About mito-disease.info

Mito-disease.info is the reference portal for information on mitochondrial diseases (MITO diseases) and orphan drugs, for all stakeholders. The aim of Mito-disease.info is to help improve the diagnosis, care and treatment of patients with mitochondrial diseases.

This portal site is organized by KOINOBORI Associate Inc.

Contact information: an inquiry email address

Latest Science Article about MITO diseases

Mito-disease.info introduces the latest science articles about MITO diseases. If you would need to check it/them more in detail, you can contact to the journal individually.

Article Summary
Redox Biol. 2014 Jan 10;2:206-10.A review of the mitochondrial and glycolytic metabolism in human platelets and leukocytes: Implications for their use as bioenergetic biomarkers.Kramer PA, Ravi S, Chacko B, Johnson MS, Darley-Usmar VM.Department of Pathology, UAB Mitochondrial Medicine Laboratory, Center for Free Radical Biology, University of Alabama at Birmingham, Birmingham, AL, USA. This opens up the possibility that circulating platelets and leukocytes can sense metabolic stress in patients and serve as biomarkers of mitochondrial dysfunction in human pathologies.
Sci Transl Med. 2014 Jan 29;6(221).Antioxidants accelerate lung cancer progression in mice.Sayin VI, Ibrahim MX, Larsson E, Nilsson JA, Lindahl P, Bergo MO.Sahlgrenska Cancer Center, Department of Molecular and Clinical Medicine, Institute of Medicine, University of Gothenburg, SE-405 30 Gothenburg, Sweden. NAC and vitamin E increase tumor cell proliferation by reducing ROS, DNA damage, and p53 expression in mouse and human lung tumor cells. Inactivation of p53 increases tumor growth to a similar degree as antioxidants and abolishes the antioxidant effect. Thus, antioxidants accelerate tumor growth by disrupting the ROS-p53 axis.
Proc Natl Acad Sci U S A. 2014 Jan 21;111(3):960-5. UCP2 transports C4 metabolites out of mitochondria, regulating glucose and glutamine oxidation.Vozza A, Parisi G, De Leonardis F, Lasorsa FM, Castegna A, Amorese D, Marmo R, Calcagnile VM, Palmieri L, Ricquier D, Paradies E, Scarcia P, Palmieri F, Bouillaud F, Fiermonte G.Department of Biosciences, Biotechnologies, and Biopharmaceutics and Center of Excellence in Comparative Genomics, University of Bari, 70125 Bari, Italy. This treatise show that UCP2 catalyzes an exchange of malate, oxaloacetate, and aspartate for phosphate, and that it exports C4 metabolites from mitochondria to the cytosol in vivo. Our findings also provide evidence that UCP2 activity limits mitochondrial oxidation of glucose and enhances glutaminolysis.
PLoS One. 2014 Jan.Improved metabolic health alters host metabolism in parallel with changes in systemic xeno-metabolites of gut origin.Campbell C, Grapov D, Fiehn O, Chandler CJ, Burnett DJ, Souza EC, Casazza GA, Gustafson MB, Keim NL, Newman JW, Hunter GR, Fernandez JR, Garvey WT, Harper ME, Hoppel CL, Meissen JK, Take K, Adams SH.Genome Center, University of California Davis, Davis, California, USA. The current study assessed, for the first time, changes in post-OGTT comprehensive metabolite patterns following a diet and fitness intervention that markedly improved metabolic health indices (weight loss, increased fitness, and improved insulin sensitivity). Shifts in plasma α-ketoglutarate, UDP-glucuronic acid, malate and pyruvate suggestive of changes in mitochondrial function and glucose oxidative and non-oxidative metabolisms;
Proc Natl Acad Sci U S A. 2014 Feb 25;111(8):3116-21.Inhibition of p53 preserves Parkin-mediated mitophagy and pancreatic β-cell function in diabetes.Hoshino A, Ariyoshi M, Okawa Y, Kaimoto S, Uchihashi M, Fukai K, Iwai-Kanai E, Ikeda K, Ueyama T, Ogata T, Matoba S.Department of Cardiovascular Medicine, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566 Japan. This treatise indicated that cytosolic p53 inhibited Parkin-mediated autophagic degradation of damaged mitochondria and promoted mitochondrial compromise, leading to impaired insulin secretion in the β-cells of diabetic mice.
J Natl Cancer Inst. 2014 Mar;106(3).Baseline selenium status and effects of selenium and vitamin e supplementation on prostate cancer risk.Kristal AR, Darke AK, Morris JS, Tangen CM, Goodman PJ, Thompson IM, Meyskens FL Jr, Goodman GE, Minasian LM, Parnes HL, Lippman SM, Klein EA.Cancer Prevention Program, Fred Hutchinson Cancer Research Center, 1100 Fairview Ave N, M4-B402, Seattle,WA 98122 Selenium supplementation did not benefit men with low selenium status but increased the risk of high-grade PCa among men with high selenium status. Vitamin E increased the risk of PCa among men with low selenium status. Men should avoid selenium or vitamin E supplementation at doses that exceed recommended dietary intakes.
PLoS Genet. 2014 May 15;10(5).G×G×E for lifespan in Drosophila: mitochondrial, nuclear, and dietary interactions that modify longevity.Zhu CT, Ingelmo P, Rand DM.Department of Ecology and Evolutionary Biology, Brown University, Providence, Rhode Island, USA. This treatise indicated that mitochondrial genotypes can have significant effects on longevity and the diet restriction response but these effects are highly dependent on nuclear genetic background and the specific diet environment.
PLoS Genet. 2014 May 29;10(5).PINK1-Parkin pathway activity is regulated by degradation of PINK1 in the mitochondrial matrix.Thomas RE, Andrews LA, Burman JL, Lin WY, Pallanck LJ.Department of Biology, University of Washington, Seattle, Washington, USA. This treatise suggest that Lon plays an essential role in regulating the PINK1-Parkin pathway by promoting the degradation of PINK1 in the matrix of healthy mitochondria.
Cell Mol Life Sci. 2014 May 28.Biosynthesis and roles of phospholipids in mitochondrial fusion, division and mitophagy.Zhang Q, Tamura Y, Roy M, Adachi Y, Iijima M, Sesaki H.Department of Cell Biology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Three conserved mitochondrial dynamin-related GTPases (i.e., mitofusin, Opa1 and Drp1) mediate mitochondrial fusion and division. In addition to dynamins, recent studies demonstrated that phospholipids in mitochondria also play key roles in mitochondrial dynamics by interacting with dynamin GTPases and by directly changing the biophysical properties of the mitochondrial membranes.
Cell Metab. 2014 May 6;19(5):757-66.Mitohormesis.Yun J, Finkel T.Center for Molecular Medicine, National Heart, Lung and Blood Institute, NIH, Bethesda, MD 20892, USA. This view has been largely supplanted by the concept that mitochondria are fully integrated into the cell and that mitochondrial stresses rapidly activate cytosolic signaling pathways that ultimately alter nuclear gene expression. Remarkably, this coordinated response to mild mitochondrial stress appears to leave the cell less susceptible to subsequent perturbations.
Curr Biol. 2014 May 19;24(10).ROS function in redox signaling and oxidative stress.Schieber M, Chandel NS.Division of Pulmonary and Critical Care Medicine, Department of Medicine, The Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA. In this review we discuss the two faces of ROS -redox biology and oxidative stress- and their contribution to both physiological and pathological conditions. Redox biology involves a small increase in ROS levels that acti- vates signaling pathways to initiate biological processes, while oxidative stress denotes high levels of ROS that result in damage to DNA, protein or lipids.
Cell Metab. 2014 Apr 1;19(4):642-52.Pathogenesis of human mitochondrial diseases is modulated by reduced activity of the ubiquitin/proteasome system.Segref A, Kevei _, Pokrzywa W, Schmeisser K, Mansfeld J, Livnat-Levanon N, Ensenauer R, Glickman MH, Ristow M, Hoppe T.Institute for Genetics and Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Z_lpicher Strasse 47a, 50674 Cologne, Germany. This treatise discovered a conserved influence of respiration defects and mitochondrial stress conditions on the efficiency of ubiquitin-dependent degradation pathways in the cytosol both in C. elegans and humans.
EMBO Mol Med. 2014 Apr 6;6(6):721-31.Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3.Khan NA, Auranen M, Paetau I, Pirinen E, Euro L, Forsstr_m S, Pasila L, Velagapudi V, Carroll CJ, Auwerx J, Suomalainen A.Molecular Neurology, Research Programs Unit, University of Helsinki, Department of Neurology, Helsinki University Central Hospital, Neuroscience Research Centre University of Helsinki, Helsinki, Finland. This treatised demonstrated that oral supplementation with NR, a vitamin B3 form and NAD+ precursor, efficiently prevented development and progression of mitochondrial myopathy in mice.
J Neurosci. 2014 Apr 23;34(17):5800-15.Expression of Nampt in hippocampal and cortical excitatory neurons is critical for cognitive function.Stein LR, Wozniak DF, Dearborn JT, Kubota S, Apte RS, Izumi Y, Zorumski CF, Imai S.Department of Developmental Biology, Department of Psychiatry, The Taylor Family Institute for Innovative Psychiatric Research, Department of Ophthalmology, and Department of Anatomy and Neurobiology, Washington University School of Medicine, St. Louis, Missouri 63110. Intracellular Nampt-mediated NAD+ biosynthesisis the primary source of NAD+ for forebrain excitatory neurons and is critical for neuronal function and survival.
Chem Phys Lipids. 2014 Apr;179:64-9. Cardiolipin asymmetry, oxidation and signaling.Kagan VE, Chu CT, Tyurina YY, Cheikhi A, Bayir H.Center for Free Radical and Antioxidant Health, University of Pittsburgh, Pittsburgh, PA 15219, USA; Department of Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA 15219, USA; Safar Center for Resuscitation Research, University of Pittsburgh, Pittsburgh, PA 15219, USA. In this review, we describe CL-based signaling mitochondrial pathways realized via modulation of trans-membrane asymmetry and leading to externalization and peroxidation of CLs in mitophagy and apoptosis, respectively.
Nat Med. 2014 Apr;20(4):415-8.Plasma phospholipids identify antecedent memory impairment in older adults.Mapstone M, Cheema AK, Fiandaca MS, Zhong X, Mhyre TR, MacArthur LH, Hall WJ, Fisher SG, Peterson DR, Haley JM, Nazar MD, Rich SA, Berlau DJ, Peltz CB, Tan MT, Kawas CH, Federoff HJ.Department of Neurobiology and Behavior, University of California, Irvine School of Medicine, Irvine, California, USA. This treatise discovered and validated a set of ten lipids from peripheral blood that predicted phenoconversion to either amnestic mild cognitive impairment or Alzheimer’s disease within a 2_3 year timeframe with over 90% accuracy.
Mol Genet Metab. 2014 Jun 30. Mitochondrial myopathy, lactic acidosis, and sideroblastic anemia (MLASA) plus associated with a novel de novo mutation (m.8969G>A) in the mitochondrial encoded ATP6 gene.Burrage LC, Tang S, Wang J, Donti TR, Walkiewicz M, Luchak JM, Chen LC, Schmitt ES, Niu Z, Erana R, Hunter JV, Graham BH, Wong LJ, Scaglia F.Department of Molecular and Human Genetics, Baylor College of Medicine, Houston,TX, USA. This case report describe a 6-year old male with an MLASA plus phenotype. Sequencing of the mitochondrial genome identified a novel de novo, heteroplasmic mutation in the mitochondrial DNA (mtDNA) encoded ATP6 gene (m.8969G>A), this mutation in MT-ATP6 may represent the first mtDNA point mutation associated with the MLASA phenotype.
Traffic. 2014 Jun 20.Phospholipid Transport via Mitochondria.Tamura Y, Sesaki H, Endo T.Research Center for Materials Science, Nagoya University, Nagoya, 464-8602, Japan. Recent studies using yeast as a model system began to provide intriguing insights into phospholipid exchange between the ER and mitochondria as well as between the mitochondrial OM and IM.
Hum Genet. 2014 Jun 6.Mitochondrial DNA copy number in peripheral blood cells declines with age and is associated with general health among elderly.Mengel-From J, Thinggaard M, Dalga_rd C, Kyvik KO, Christensen K, Christiansen L.Epidemiology, Biostatistics and Biodemography Unit, The Danish Aging Research Center, The Danish Twin Registry, Institute of Public Health, University of Southern Denmark, J.B. Winsl_ws Vej 9, 5000, Odense, Denmark. This study suggests that high mitochondrial DNA copy number in blood is associated with better health and survival among elderly.
PLoS Genet. 2014 Jun 19;10(6)4.Loss of UCP2 attenuates mitochondrial dysfunction without altering ROS production and uncoupling activity.Kukat A, Dogan SA, Edgar D, Mourier A, Jacoby C, Maiti P, Mauer J, Becker C, Senft K, Wibom R, Kudin AP, Hultenby K, Fl_gel U, Rosenkranz S, Ricquier D, Kunz WS, Trifunovic A.Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD) and Institute for Mitochondrial Diseases and Aging, Medical Faculty, University of Cologne, Cologne, Germany. This treatise demonstrate that the presence of UCP2 wields beneficial effect on respiratory deficient mitochondria without affecting ROS production or uncoupling. Instead, UCP2 protein seems to mediate a valuable upregulation of fatty acid metabolism detected in mtDNA mutator hearts.
Biomed Res Int. 2014 Jun 9.Essential Amino Acids and Exercise Tolerance in Elderly Muscle-Depleted Subjects with Chronic Diseases: A Rehabilitation without Rehabilitation?Aquilani R, D'Antona G, Baiardi P, Gambino A, Iadarola P, Viglio S, Pasini E, Verri M, Barbieri A, Boschi F.Dipartimento di Scienze del Farmaco, Universita_ degli Studi di Pavia, Viale Taramelli 14, 27100 Pavia, Italy. Recent studies show that supplemented essential amino acids (EAAs) may exert beneficial effects on CHF/COPD physical capacity. These mechanisms could be accounted for by EAA's intrinsic physiological activity which increases myofibrils and mitochondria genesis in skeletal muscle and myocardium and glucose control.
Cell Metab. 2014 Jun 3;19(6):1042-9.NAD(+)-dependent activation of Sirt1 corrects the phenotype in a mouse model of mitochondrial disease.Cerutti R, Pirinen E, Lamperti C, Marchet S, Sauve AA, Li W, Leoni V, Schon EA, Dantzer F, Auwerx J, Viscomi C, Zeviani M.Unit of Molecular Neurogenetics, The Foundation "Carlo Besta" Institute of Neurology IRCCS, 20133 Milan, Italy; MRC-Mitochondrial Biology Unit, Cambridge CB2 0XY, UK. This treatise indicated that supplementation with nicotinamide riboside, a natural NAD+ precursors, leads to marked improvement of the respiratory chain defect and exercise intolerance of mitochondrial disease model mice.
Nature. 2014 Jun 19;510(7505):397-401.The metabolite α-ketoglutarate extends lifespan by inhibiting ATP synthase and TOR.Chin RM, Fu X, Pai MY, Vergnes L, Hwang H, Deng G, Diep S, Lomenick B, Meli VS, Monsalve GC, Hu E, Whelan SA, Wang JX, Jung G, Solis GM, Fazlollahi F, Kaweeteerawat C, Quach A, Nili M, Krall AS, Godwin HA, Chang HR, Faull KF, Guo F, Jiang M, Trauger SA, Saghatelian A, Braas D, Christofk HR, Clarke CF, Teitell MA, Petrascheck M, Reue K, Jung ME, Frand AR, Huang J.Molecular Biology Institute, University of California Los Angeles, Los Angeles, California 90095, USA. Department of Molecular and Medical Pharmacology, University of California Los Angeles, Los Angeles, California 90095, USA. This treatise provide evidence that the lifespan increase by a-KG requires ATP synthase subunit b and is dependent on target of rapamycin (TOR) downstream. Endogenous a-KG levels are increased on starvation and a-KG does not extend the lifespan of dietary-restricted animals, indicating that a-KG is a key metabolite that mediates lon- gevity by dietary restriction.
J Biol Chem. 2014 Jul 28.Mitochondria are gate-keepers of T cell function by producing the ATP that drives purinergic signaling.Ledderose C, Bao Y, Lidicky M, Zipperle J, Li L, Strasser K, Shapiro NI, Junger WG.Beth Israel Deaconess Medical Center and Harvard Medical School, United States T cells play a central role in host defense. ATP release and autocrine feedback via purinergic receptors has been shown to regulate T cell function. This treatise indicated that rapid activation of mitochondrial ATP production fuels the purinergic signaling mechanisms that regulate T cells and define their role in host defense.
Biochem Biophys Res Commun. 2014 Jul 24.Mitochondrial tRNA cleavage by tRNA-targeting ribonuclease causes mitochondrial dysfunction observed in mitochondrial disease.Ogawa, Shimizu A, Takahashi K, Hidaka M, Masaki H.Department of Biotechnology, The University of Tokyo, Japan. The correlation between mt tRNA activity and mitochondrial dysfunction has been assessed. Then, cybrid cells have long been used for the analysis due to difficulty in mtDNA manipulation. This treatise propose a new method that involves mt tRNA cleavage by a bacterial tRNA-specific ribonuclease.
Mitochondrion. 2014 Jul 22.Comparative analysis of human mitochondrial methylomes shows distinct patterns of epigenetic regulation in mitochondria.Ghosh S, Sengupta, Scaria V.GN Ramachandran Knowledge Center for Genome Informatics, CSIR Institute of Genomics and Integrative Biology, Mall Road, Delhi 110007, India; Academy of Scientific and Innovative Research (AcSIR), New Delhi, India. The epigenetic modifications associated with the mitochondrial genome have not yet been analyzed at high resolutions. In the present work, This treatise analyzed methyl-cytosine profiles from methylated DNA immunoprecipitation datasets from 39 different human cell and tissue types from the NIH Roadmap Epigenomics project.
Br J Pharmacol. 2014 Jul 15.Glutathione administration reduces mitochondrial damage and shifts cell death from necrosis to apoptosis in aging diabetic mice hearts during exercise.Golbidi S, Botta A, Gottfred S, Nusrat A, Laher I, Ghosh S.Department of Anesthesiology, Pharmacology and Therapeutics, University of British Columbia, Vancouver, Canada. The effect of antioxidants in aging, type 2 diabetic (T2D) hearts during exercise is unclear. This treatise conclude that while GSH support is useful in reducing mOXS and attenuating necrosis and fibrosis in aging T2D hearts during exercise, such antioxidant treatment could be counterproductive in the healthy heart during exercise.
Hum Mol Genet. 2014 Jul 15. Oxaloacetate activates brain mitochondrial biogenesis, enhances the insulin pathway, reduces inflammation and stimulates neurogenesis.Wilkins HM, Harris JL, Carl SM, E L, Lu J, Eva Selfridge J, Roy N, Hutfles L, Koppel S, Morris J, Burns JM, Michaelis ML, Michaelis EK, Brooks WM, Swerdlow RH.Department of Neurology, University of Kansas Alzheimer's Disease Center, Department of Molecular and Integrative Physiology, Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City, KS 66160, USA Brain bioenergetic function declines in some neurodegenerative diseases. In mice, OAA promotes brain mitochondrial biogenesis, activates the insulin signaling pathway, reduces neuroinflammation and activates hippocampal neurogenesis.
J Cell Biol. 2014 Jul 21;206(2):289-305.Drosophila Sirt2/mammalian SIRT3 deacetylates ATP synthase β and regulates complex V activity.Rahman M, Nirala NK, Singh A, Zhu LJ, Taguchi K, Bamba T, Fukusaki E, Shaw LM, Lambright DG, Acharya JK, Acharya UR.Program in Gene Function and Expression, Program in Molecular Medicine, Program in Bioinformatics and Integrative Biology, and Department of Cancer Biology, University of Massachusetts Medical School, Worcester, MA 01605, USA Adenosine triphosphate (ATP) synthase β, the catalytic subunit of mitochondrial complex V, synthesizes ATP. This treatise show that ATP synthase β is deacetylated by aNAD+ dependent protein deacetylase, sirtuin 3, and its Drosophila melanogaster homologue, dSirt2.
Mol Cell. 2014 Jul 3.Bcl-2 Family Proteins Participate in Mitochondrial Quality Control by Regulating Parkin/PINK1-Dependent Mitophagy.Hollville E, Carroll RG, Cullen SP, Martin SJ.Molecular Cell Biology Laboratory, Department of Genetics, The Smurfit Institute, Trinity College Dublin, College Green, Dublin 2, Ireland. This treatise suggests that Bcl-2 family proteins act as global regulators of mitochondrial homeostasis.
BMC Res Notes. 2014 Jul 4;7(1):427. Ubiquinol reduces gamma glutamyltransferase as a marker of oxidative stress in humans.Onur S, Niklowitz P, Jacobs G, No_thlings U, Lieb W, Menke T, Do_ring F.Author information:Institute of Human Nutrition and Food Science, Division of Molecular Prevention, Christian Albrechts University Kiel, Heinrich-Hecht-Platz 10, 24118 Kiel, Germany. The reduced form of Coenzyme Q10 (CoQ10), ubiquinol (Q10H2), serves as a potent antioxidant in mitochondria and lipid membranes. Serum gamma-glutamyltransferase (GGT) activity is associated with cardiovascular diseases. Supplementation with Q10H2 reduces serum GGT activity.
Cell. 2014 Jul 3;158(1):84-97.A Nuclear Pyruvate Dehydrogenase Complex Is Important for the Generation of Acetyl-CoA and Histone Acetylation.Sutendra G, Kinnaird A, Dromparis P, Paulin R, Stenson TH, Haromy A, Hashimoto K, Zhang N, Flaim E, Michelakis ED.Department of Medicine, University of Alberta, Edmonton, AB T6G 2B7, Canada. A functional PDC generates acetyl-CoA in the nucleus independent of mitochondria. PDC translocates from mitochondria to the nucleus in response to multiple signals. Dynamic translocation of mitochondrial PDC to the nucleus provides a pathway for nuclear acetyl-CoA synthesis required for histone acetylation and epigenetic regulation.
Cell. 2014 Jul 3;158(1):54-68. Glucose Regulates Mitochondrial Motility via Milton Modification by O-GlcNAc Transferase.Pekkurnaz G, Trinidad JC, Wang X, Kong D, Schwarz TL.The F.M. Kirby Neurobiology Center, Boston Children's Hospital and Department ofNeurobiology, Harvard Medical School, Boston, MA 02115, USA. This treatise find that the GlcNAcylation state of Milton is altered by extracellular glucose and that OGT alters mitochondrial motility in vivo.
PLoS One. 2014 Jul 1;9(7).L-carnitine prevents progression of non-alcoholic steatohepatitis in a mouse model with upregulation of mitochondrial pathway.Ishikawa H, Takaki A, Tsuzaki R, Yasunaka T, Koike K, Shimomura Y, Seki H,Matsushita H, Miyake Y, Ikeda F, Shiraha H, Nouso K, Yamamoto K.Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. L-carnitine prevents progression of non-alcoholic steatohepatitis in a mouse model by upregulating the mitochondrial β-oxidation and redox system.
Mol Cell. 2014 Jul 17;55(2):253-63Tracing compartmentalized NADPH metabolism in the cytosol and mitochondria of Mammalian cells.Lewis CA, Parker SJ, Fiske BP, McCloskey D, Gui DY, Green CR, Vokes NI, Feist AM, Vander Heiden MG, Metallo CM.Department of Bioengineering, University of California, San Diego, La Jolla, CA 92093, USA; Institute of Engineering and Medicine, University of California, San Diego, La Jolla, CA 92093, USA. Here we develop an approach to resolve NADP(H)-dependent pathways present within both the cytosol and the mitochondria.
Cell Metab. 2014 Jul 4.The Lysosomal v-ATPase-Ragulator Complex Is a Common Activator for AMPK and mTORC1, Acting as a Switch between Catabolism and Anabolism.Zhang CS, Jiang B, Li M, Zhu M, Peng Y, Zhang YL, Wu YQ, Li TY, Liang Y, Lu Z, Lian G, Liu Q, Guo H, Yin Z, Ye Z, Han J, Wu JW, Yin H, Lin SY, Lin SC.State Key Laboratory for Cellular Stress Biology, School of Life Sciences, Xiamen University, Fujian 361102, China. This treatised discoveredthat the late endosomal/lysosomal protein complex v-ATPase-Ragulator, essential for activation of mTORC1, is also required for AMPK activation.
Mol Cell Biol. 2014 Aug 1;34(15):2890-902.Mitochondrial Matrix Ca2+ Accumulation Regulates Cytosolic NAD+/NADH Metabolism, Protein Acetylation, and Sirtuin Expression.Marcu R(1), Wiczer BM(1), Neeley CK(1), Hawkins BJ(2). Mitochondria and Metabolism Center, Department of Anesthesiology and PainMedicine, University of Washington, Seattle, Washington, USA . Mitochondrial calcium uptake stimulates bioenergetics and drives energy production in metabolic tissue. In endothelial cells, the mitochondrion-driven reduction in both the cytosolic and mitochondrial NAD(+)/NADH ratio stimulated a compensatory increase in SIRT1 protein levels that had an anti-inflammatory effect.

Latest Clinical Trial, Clinical Study and Clinical Research about MITO diseases

Clinical research, clinical trial, and clinical trial in Japan

Mito-disease.info introduces the latest Clinical Trial, Clinical Study and Clinical Research about MITO diseases. If you would need to check it/them more in detail, you can contact to the sponsor web individually.

Compound Action Type of disease Status Sponsor
EPI-743(UMIN000010783) Quality control:Anti-oxidant MELAS Clinical research National Center Hospital, National Center of Neurology and Psychiatry
EPI-743(JapicCTI-132349) Quality control:Anti-oxidant Leigh Syndrome Clinical trial (P2/3) Dainippon Sumitomo Pharma Co., Ltd
Taurine(UMIN000005592) Quality control: Post transcription modification MELAS Clinical study Kawasaki Medical School
Sodium succinate(UMIN000013512) Energy production: TCA cycle Mitochondrial disease Clinical study Tottori University Hospital
BF-759(UMIN000012934) Energy production: PPARγ agonist Mitochondrial fatty acid Beta-oxidation disorders Clinical study Shimane University School of Medicine
Sodium pyruvate(UMIN000008341) Energy production: Glycolysis Mitochondrial disease Clinical study Hiroshima University Graduate School of Biomedical & Health Sciences
Arginine(JMA-IIA00025) Quality control:Vessel protection by NO production MELAS Clinical study(Application preparation) Kurume University School of Medicine
5-ALA Energy production: Respiratory complex Mitochondrial disease Clinical study Saitama Medical University
Idebenone Energy production: Respiratory complex LHON Clinical study Hyogo Medical University

海外の臨床試験

Compound Action Type of disease Status Sponsor
EPI-743 (NCT01721733) Quality control: Anti-oxidant Leigh Syndrome Clinical study (P2) Edison Pharmaceuticals Inc
EPI-743 (NCT02104336) Quality control:Anti-oxidant Person Syndrome Clinical study (P2) Edison Pharmaceuticals Inc
RP 103 (NCT02023866) Quality control:Anti-oxidant Leigh Syndrome Clinical study (P2) Raptor Pharmaceuticals Inc
Dapagliflozin (NCT01439854) Insulin Sensitivity Multiple Mitochondrial Dysfunctions Syndrome Clinical study The University of Texas Health Science Center at San Antonio; National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Trihepatonin (NCT01379625) Energy production: β-oxidation Very Long-chain acylCoA Dehydrogenase (VLCAD) Deficiency; Carnitine Palmitoyltransferase 2 (CPT2) Deficiency; Mitochondrial Trifunctional Protein (TFP) Deficiency; Long-chain 3 hydroxyacylCoA Dehydrogenase (LCHAD) Deficiency Clinical study Oregon Health and Science University; University of Pittsburgh
GS-010 (NCT02064569) Gene therapy Mitochondrial disease Clinical study (P1/2) GenSight Biologics
rAAV2-ND4 (NCT01267422) Gene therapy MELAS Clinical study Huazhong University of Science and Technology

Research Topics about MITO diseases

Research topics:UMDF Mitochondrial Medicine (June, 2014)

Compound Action Status Sponsor
Bendavia Quality control:Mitophagy
Energy production: Respiratory complex
Clinical study (P2) Stealth Peptides Incorporated
Raxone Energy production: Respiratory complex
(14484>3640>11778, SD:83%, RR:50%)
NDA (EU: LHON) Santhera pharmaceuticals
NV189(Succinic acid prodrug) Energy production: TCA cycle Pre-clinical NeuroVive pharmaceuticals
XJB-5-131 Quality control:Anti-oxidant Pre-clinical University of Pittsburgh
Indole related compound Energy production: Respiratory complex Research Tohoku university
MRQ34 Quality control:Anti oxidant
Energy production: Respiratory complex
Research Arizona State University
mitoTALEN Abnormal mitochondrial gene correction Research Miami University
AIF : Apoptosis inducing factor Energy production: Respiratory complex Research University of Pennsylvania

Research topics:EuroMit (June, 2014)

Compound Action Status Institute
"High-fat, low-carbohydrate modified Atkins diet" Energy production: β-oxidation Clinical study (MELAS) University of Helsinki
L-cysteine Energy production: Respiratory complex "Research (MELAS, MARRF)" Newcastle University
Decanoic acid Energy production: β-oxidation Research UCL Institute of Child Health
GW1929 Energy production: PPARγ agonist Research University of Helsinki
Mdivi-1 Quality control: Fusion-promoting Research Columbia University
Ubiquinol-10 Quality control:Anti-oxidant Energy production: Respiratory complex Research Universidad de Granada
Nicotinamide riboside Energy production: Mitochondrial biogensis Research University of Helsinki
Riboflavin Energy production: Respiratory complex Research “Bambino Gesù” Children's Hospital

Information about Patient organizations related to MITO diseases

* under construction

Downloads

Events Calendar for MITO diseases (written in Japanese)