Metab 90:30-6. 2007
    ..We disclosed that citrin was deficient in lymphocytes among patients with citrin deficiency. Analysis of citrin is useful to diagnose citrin deficiency even in patients without known mutations or hypercitrullinemia...
  65. ncbi Simple and rapid genetic testing for citrin deficiency by screening 11 prevalent mutations in SLC25A13
    Atsuo Kikuchi
    Department of Pediatrics, Tohoku University Graduate School of Medicine, 1 1 Seiryo machi, Aoba ku, Sendai, Miyagi 980 8574, Japan
    Mol Genet Metab 105:553-8. 2012
    ..Therefore, this assay could be used for newborn screening and for facilitating the genetic diagnosis of citrin deficiency, especially in East Asian populations...
  66. ncbi Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency
    Takeyori Saheki
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, Kagoshima, Japan
    Metab Brain Dis 17:335-46. 2002
    ..From the viewpoint of the metabolic functions of citrin as aspartate glutamate carrier in urea synthesis and NADH shuttle, symptoms of CTLN2 and neonatal intrahepatic cholestasis caused by citrin deficiency are analyzed...
  67. pmc The nucleotide exchange factor MGE exerts a key function in the ATP-dependent cycle of mt-Hsp70-Tim44 interaction driving mitochondrial protein import
    H C Schneider
    Institut fur Physiologische Chemie, Universitat Munchen, Germany
    EMBO J 15:5796-803. 1996
    ..Subsequently, the release of mt-Hsp70 from the polypeptide chain is triggered by Mge1p which promotes release of ADP from mt-Hsp70. Rebinding of ATP to mt-Hsp70 completes the reaction cycle...
  68. ncbi A new Caucasian case of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD): a clinical, molecular, and functional study
    Giuseppe Fiermonte
    Department of Pharmaco Biology, Laboratory of Biochemistry and Molecular Biology, University of Bari, Bari, Italy
    Mol Genet Metab 104:501-6. 2011
    ....
  69. pmc Cyclosporin A and cardioprotection: from investigative tool to therapeutic agent
    Derek J Hausenloy
    The Hatter Cardiovascular Institute, University College London Hospital and Medical School, London, UK
    Br J Pharmacol 165:1235-45. 2012
    ..In this article, we review the intriguing role of CsA as a tool for investigating the mPTP as a target for cardioprotection and its potential role as a therapeutic agent for patients with IHD...
  70. ncbi Transient opening of mitochondrial permeability transition pore by reactive oxygen species protects myocardium from ischemia-reperfusion injury
    Masao Saotome
    Division of Cardiology, Internal Medicine III, Hamamatsu Univ School of Medicine, 1 20 1 Handayama, Higashi ku, Hamamatsu, 431 3192, Japan
    Am J Physiol Heart Circ Physiol 296:H1125-32. 2009
    ....
  71. ncbi Mitochondrial Ca2+ transport and permeability transition in zebrafish (Danio rerio)
    Luca Azzolin
    Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padua, Italy
    Biochim Biophys Acta 1797:1775-9. 2010
    ..Our results represent a necessary step toward the use of zebrafish for the screening and validation of PTP inhibitors of potential use in human diseases, as recently shown for collagen VI muscular dystrophy [Telfer et al., 2010]...
  72. pmc Inhibition of mitochondrial permeability transition pore opening by ischemic preconditioning is probably mediated by reduction of oxidative stress rather than mitochondrial protein phosphorylation
    Samantha J Clarke
    Department of Biochemistry and The Bristol Heart Institute, University of Bristol, Bristol BS8 1TD, United Kingdom
    Circ Res 102:1082-90. 2008
    ..The signaling pathways mediating this effect and maintaining it during reperfusion are discussed...
  73. pmc Assembly of the mitochondrial apoptosis-induced channel, MAC
    Sonia Martinez-Caballero
    Department of Basic Sciences, New York University College of Dentistry, New York, New York 10010, USA
    J Biol Chem 284:12235-45. 2009
    ..5-6 nm. The mitochondrial permeability data are inconsistent with formation of lipidic pores capable of transporting megadalton-sized macromolecules as observed with recombinant Bax in liposomes...
  74. pmc Cyclosporine A attenuates mitochondrial permeability transition and improves mitochondrial respiratory function in cardiomyocytes isolated from dogs with heart failure
    Victor G Sharov
    Department of Medicine, Division of Cardiovascular Medicine, Henry Ford Heart and Vascular Institute, Detroit, Michigan 48202, USA
    J Mol Cell Cardiol 42:150-8. 2007
    ..Attenuation of calcein exit, DeltaPsi(m) and improvement of state-3 respiration achieved with CsA (0.2 microM) show that permeability transition opening could be a cause of mitochondrial dysfunction described in the failing heart...
  75. pmc Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol
    Christine Klöppel
    Cell Biology and University of Kaiserslautern, 67663 Kaiserslautern, Germany
    Mol Biol Cell 22:3749-57. 2011
    ..We propose a model in which the Mia40-mediated oxidative folding of domain I controls the cellular distribution of Ccs1 and, consequently, active Sod1...
  76. pmc Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues
    A Del Arco
    Departamento de Biologia Molecular, Centro de Biologia Molecular Severo Ochoa, Campus de Cantoblanco, 28049 Madrid, Spain
    Biochem J 345:725-32. 2000
    ..Thus Aralar proteins are probably expressed in all tissues in an isoform-specific fashion, where they function as calcium-regulated metabolite (possibly anionic) carriers...
  77. pmc MICU1 is an essential gatekeeper for MCU-mediated mitochondrial Ca(2+) uptake that regulates cell survival
    Karthik Mallilankaraman
    Department of Biochemistry, Temple University, Philadelphia, PA 19140, USA
    Cell 151:630-44. 2012
    ..Thus, MICU1 is a gatekeeper of MCU-mediated Ca(2+)(m) uptake that is essential to prevent [Ca(2+)](m) overload and associated stress...
  78. pmc Properties of Ca(2+) transport in mitochondria of Drosophila melanogaster
    Sophia von Stockum
    Consiglio Nazionale delle Ricerche Institute of Neuroscience and Department of Biomedical Sciences, University of I 35121 Padova, Italy
    J Biol Chem 286:41163-70. 2011
    ..We conclude that Drosophila mitochondria possess a selective Ca(2+) release channel with features intermediate between the PTP of yeast and mammals...
  79. 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...
  80. ncbi Targeting post-mitochondrial effectors of apoptosis for neuroprotection
    Lorenzo Galluzzi
    INSERM, U848, 94805 Villejuif, France
    Biochim Biophys Acta 1787:402-13. 2009
    ..Our review recapitulates the results of these studies and proposes novel strategies of inhibiting post-mitochondrial apoptosis in neurons...
  81. pmc Measuring mitochondrial function in intact cardiac myocytes
    Elena N Dedkova
    Department of Molecular Biophysics and Physiology, Rush University Medical Center, Chicago, IL 60612, USA
    J Mol Cell Cardiol 52:48-61. 2012
    ..Where appropriate we complement this review on intact myocytes with seminal studies that were performed on isolated mitochondria, permeabilized cells, and in whole hearts...
  82. ncbi Modulation of the mitochondrial permeability transition pore complex in GSK-3beta-mediated myocardial protection
    Masahiro Nishihara
    Second Department of Internal Medicine, Sapporo Medical University School of Medicine, S 1 W 16, Chuo Ku, Sapporo 060 8543, Japan
    J Mol Cell Cardiol 43:564-70. 2007
    ..These results suggest that reduction in affinity of ANT to cyclophilin D by increased phospho-GSK-3beta binding to ANT may be responsible for suppression of mPTP opening and myocardial protection afforded by IPC+EPO...
  83. ncbi Inhibition of the mitochondrial permeability transition by the nonimmunosuppressive cyclosporin derivative NIM811
    Peter C Waldmeier
    Nervous System Research, Novartis Pharma Ltd, Basel, Switzerland
    Mol Pharmacol 62:22-9. 2002
    ..We conclude that NIM811 is a useful alternative to PKF220-384 to investigate the role of the mitochondrial permeability transition in apoptotic and necrotic cell death...
  84. ncbi Hyperglycaemia blocks sevoflurane-induced postconditioning in the rat heart in vivo: cardioprotection can be restored by blocking the mitochondrial permeability transition pore
    R Huhn
    Laboratory of Experimental Intensive Care and Anaesthesiology, Academic Medical Center, Department of Anaesthesiology, University of Amsterdam, Meibergdreef 9, 1100 DD Amsterdam, The Netherlands
    Br J Anaesth 100:465-71. 2008
    ..We investigated whether sevoflurane (Sevo)-induced postconditioning is blocked by HG and whether the blockade could be reversed by inhibiting the mitochondrial permeability transition pore (mPTP) with cyclosporine A (CsA)...
  85. pmc The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice
    Lee J Martin
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205 2196, USA
    Exp Neurol 218:333-46. 2009
    ..These results demonstrate that mitochondria have causal roles in the disease mechanisms in MNs in ALS mice. This work defines a new mitochondrial mechanism for MN degeneration in ALS...
  86. pmc The mitochondrial permeability transition pore and ischemia-reperfusion injury
    Christopher P Baines
    Department of Biomedical Sciences, Dalton Cardiovascular Research Center, University of Missouri Columbia, Columbia, MO 65211, USA
    Basic Res Cardiol 104:181-8. 2009
    ..The purpose of this review is to focus on the role of the MPT pore in ischemia-reperfusion injury, the mechanisms involved, and, in particular, what we do and do not know regarding the pore's molecular composition...
  87. ncbi Inhibition of mitochondrial permeability transition pore opening: translation to patients
    Ludovic Gomez
    Laboratoire de Physiologie Lyon Nord, INSERM U 886, 8, avenue Rockefeller, 69373 Lyon, France
    Cardiovasc Res 83:226-33. 2009
    ..Large-scale trials are ongoing to address whether these new treatments may improve clinical outcome in reperfused AMI patients...
  88. pmc The mitochondrial permeability transition from yeast to mammals
    Luca Azzolin
    Department of Biomedical Sciences, CNR Institute of Neuroscience, University of Padova, Padova, Italy
    FEBS Lett 584:2504-9. 2010
    ..Biochim. Biophys. Acta 1787, 1486-1491] suggest that the mitochondrial permeability transition has been conserved during evolution...
  89. pmc Calcium elevation in mitochondria is the main Ca2+ requirement for mitochondrial permeability transition pore (mPTP) opening
    Heidi K Baumgartner
    Physiological Laboratory, School of Biomedical Sciences, Liverpool University, Liverpool L69 3BX, United Kingdom
    J Biol Chem 284:20796-803. 2009
    ..We conclude that elevated Ca2+ in mitochondria is the crucial factor in determining whether cells undergo oxidative stress-induced apoptosis...
  90. ncbi Mitochondrial oxidative stress and cell death in astrocytes--requirement for stored Ca2+ and sustained opening of the permeability transition pore
    Jake Jacobson
    Department of Physiology, University College London, London, WC1E 6BT, UK
    J Cell Sci 115:1175-88. 2002
    ..The amplification of oxidative stress and Ca2+ loading culminates in opening of the mPTP and necrotic cell death in primary brain cells...
  91. ncbi The integration of mitochondrial calcium transport and storage
    David G Nicholls
    Buck Institute for Age Research, Novato, California 94945, USA
    J Bioenerg Biomembr 36:277-81. 2004
    ..Taking this into account we may now finally have a model that explains the major features of physiological mitochondrial Ca(2+) transport...
  92. pmc Fluctuations in mitochondrial membrane potential caused by repetitive gating of the permeability transition pore
    J Huser
    Department of Physiology, Stritch School of Medicine, Loyola University Chicago, 2160 S First Avenue, Maywood, IL 60153, USA
    Biochem J 343:311-7. 1999
    ..In contrast, loss of matrix metabolites, swelling and other structural damage of the organelle render prolonged openings of the transition pore deleterious to mitochondria and to the cell...
  93. ncbi A CaPful of mechanisms regulating the mitochondrial permeability transition
    Fabio Di Lisa
    Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Padova, Italy
    J Mol Cell Cardiol 46:775-80. 2009
    ....
  94. ncbi Mechanistically distinct steps in the mitochondrial death pathway triggered by oxidative stress in cardiac myocytes
    Masaharu Akao
    Institute of Molecular Cardiobiology, The Johns Hopkins University, Baltimore, MD 21205, USA
    Circ Res 92:186-94. 2003
    ..The priming phase, during which mitochondria undergo major ultrastructural alterations but remain functional, represents a particularly attractive target for intervention in the prevention of cell death...
  95. pmc The mitochondrial import protein Mim1 promotes biogenesis of multispanning outer membrane proteins
    Thomas Becker
    Institute for Biochemistry and Molecular Biology, Centre for Biochemistry and Molecular Cell Research, Freiburg, Germany
    J Cell Biol 194:387-95. 2011
    ..We conclude that the Mim1 complex plays a central role in the import of α-helical outer membrane proteins with multiple transmembrane segments...
  96. pmc Dual role of the receptor Tom20 in specificity and efficiency of protein import into mitochondria
    Hayashi Yamamoto
    Department of Chemistry, Graduate School of Science, Nagoya University, Chikusa ku, Nagoya 464 8602, Japan
    Proc Natl Acad Sci U S A 108:91-6. 2011
    ..Therefore Tom20 has a dual role in protein import into mitochondria: recognition of the targeting signal in the presequence and tethering the presequence to the TOM40 complex to increase import efficiency...
  97. pmc Leptin-induced cardioprotection involves JAK/STAT signaling that may be linked to the mitochondrial permeability transition pore
    Christopher C T Smith
    The Hatter Cardiovascular Institute, University College London and Hospital Medical School, London, United Kingdom
    Am J Physiol Heart Circ Physiol 299:H1265-70. 2010
    ..Further research into the interactions between these two signaling pathways in the setting of I/R injury is, however, required...
  98. pmc Reversible cyclosporin A-sensitive mitochondrial depolarization occurs within minutes of stroke onset in mouse somatosensory cortex in vivo: a two-photon imaging study
    Ran R Liu
    Department of Psychiatry, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada
    J Biol Chem 284:36109-17. 2009
    ..Our findings using in vivo imaging suggest that mitochondrial dysfunction and specifically the activation of the mPTP are early reversible events during brain ischemia that could trigger delayed cell death...
  99. pmc Mitochondrial cyclophilin-D as a critical mediator of ischaemic preconditioning
    Derek J Hausenloy
    The Hatter Cardiovascular Institute, University College London Hospital and Medical School, London WC1E 6HX, UK
    Cardiovasc Res 88:67-74. 2010
    ..The aim of the present study is to determine whether the cyclophilin-D (CYPD) component of the mPTP is required by IPC to generate mitochondrial ROS and subsequently activate Akt and Erk1/2...
  100. pmc Evolutionary diversity of the mitochondrial calcium uniporter
    Alexander G Bick
    Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA
    Science 336:886. 2012
    ..Several bacterial genomes also contain putative MCU homologs that may represent prokaryotic calcium channels. The analyses indicate that the uniporter may have been an early feature of mitochondria...
  101. pmc Roles of Tom70 in import of presequence-containing mitochondrial proteins
    Hayashi Yamamoto
    Department of Chemistry, Graduate School of Science, Japan Science and Technology Agency, Research Centre for Material Science, Nagoya University, Chikusa ku, Nagoya 464 8602, Japan
    J Biol Chem 284:31635-46. 2009
    ..Therefore Tom70 plays the role of a docking site for not only cytosolic chaperones but also aggregate-prone substrates to maintain their solubility for efficient transfer to downstream components of the mitochondrial import machineries...

Research Grants63

  1. DNA Damage/Repair and Cell Death
    Lee Martin; Fiscal Year: 2009
    ....
  2. Mechanisms of Motor Neuron Death
    Lee Martin; Fiscal Year: 2006
    ..abstract_text> ..
  3. MECHANISMS OF NEURONAL APOPTOSIS IN VIVO
    Lee Martin; Fiscal Year: 2009
    ..This work can define a new mitochondrial mechanism for target deprivation-induced neurodegeneration and can improve the understanding of the cellular and molecular mechanisms of neuronal apoptosis in vivo ..
  4. PHOSPHOLIPASE C AND CARDIAC HYPERTROPHY
    GERALD DORN; Fiscal Year: 2005
    ..abstract_text> ..
  5. Calpains as Mediators of Cardiac Ischemic Injury
    Gerald W Dorn; Fiscal Year: 2010
    ..Collectively, these studies will apply state-of-the-art techniques for in vivo genetic manipulation, microphysiologic analysis, and biochemical assessment to achieve insight into the roles of myocardial calpains in health and disease. ..
  6. Effect of Drugs on Myocardial Hypoxia
    Garrett Gross; Fiscal Year: 2009
    ..Information gained from these studies will hopefully lead to new therapies to treat patients with ischemic heart disease. ..
  7. PHOSPHATE TRANSPORT PATHS WITHIN HOMODIMERIC PTP
    Hartmut Wohlrab; Fiscal Year: 2001
    ..The structure will permit the accurate localization of the functionally relevant residues and regions of PTP and should lead to a good understanding of transmembrane metabolite transport. ..
  8. CATECHOLS AND ADENOSINE IN MYOCARDIAL PRECONDITIONING
    Michael Cohen; Fiscal Year: 2006
    ..Finally, I will see if NECA's salvage of myocardium at reperfusion translates into rapid functional benefits. It is hoped that the potential of this new pharmacologic approach can be defined. ..
  9. Mitochondrial control of neuronal excitotoxicity
    David Nicholls; Fiscal Year: 2005
    ..4. To test the hypothesis that mitochondria at specific intracellular locations may be selectively vulnerable to glutamate excitotoxicity because of their proximity to NMDA receptors, or their location in regions of high-energy demand. ..
  10. Aging-Related Models and Mitochondrial Bioenergetics
    David Nicholls; Fiscal Year: 2006
    ..3. To indicate therapeutic strategies whereby the deleterious consequences of aging-related changes in mitochondrial function can be minimized. ..
  11. Tissue Factor in Myocardial Reperfusion
    Jakob Vinten Johansen; Fiscal Year: 2006
    ..abstract_text> ..
  12. Mitochondrial Role in Manganese Toxicity
    Thomas Gunter; Fiscal Year: 2010
    ....
  13. Nitric oxide and mitochondrial biogenesis in sepsis
    Claude A Piantadosi; Fiscal Year: 2010
    ..abstract_text> ..
  14. Lung Injury Protection by Coagulation Blockade
    CLAUDE PIANTADOSI; Fiscal Year: 2008
    ..A successfully optimized strategy could greatly attenuate persistence of pulmonary inflammation and facilitate the resolution of human ARDS. ..
  15. CARP, A Novel Cell Death Regulator
    Wafik El Deiry; Fiscal Year: 2006
    ..Specific Aim 3: To study the functional consequences of perturbing or blocking CARP expression. These studies should provide novel insights into the cellular control of the extrinsic pathway of apoptosis. ..
  16. BRAIN TO MIDDLE SCHOOLS
    JANET DUBINSKY; Fiscal Year: 2006
    ..The two cohorts of teachers to be trained will reach a total of 22,500 students over the life of the grant. ..
  17. Gs signaling in synaptic development and function
    Michael Forte; Fiscal Year: 2006
    ..Thus, the goal of this aim is to identify participants in the Gsa-activated pathway operating specifically during NMJ formation through the use of the genetic interaction strategies possible in Drosophila. ..
  18. Il-6 type cytokine protection from hyperoxic lung injury
    Aaron Waxman; Fiscal Year: 2007
    ..abstract_text> ..
  19. Cardiolipin remodeling and its role in mitochondrial function in Barth Syndrome
    Carla Koehler; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  20. LIVER PRESERVATION FOR TRANSPLANTATION
    John Lemasters; Fiscal Year: 2007
    ..Further, the investigators will validate their findings in a clinically relevant model of rat liver transplantation. ..
  21. Mechanisms of In Vivo Ethanol-Induced Mitochondrial Depolarization
    Zhi Zhong; Fiscal Year: 2010
    ....
  22. Molecular Targeting of the Survivin Pathway in Cancer
    Dario C Altieri; Fiscal Year: 2010
    ..This work will provide a mechanistic foundation for the validation and further development of molecular antagonists of the survivin pathway for novel cancer therapeutics in humans. ..
  23. THE ROLE OF MITOCHONDRIAL CHANNELS IN CELL DEATH
    Kathleen W Kinnally; Fiscal Year: 2010
    ..These experiments may identify novel therapeutic targets, e.g., MAC, to modulate apoptosis in cancer and other pathologies, e.g., myocardial infarction. ..
  24. Inhibition of Alzheimer's Beta-Amyloid Fibril Formation
    Duy Hua; Fiscal Year: 2009
    ..However, soluble oligomeric Abeta showed high neuronal toxicity. Inhibition of the formation of these toxic soluble Abeta oligomers would provide therapeutics for AD. ..
  25. Release of Apoptogenic Proteins from Brain Mitochondria
    Nickolay Brustovetsky; Fiscal Year: 2009
    ..abstract_text> ..
  26. Evaluation of resveratrol and curcumin as therapeutics against high-risk leukemia
    SUSAN ZUNINO; Fiscal Year: 2008
    ..These studies will provide information about whether the plant antioxidants resveratrol and curcumin may be novel and efficient alternatives for the treatment of high-risk leukemia. [unreadable] [unreadable] [unreadable]..
  27. Multi-user fermentation facility upgrade
    Carla Koehler; Fiscal Year: 2008
    ..The fermentation system will give the NIH-funded researchers at UCLA the ability to effectively conduct these types of experiments that impact many aspects of health and well-being. [unreadable] [unreadable] [unreadable]..
  28. 2007 Protein Transport Across Membranes Gordon Conference
    Carla Koehler; Fiscal Year: 2007
    ..As a result, this conference leads to an increased understanding in the cause of disease and potential therapeutic approaches. [unreadable] [unreadable] [unreadable] [unreadable]..
  29. Characterization of Sleep Mutants of Drososphila
    Chiara Cirelli; Fiscal Year: 2009
    ..This research will help to identify the molecular mechanisms regulating the need for sleep and provide novel clues to its functions. ..
  30. Anesthetics, GABA and the Injured Brain
    David Warner; Fiscal Year: 2006
    ..We believe that GABAAergic pharmacologic properties of volatile anesthetics known to be critical for anesthesia are the same properties that confer cerebral protection. ..
  31. PHARMACOKINETICS AND FETAL EXPOSURE TO NARCOTICS
    Hazel Szeto; Fiscal Year: 2002
    ..The proposed studies will provide new information on the functional roles of opiate receptor subtypes in the fetus, and should be helpful in the design of better analgesics for perinatal use. ..
  32. STRUCTURE/FUNCTION ANALYSIS OF MOLECULAR CHAPERONES
    Celia Harrison; Fiscal Year: 2002
    ..The long-term goals of this proposal ar to understand the function of nucleotide exchange factors in a structural context, and to further structural knowledge of the molecular chaperones. ..
  33. STRIATAL ENERGETICS AND HUNTINGTON'S DISEASE
    JANET DUBINSKY; Fiscal Year: 2003
    ....
  34. ANALYSIS OF THE BCL2 FAMILY IN YEAST
    Michael Forte; Fiscal Year: 2003
    ..We propose to use genetic strategies to identify the molecular components required for BAX mediated growth arrest, and subsequently, use these mutants to address the question of whether BAX generated cell killing requires growth arrest. ..
  35. CYCLIC AMP/ADENOSINE PATHWAY
    Edwin Jackson; Fiscal Year: 2002
    ..abstract_text> ..
  36. ALTERATIONS IN THE DYAD CLEFT IN FAILING CARDIOMYOCYTES
    ROBERT HAWORTH; Fiscal Year: 2002
    ..End of Abstract) ..
  37. APOPTOSIS OF CARDIAC MUSCLE AS A TARGET FOR GENE THERAPY
    Michael Schneider; Fiscal Year: 2002
    ....
  38. ANKYRIN: CRUCIAL LINKER IN RENAL CELL APOPTOTIC CASCADES
    Prasad Devarajan; Fiscal Year: 2003
    ..The results will provide important insights into apoptotic cell death that accompanies ischemia and several other renal pathophysiologic states, and reveal critical clues for potential therapeutic interventions. ..
  39. EXTRACELLULAR OXYGEN RADICALS AND ISCHEMIC BRAIN INJURY
    David Warner; Fiscal Year: 2002
    ....
  40. Structure-Function Study of Angiogenic Protein,Ephrin B2
    WALTER STAFFORD; Fiscal Year: 2004
    ..Finally, we propose to investigate the relationship between structure and function of the entire Eph receptor ectodomains via crystallography, to ascertain the function of the non-ligand binding domains. ..
  41. Rare Liver Disease Network
    Ronald Sokol; Fiscal Year: 2005
    ..The RLDN will be a full partner in the Rare Diseases Clinical Research Network and will participate collaboratively with the other Clinical Research Centers and the Data and Technology Coordinating Center. ..
  42. NGAL: A novel early biomarker of acute renal injury
    Prasad Devarajan; Fiscal Year: 2005
    ..The long-term goal is to identify novel biomarkers for the early diagnosis of human ARF, to facilitate the early institution of therapy ..
  43. Protective Cognitive Phenotypes in Costa Rica
    Jeremy Silverman; Fiscal Year: 2005
    ..Ultimately, a greatly enlarged longitudinal/genetic study using this population will identify new phenotypes for molecular genetic studies aimed at identifying genes with genetic protective variants. ..
  44. PROTEIN AND NUCLEIC ACID DETECTION/QUANTITATION SYSTEM
    Carla Koehler; Fiscal Year: 2001
    ..By enhancing the effectiveness of the research efforts of these individual projects, this application users will find this instrumentation valuable for their research. ..
  45. APOLIPOPROTEIN E AND THE ISCHEMIC BRAIN
    David Warner; Fiscal Year: 2001
    ..We believe that this information would be of substantial value in defining both the relevance of Apo E mechanisms to ischemic outcome and provide a basis for therapeutic approaches to the treatment of stroke. ..
  46. CALCIUM AND THE PATHOPHYSIOLOGY OF THE ADULT HEART CELL
    ROBERT HAWORTH; Fiscal Year: 2001
    ....
  47. PEROXYNITRITE IN ACETAMINOPHEN INDUCED HEPATOTOXICITY
    JACK HINSON; Fiscal Year: 2002
    ..By understanding the role of peroxynitrite in acetaminophen hepatotoxicity, new treatment paradigms may be developed for hepatotoxicity, and this mechanism may be important with other toxins. ..
  48. Effect of Age on Exercise-Induced Cardioprotection
    JOSEPH STARNES; Fiscal Year: 2003
    ..The results of this Aim will provide key direction for the effective design of further comprehensive studies using inhibitors and/or activators of specific proteins or signaling pathways. ..
  49. Conference on Mitochondria and Pathogenesis
    John Lemasters; Fiscal Year: 2002
    ..The purpose of this conference is to bring together leading researchers studying mitochondrial dysfunction in many different disease settings to share ideas and recent research findings. ..
  50. The Role of Mitochondrial DNA Alterations in Cancer
    Lee Jun Wong; Fiscal Year: 2008
    ..Results from this research project will help us understand the functional role of mitochondrial DNA alterations in cancer and identify potential novel targets for more effective therapeutic development. ..
  51. In Vivo Delivery of Nucleic Acids by Anionic Lipsomes
    JANET DUBINSKY; Fiscal Year: 2003
    ....
  52. Mitoprotective and antioxidant peptides for DM treatment
    Hazel Szeto; Fiscal Year: 2006
    ..abstract_text> ..
  53. Molecular Pathogenesis of Apoptosis in Liver Cells
    John Lemasters; Fiscal Year: 2005
    ..abstract_text> ..
  54. Mitochondrial biogenesis & neurodegenerative disorders
    Carla Koehler; Fiscal Year: 2005
    ..Also, these studies will develop new animal models that will potentially play a role in understanding the molecular basis of other mitochondrial diseases such as mitochondrial myopathies and neuropathies. ..
  55. Small Molecule Modulators for Redox Regulation in the Mitochondrial Intermembrane
    Carla Koehler; Fiscal Year: 2008
    ..Long-term, this project may lead to the development of therapeutics that modulate mitochondrial function in these diseases. [unreadable] [unreadable] [unreadable]..
  56. Proteomic analyses of Drosophila Parkinson's disease model
    Leo Pallanck; Fiscal Year: 2007
    ..This work should clarify the biological role of Parkin and will serve as a foundation for future hypothesis-driven investigation of parkin pathogenesis. [unreadable] [unreadable]..
  57. Factor B and energy coupling in mitochondria
    GRIGORY BELOGRUDOV; Fiscal Year: 2007
    ..The proposed research is expected to advance the knowledge of the molecular mechanisms of energy transfer and conservation in mitochondria, and shed light on the role of mitochondria in a number of human disorders. ..
  58. Cyclin-dependent Kinases and Cardiac Growth
    Michael Schneider; Fiscal Year: 2007
    ..3) To test the prediction that hypertrophic signals, through cyclin T-Cdk9, induce promoter escape by RNAPII. ..
  59. Network for Translational Research: Optical Imaging
    Wafik El Deiry; Fiscal Year: 2007
    ..abstract_text> ..
  60. Multi-Well Extracellular Flux Analyzer
    John Lemasters; Fiscal Year: 2008
    ..The results of those studies could lead to the development of new therapeutic treatments for some of these diseases. [unreadable] [unreadable] [unreadable]..
  61. MOLECULAR GENETICS OF SCHIZOPHRENIA
    Jeremy Silverman; Fiscal Year: 2006
    ..The goal of the study is to identify one or more SZ susceptibility genes whose subsequent characterization inmolecular and functional studies will elucidate the pathophysiology and treatment of SZ. ..
  62. KILLER/DR5 AND TUMORIGENESIS
    Wafik El Deiry; Fiscal Year: 2001
    ..These experiments could lead to novel strategies for anti-cancer therapy. ..
  63. NYU Short Term Research Training-Minority Dental Student
    Kathleen Kinnally; Fiscal Year: 2007
    ..Fulfillment of these aims will facilitate attaining racial/ethnic parity in the profession of dentistry, and specifically within the subcommunity of academic/research dentists. ..