Applications in Translational Medicine

App Notes & Tech Briefs

• Bioenergetic analysis of suspension cells: hematopoietic stem cells and lymphocytes

  A real time assay that quantifies the ATP and biosynthetic demands of immune cell proliferation, differentiation and effector function.

  Normal suspension cells, such as hematopoietic cells and lymphocytes are responsible for supplying oxygen to the body and protecting the host. Cellular metabolism is markedly dynamic in immune responses during proliferation and immune effector functions...

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Publications

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• Mechanisms underlying metabolic and neural defects in zebrafish and human multiple acyl-CoA dehydrogenase deficiency (MADD)

  Song Y, Selak M A, Watson C T, Coutts C, Scherer P C, Panzer J A, Gibbs S, Scott M O, Willer G, Gregg R G, Ali D W, Bennett M J and Balice-Gordon R J

  PLoS One; 2009 Dec 17. 4 (12):e8329.

• Regulation of skeletal muscle oxidative capacity and insulin signaling by the mitochondrial rhomboid protease PARL

  Civitarese A E, MacLean P S, Carling S, Kerr-Bayles L, McMillan R P, Pierce A, Becker T C, Moro C, Finlayson J, Lefort N, Newgard C B, Mandarino L, Cefalu W, Walder K, Collier G R, Hulver M W, Smith S R and Ravussin E

  Cell Metab; 2010 May 5. 11 (5):412-426.

• Medroxyprogesterone Acetate Antagonizes Estrogen Up-Regulation of Brain Mitochondrial Function

  Irwin R W, Yao J, Ahmed S S, Hamilton R T, Cadenas E and Brinton R D

  Endocrinology; 2010 Dec 15. 152 (2):556-567.

• Acquisition of temozolomide chemoresistance in gliomas leads to remodeling of mitochondrial electron transport chain

  Oliva C R, Nozell S E, Diers A, McClugage S G, 3rd, Sarkaria J N, Markert J M, Darley-Usmar V M, Bailey S M, Gillespie G Y, Landar A and Griguer C E

  J Biol Chem; 2010 Dec 17. 285 (51):39759-39767.

• Apolipoprotein e4 domain interaction mediates detrimental effects on mitochondria and is a potential therapeutic target for Alzheimer disease

  Chen H K, Ji Z S, Dodson S E, Miranda R D, Rosenblum C I, Reynolds I J, Freedman S B, Weisgraber K H, Huang Y and Mahley R W

  J Biol Chem; 2011 Feb 18. 286 (7):5215-5221.

• Exploring a glycolytic inhibitor for the treatment of an FH-deficient type-2 papillary RCC

  Yamasaki T, Tran T A, Oz O K, Raj G V, Schwarz R E, Deberardinis R J, Zhang X and Brugarolas J

  Nat Rev Urol; 2011 Mar 1. 8 (3):165-171.

• Mutation in the mitochondrial tRNAVal causes mitochondrial encephalopathy, lactic acidosis and stroke-like episodes

  Glatz C, D'Aco K, Smith S and Sondheimer N

  Mitochondrion; 2011 Jul 1. 11 (4):615-619.

• 2-deoxy-d-glucose treatment induces ketogenesis, sustains mitochondrial function, and reduces pathology in female mouse model of Alzheimer's disease

  Yao J, Chen S, Mao Z, Cadenas E and Brinton R D

  PLoS One; 2011 Jul 1. 6 (7):e21788.

• Targeting GLUT1 and the Warburg Effect in Renal Cell Carcinoma by Chemical Synthetic Lethality

  Chan D A, Sutphin P D, Nguyen P, Turcotte S, Lai E W, Banh A, Reynolds G E, Chi J T, Wu J, Solow-Cordero D E, Bonnet M, Flanagan J U, Bouley D M, Graves E E, Denny W A, Hay M P and Giaccia A J

  Sci Transl Med; 2011 Aug 3. 3 (94):94ra70.

• Withaferin a-induced apoptosis in human breast cancer cells is mediated by reactive oxygen species

  Hahm E R, Moura M B, Kelley E E, Van Houten B, Shiva S and Singh S V

  PLoS One; 2011 Aug 10. 6 (8):e23354.

• Hydrogen sulfide replacement therapy protects the vascular endothelium in hyperglycemia by preserving mitochondrial function

  Suzuki K, Olah G, Modis K, Coletta C, Kulp G, Gero D, Szoleczky P, Chang T, Zhou Z, Wu L, Wang R, Papapetropoulos A and Szabo C

  Proc Natl Acad Sci U S A; 2011 Aug 16. 108 (33):13829-13834.

• SRT1720 improves survival and healthspan of obese mice

  Minor R K, Baur J A, Gomes A P, Ward T M, Csiszar A, Mercken E M, Abdelmohsen K, Shin Y-K, Canto C, Scheibye-Knudsen M, Krawczyk M, Irusta P M, Martin-Montalvo A, Hubbard B P, Zhang Y, Lehrmann E, White A A, Price N L, Swindell W R, Pearson K J, Becker K G, Bohr V A, Gorospe M, Egan J M, Talan M I, Auwerx J, Westphal C H, Ellis J L, Ungvari Z, Vlasuk G P, Elliott P J, Sinclair D A and de Cabo R

  Sci. Rep.; 2011 Aug 18. 1 (2011)

• Human iPSCs Harbor Homoplasmic and Heteroplasmic Mitochondrial DNA Mutations While Maintaining hESC-Like Metabolic Reprogramming

  Prigione A, Lichtner B, Kuhl H, Struys E A, Wamelink M, Lehrach H, Ralser M, Timmermann B and Adjaye J

  Stem Cells; 2011 Sep 1. 29 (9):1338-1348.

• The effects of idebenone on mitochondrial bioenergetics

  Giorgio V, Petronilli V, Ghelli A, Carelli V, Rugolo M, Lenaz G and Bernardi P

  Biochimica et Biophysica Acta (BBA) - Bioenergetics; 2011 Nov 4.

• Galactose enhances oxidative metabolism and reveals mitochondrial dysfunction in human primary muscle cells

  Aguer C, Gambarotta D, Mailloux R J, Moffat C, Dent R, McPherson R and Harper M E

  PLoS One; 2011 Dec 15. 6 (12):e28536.

• Identification and analysis of the active phytochemicals from the anti-cancer botanical extract bezielle

  Chen V, Staub R E, Baggett S, Chimmani R, Tagliaferri M, Cohen I and Shtivelman E

  PLoS One; 2012 Jan 17. 7 (1):e30107.

• Mitochondrial Stress Engages E2F1 Apoptotic Signaling to Cause Deafness

  Raimundo N, Song L, Shutt T E, McKay S E, Cotney J, Guan M X, Gilliland T C, Hohuan D, Santos-Sacchi J and Shadel G S

  Cell; 2012 Feb 17. 148 (4):716-726.

• Microscale oxygraphy reveals OXPHOS impairment in MRC mutant cells

  Invernizzi F, D'Amato I, Jensen P B, Ravaglia S, Zeviani M and Tiranti V

  Mitochondrion; 2012 Mar 1. 12 (2):328-35.

• The Transcriptional Coactivators, PGC-1? and ?, Cooperate to Maintain Cardiac Mitochondrial Function During the Early Stages of Insulin Resistance

  Mitra R, Nogee D P, Zechner J F, Yea K, Gierasch C M, Kovacs A, Medeiros D M, Kelly D P and Duncan J G

  J Mol Cell Cardiol; 2012 Mar 1. 52 (3):701-10.

• Correcting human mitochondrial mutations with targeted RNA import

  Wang G, Shimada E, Zhang J, Hong J S, Smith G M, Teitell M A and Koehler C M

  Proc Natl Acad Sci U S A; 2012 Mar 27. 109 (13):4840-4845.

• Platelet Mitochondrial Dysfunction is Evident in Type 2 Diabetes in Association with Modifications of Mitochondrial Anti-Oxidant Stress Proteins

  Avila C, Huang R J, Stevens M V, Aponte A M, Tripodi D, Kim K Y and Sack M N

  Exp Clin Endocrinol Diabetes; 2012 Apr 1. 120 (4):248-51.

• Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing

  Haack T B, Haberberger B, Frisch E M, Wieland T, Iuso A, Gorza M, Strecker V, Graf E, Mayr J A, Herberg U, Hennermann J B, Klopstock T, Kuhn K A, Ahting U, Sperl W, Wilichowski E, Hoffmann G F, Tesarova M, Hansikova H, Zeman J, Plecko B, Zeviani M, Wittig I, Strom T M, Schuelke M, Freisinger P, Meitinger T and Prokisch H

  J Med Genet; 2012 Apr 1. 49 (4):277-283.

• Measurement of mitochondrial respiration in trophoblast culture

  Maloyan A, Mele J, Muralimanohara B and Myatt L

  Placenta; 2012 May 1. 33 (5):456-8.

• Leukoencephalopathy with thalamus and brainstem involvement and high lactate 'LTBL' caused by EARS2 mutations

  Steenweg M E, Ghezzi D, Haack T, Abbink T E, Martinelli D, van Berkel C G, Bley A, Diogo L, Grillo E, Te Water Naude J, Strom T M, Bertini E, Prokisch H, van der Knaap M S and Zeviani M

  Brain; 2012 May 1. 135 (Pt 5):1387-94.

• The Ancient Drug Salicylate Directly Activates AMP-Activated Protein Kinase

  Hawley S A, Fullerton M D, Ross F A, Schertzer J D, Chevtzoff C, Walker K J, Peggie M W, Zibrova D, Green K A, Mustard K J, Kemp B E, Sakamoto K, Steinberg G R and Hardie D G

  Science; 2012 May 18. 336 (6083):918-22.

• Inhibiting Systemic Autophagy during Interleukin 2 (IL-2) Immunotherapy Promotes Long-term Tumor Regression

  Liang X, De Vera M E, Buchser W J, de Vivar Chavez A R, Loughran P, Stolz D B, Basse P, Wang T, Van Houten B, Zeh H J, 3rd and Lotze M T

  Cancer Res; 2012 Jun 1. 72 (11):2791-801.

• An altered redox balance mediates the hypersensitivity of Cockayne syndrome primary fibroblasts to oxidative stress

  Pascucci B, Lemma T, Iorio E, Giovannini S, Vaz B, Iavarone I, Calcagnile A, Narciso L, Degan P, Podo F, Roginskya V, Janjic B M, Van Houten B, Stefanini M, Dogliotti E and D'Errico M

  Aging Cell; 2012 Jun 1. 11 (3):520-29.

• Mutations of the Mitochondrial-tRNA Modifier MTO1 Cause Hypertrophic Cardiomyopathy and Lactic Acidosis

  Ghezzi D, Baruffini E, Haack T B, Invernizzi F, Melchionda L, Dallabona C, Strom T M, Parini R, Burlina A B, Meitinger T, Prokisch H, Ferrero I and Zeviani M

  Am J Hum Genet; 2012 Jun 8. 90 (6):1079-87.

• Rationale and Design of MAGNET (Mitochondria-AGing in NorthErn Taiwan) Study: A Community-based Cohort Investigating Mitochondria-related Aging and Cardiovascular Diseases in Suburban Areas of Northern Taiwan

  Hung C-L, Wu Y-J, Liu C-C, Chi B-C, Chang S-S, Wang L-Y, Yeh H-I and Wei Y-H

  International Journal of Gerontology; 2012 Jun 8. 6 (2):122-126.

• Methazolamide Is a New Hepatic Insulin Sensitizer That Lowers Blood Glucose In Vivo

  Konstantopoulos N, Molero J C, McGee S L, Spolding B, Connor T, de Vries M, Wanyonyi S, Fahey R, Morrison S, Swinton C, Jones S, Cooper A, Garcia-Guerra L, Foletta V C, Krippner G, Andrikopoulos S and Walder K R

  Diabetes; 2012 Aug 1. 61 (8):2146-54.

• MIR-210 modulates mitochondrial respiration in placenta with preeclampsia

  Muralimanoharan S, Maloyan A, Mele J, Guo C, Myatt L G and Myatt L

  Placenta; 2012 Oct 1. 33 (10):816-23.

• Importance of glycolysis and oxidative phosphorylation in advanced melanoma

  Ho J, de Moura M B, Lin Y, Vincent G, Thorne S, Duncan L M, Hui-Min L, Kirkwood J M, Becker D, Van Houten B and Moschos S J

  Mol Cancer; 2012 Oct 9. 11 (1):76.

• Alteration of Fatty-Acid-Metabolizing Enzymes Affects Mitochondrial Form and Function in Hereditary Spastic Paraplegia

  Tesson C, Nawara M, Salih M A, Rossignol R, Zaki M S, Al Balwi M, Schule R, Mignot C, Obre E, Bouhouche A, Santorelli F M, Durand C M, Oteyza A C, El-Hachimi K H, Al Drees A, Bouslam N, Lamari F, Elmalik S A, Kabiraj M M, Seidahmed M Z, Esteves T, Gaussen M, Monin M L, Gyapay G, Lechner D, Gonzalez M, Depienne C, Mochel F, Lavie J, Schols L, Lacombe D, Yahyaoui M, Al Abdulkareem I, Zuchner S, Yamashita A, Benomar A, Goizet C, Durr A, Gleeson J G, Darios F, Brice A and Stevanin G

  Am J Hum Genet; 2012 Dec 7. 91 (6):1051-64.

• Homozygous missense mutation in BOLA3 causes multiple mitochondrial dysfunctions syndrome in two siblings

  Haack T B, Rolinski B, Haberberger B, Zimmermann F, Schum J, Strecker V, Graf E, Athing U, Hoppen T, Wittig I, Sperl W, Freisinger P, Mayr J A, Strom T M, Meitinger T and Prokisch H

  J Inherit Metab Dis; 2013 Jan 1. 36 (1):55-62.

• Studying arrhythmogenic right ventricular dysplasia with patient-specific iPSCs

  Kim C, Wong J, Wen J, Wang S, Wang C, Spiering S, Kan N G, Forcales S, Puri P L, Leone T C, Marine J E, Calkins H, Kelly D P, Judge D P and Chen H S

  Nature; 2013 Feb 7. 494 (7435):105-110.

• Induced Pluripotent Stem Cells with a Pathological Mitochondrial DNA Deletion

  Cherry A B, Gagne K E, McLoughlin E M, Baccei A, Gorman B, Hartung O, Miller J D, Zhang J, Zon R L, Ince T A, Neufeld E J, Lerou P H, Fleming M D, Daley G Q and Agarwal S

  Stem Cells. 2013 Feb 8; [Epub ahead of print].

• Methods for defining distinct bioenergetic profiles in platelets, lymphocytes, monocytes, and neutrophils, and the oxidative burst from human blood

  Chacko B K, Kramer P A, Ravi S, Johnson M S, Hardy R W, Ballinger S W and Darley-Usmar V M

  Lab Invest. 2013 Mar 25; [Epub ahead of print].

• Mechanisms of Mitochondrial Damage in Keratinocytes by Pemphigus Vulgaris Antibodies

  Kalantari-Dehaghi M, Chen Y, Deng W, Chernyavsky A, Marchenko S, Wang P H and Grando S A

  J Biol Chem. 2013 Apr 18; [Epub ahead of print].

• Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy

  Lopez-Erauskin J, Galino J, Ruiz M, Cuezva J M, Fabregat I, Cacabelos D, Boada J, Martinez J, Ferrer I, Pamplona R, Villarroya F, Portero-Otin M, Fourcade S and Pujol A

  Human molecular genetics; 2013 May 3.

Technical Posters

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• Increased Susceptibility to Reactive Oxygen Species in Autism Lymphoblastoid Cells is mediated by Mitochondrial Dysfunction

  Shannon Rose, Stepan Melnyk, Richard E. Frye and S. Jill James

  Neuroscience 2012
  October 14-17, 2012
  New Orleans, LA

• Menadione-induced Oxidative Stress Modulates Mitochondrial Bioenergetics in Pancreatic Acinar Cells

  Armstrong J, Sutton R & Criddle DN

  International Congress on Cell Membranes and Oxidative Stress Focus on Calcium Signaling and TRP Channels 2012
  June 26-29, 2012
  Isparta, Turkey

• Mitochondrial Stress Triggers E2F1 Apoptotic Signaling to Cause Deafness - Insights into Tissue-Specific Mechanisms

  Nuno Raimundo, Lei Song, Tim Shutt, Sharen McKay, Joseph Santos-Sacchi, Gerald Shadel

  Keystone Symposia: Mitochondrial Dynamics and Function
  March 19-24, 2012
  Banff, Alberta, CA

Webinars

Sort by: Title | Speaker | Date
• 

  Mitochondrial Bioenergetics in Disease: Toward a Mitochondrial Therapy of Muscular Dystrophies and Beyond

  Paolo Bernardi, MD, University of Padova
  September 15, 2009

• 

  Acute Brain Injury: Assessing Mitochondrial Dysfunction and the Role of Calpain Proteases in vitro

  Brian Polster, PhD, University of Maryland Medical School
  August 19, 2009

• 

  How to Measure Mitochondrial Function in Whole Islets. Sensing the nutrient sensor: measuring bioenergetic parameters from intact mouse and human islets of langerhans

  Orian Shirihai, MD, PhD, Boston University Medical Center
  July 16, 2009

• 

  Primary Renal Proximal Tubules as a Platform for Mitochondrial Biogenesis and Toxicity Assays

  Craig Beeson, PhD , Medical University of South Carolina
  June 24, 2009

• 

  Cybrid Modeling of Sporadic Neurodegenerative Disorders

  Russell H. Swerdlow, MD, University of Kansas School of Medicine
  March 16, 2009

• 

  Bioenergetic Profiling in Translational Neuroscience

  Jon Nilsen, PhD, University of Southern California
  June 25, 2008

• 

  Human Disorders of Bioenergetics

  Vamsi Mootha, MD, Harvard Medical School Center for Human Genetics, Massachusetts General Hospital
  February 26, 2008

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