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Items: 1 to 50 of 124


Non-phagocytic Activation of NOX2 is Implicated in Progressive Non-alcoholic Steatohepatitis During Aging.

Jiang JX, Fish SR, Tomilov A, Li Y, Fan W, Dehnad A, Gae D, Das S, Mozes G, Charville GW, Ramsey J, Cortopassi G, Török NJ.

Hepatology. 2020 Jan 17. doi: 10.1002/hep.31118. [Epub ahead of print]


PPARα-targeted mitochondrial bioenergetics mediate repair of intestinal barriers at the host-microbe intersection during SIV infection.

Crakes KR, Santos Rocha C, Grishina I, Hirao LA, Napoli E, Gaulke CA, Fenton A, Datta S, Arredondo J, Marco ML, Sankaran-Walters S, Cortopassi G, Giulivi C, Dandekar S.

Proc Natl Acad Sci U S A. 2019 Dec 3;116(49):24819-24829. doi: 10.1073/pnas.1908977116. Epub 2019 Nov 18.


Clinical and molecular profile of a Brazilian cohort of patients with classical BCR-ABL1-negative myeloproliferative neoplasms.

Porto-Soares MA, de Oliveira RD, Cortopassi GM, Machado-Neto JA, Palma LC, Figueiredo-Pontes LL.

Hematol Transfus Cell Ther. 2019 Oct 10. pii: S2531-1379(19)30140-3. doi: 10.1016/j.htct.2019.07.008. [Epub ahead of print]


Potential biomarker identification for Friedreich's ataxia using overlapping gene expression patterns in patient cells and mouse dorsal root ganglion.

McMackin MZ, Durbin-Johnson B, Napierala M, Napierala JS, Ruiz L, Napoli E, Perlman S, Giulivi C, Cortopassi GA.

PLoS One. 2019 Oct 30;14(10):e0223209. doi: 10.1371/journal.pone.0223209. eCollection 2019.


Cetylpyridinium chloride is a potent AMP-activated kinase (AMPK) inducer and has therapeutic potential in cancer.

Allen SA, Datta S, Sandoval J, Tomilov A, Sears T, Woolard K, Angelastro JM, Cortopassi GA.

Mitochondrion. 2020 Jan;50:19-24. doi: 10.1016/j.mito.2019.09.009. Epub 2019 Oct 22.


Dimethyl fumarate dosing in humans increases frataxin expression: A potential therapy for Friedreich's Ataxia.

Jasoliya M, Sacca F, Sahdeo S, Chedin F, Pane C, Brescia Morra V, Filla A, Pook M, Cortopassi G.

PLoS One. 2019 Jun 3;14(6):e0217776. doi: 10.1371/journal.pone.0217776. eCollection 2019.


Idebenone is a cytoprotective insulin sensitizer whose mechanism is Shc inhibition.

Tomilov A, Allen S, Hui CK, Bettaieb A, Cortopassi G.

Pharmacol Res. 2018 Nov;137:89-103. doi: 10.1016/j.phrs.2018.09.024. Epub 2018 Oct 2.


Small molecules bind human mTOR protein and inhibit mTORC1 specifically.

Allen SA, Tomilov A, Cortopassi GA.

Biochem Pharmacol. 2018 Sep;155:298-304. doi: 10.1016/j.bcp.2018.07.013. Epub 2018 Jul 17.


A Ketogenic Diet Extends Longevity and Healthspan in Adult Mice.

Roberts MN, Wallace MA, Tomilov AA, Zhou Z, Marcotte GR, Tran D, Perez G, Gutierrez-Casado E, Koike S, Knotts TA, Imai DM, Griffey SM, Kim K, Hagopian K, McMackin MZ, Haj FG, Baar K, Cortopassi GA, Ramsey JJ, Lopez-Dominguez JA.

Cell Metab. 2018 May 1;27(5):1156. doi: 10.1016/j.cmet.2018.04.005. No abstract available.


An innate immune response and altered nuclear receptor activation defines the spinal cord transcriptome during alpha-tocopherol deficiency in Ttpa-null mice.

Finno CJ, Bordbari MH, Gianino G, Ming-Whitfield B, Burns E, Merkel J, Britton M, Durbin-Johnson B, Sloma EA, McMackin M, Cortopassi G, Rivas V, Barro M, Tran CK, Gennity I, Habib H, Xu L, Puschner B, Miller AD.

Free Radic Biol Med. 2018 May 20;120:289-302. doi: 10.1016/j.freeradbiomed.2018.02.037. Epub 2018 Mar 9.


Disruption of mitochondrial function as mechanism for anti-cancer activity of a novel mitochondriotropic menadione derivative.

Teixeira J, Amorim R, Santos K, Soares P, Datta S, Cortopassi GA, Serafim TL, Sardão VA, Garrido J, Borges F, Oliveira PJ.

Toxicology. 2018 Jan 15;393:123-139. doi: 10.1016/j.tox.2017.11.014. Epub 2017 Nov 12.


Rescue of cell death and inflammation of a mouse model of complex 1-mediated vision loss by repurposed drug molecules.

Yu AK, Datta S, McMackin MZ, Cortopassi GA.

Hum Mol Genet. 2017 Dec 15;26(24):4929-4936. doi: 10.1093/hmg/ddx373.


In Vitro Evaluation of Mitochondrial Function and Estrogen Signaling in Cell Lines Exposed to the Antiseptic Cetylpyridinium Chloride.

Datta S, He G, Tomilov A, Sahdeo S, Denison MS, Cortopassi G.

Environ Health Perspect. 2017 Aug 22;125(8):087015. doi: 10.1289/EHP1404.


A Ketogenic Diet Extends Longevity and Healthspan in Adult Mice.

Roberts MN, Wallace MA, Tomilov AA, Zhou Z, Marcotte GR, Tran D, Perez G, Gutierrez-Casado E, Koike S, Knotts TA, Imai DM, Griffey SM, Kim K, Hagopian K, McMackin MZ, Haj FG, Baar K, Cortopassi GA, Ramsey JJ, Lopez-Dominguez JA.

Cell Metab. 2017 Sep 5;26(3):539-546.e5. doi: 10.1016/j.cmet.2017.08.005. Erratum in: Cell Metab. 2018 May 1;27(5):1156.


Synthesis and biochemical characterization of EGF receptor in a water-soluble membrane model system.

Scharadin TM, He W, Yiannakou Y, Tomilov AA, Saldana M, Cortopassi GA, Carraway KL 3rd, Coleman MA, Henderson PT.

PLoS One. 2017 Jun 6;12(6):e0177761. doi: 10.1371/journal.pone.0177761. eCollection 2017.


Dimethyl fumarate mediates Nrf2-dependent mitochondrial biogenesis in mice and humans.

Hayashi G, Jasoliya M, Sahdeo S, Saccà F, Pane C, Filla A, Marsili A, Puorro G, Lanzillo R, Brescia Morra V, Cortopassi G.

Hum Mol Genet. 2017 Aug 1;26(15):2864-2873. doi: 10.1093/hmg/ddx167.


The Eye Drop Preservative Benzalkonium Chloride Potently Induces Mitochondrial Dysfunction and Preferentially Affects LHON Mutant Cells.

Datta S, Baudouin C, Brignole-Baudouin F, Denoyer A, Cortopassi GA.

Invest Ophthalmol Vis Sci. 2017 Apr 1;58(4):2406-2412. doi: 10.1167/iovs.16-20903.


Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans.

Jasoliya MJ, McMackin MZ, Henderson CK, Perlman SL, Cortopassi GA.

Hum Mol Genet. 2017 Jul 15;26(14):2627-2633. doi: 10.1093/hmg/ddx141.


Mice with low levels of Shc proteins display reduced glycolytic and increased gluconeogenic activities in liver.

Hagopian K, Kim K, López-Dominguez JA, Tomilov AA, Cortopassi GA, Ramsey JJ.

Biochem Biophys Rep. 2016 Sep;7:273-286. doi: 10.1016/j.bbrep.2016.06.021. Epub 2016 Jun 30.


Parkin deficiency accelerates consequences of mitochondrial DNA deletions and Parkinsonism.

Song L, McMackin M, Nguyen A, Cortopassi G.

Neurobiol Dis. 2017 Apr;100:30-38. doi: 10.1016/j.nbd.2016.12.024. Epub 2016 Dec 29.


Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model.

Song L, Yu A, Murray K, Cortopassi G.

Brain Res. 2017 Feb 15;1657:232-244. doi: 10.1016/j.brainres.2016.12.019. Epub 2016 Dec 24.


A high-throughput screen for mitochondrial function reveals known and novel mitochondrial toxicants in a library of environmental agents.

Datta S, Sahdeo S, Gray JA, Morriseau C, Hammock BD, Cortopassi G.

Mitochondrion. 2016 Nov;31:79-83. doi: 10.1016/j.mito.2016.10.001. Epub 2016 Oct 4.


Galectin-3 regulates inflammasome activation in cholestatic liver injury.

Tian J, Yang G, Chen HY, Hsu DK, Tomilov A, Olson KA, Dehnad A, Fish SR, Cortopassi G, Zhao B, Liu FT, Gershwin ME, Török NJ, Jiang JX.

FASEB J. 2016 Dec;30(12):4202-4213. Epub 2016 Sep 14.


Neurobehavioral deficits in the KIKO mouse model of Friedreich's ataxia.

McMackin MZ, Henderson CK, Cortopassi GA.

Behav Brain Res. 2017 Jan 1;316:183-188. doi: 10.1016/j.bbr.2016.08.053. Epub 2016 Aug 26.


Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutations.

Datta S, Tomilov A, Cortopassi G.

Mitochondrion. 2016 Sep;30:177-86. doi: 10.1016/j.mito.2016.08.002. Epub 2016 Aug 4.


Lactate and Pyruvate Are Major Sources of Energy for Stallion Sperm with Dose Effects on Mitochondrial Function, Motility, and ROS Production.

Darr CR, Varner DD, Teague S, Cortopassi GA, Datta S, Meyers SA.

Biol Reprod. 2016 Aug;95(2):34. doi: 10.1095/biolreprod.116.140707. Epub 2016 Jun 22.


Mitochondrial oxygen consumption is a unique indicator of stallion spermatozoal health and varies with cryopreservation media.

Darr CR, Cortopassi GA, Datta S, Varner DD, Meyers SA.

Theriogenology. 2016 Sep 15;86(5):1382-92. doi: 10.1016/j.theriogenology.2016.04.082. Epub 2016 May 6.


Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia.

Hayashi G, Cortopassi G.

PLoS One. 2016 Apr 14;11(4):e0153574. doi: 10.1371/journal.pone.0153574. eCollection 2016.


p46Shc Inhibits Thiolase and Lipid Oxidation in Mitochondria.

Tomilov A, Tomilova N, Shan Y, Hagopian K, Bettaieb A, Kim K, Pelicci PG, Haj F, Ramsey J, Cortopassi G.

J Biol Chem. 2016 Jun 10;291(24):12575-85. doi: 10.1074/jbc.M115.695577. Epub 2016 Apr 8.


Frataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34.

Shen Y, McMackin MZ, Shan Y, Raetz A, David S, Cortopassi G.

PLoS One. 2016 Mar 8;11(3):e0151026. doi: 10.1371/journal.pone.0151026. eCollection 2016.


Mitochondrial Hspa9/Mortalin regulates erythroid differentiation via iron-sulfur cluster assembly.

Shan Y, Cortopassi G.

Mitochondrion. 2016 Jan;26:94-103. doi: 10.1016/j.mito.2015.12.005. Epub 2015 Dec 17.


Oxidative stress in inherited mitochondrial diseases.

Hayashi G, Cortopassi G.

Free Radic Biol Med. 2015 Nov;88(Pt A):10-7. doi: 10.1016/j.freeradbiomed.2015.05.039. Epub 2015 Jun 12. Review.


Key glycolytic enzyme activities of skeletal muscle are decreased under fed and fasted states in mice with knocked down levels of Shc proteins.

Hagopian K, Tomilov AA, Kim K, Cortopassi GA, Ramsey JJ.

PLoS One. 2015 Apr 16;10(4):e0124204. doi: 10.1371/journal.pone.0124204. eCollection 2015.


Dysregulation of glutamine transporter SNAT1 in Rett syndrome microglia: a mechanism for mitochondrial dysfunction and neurotoxicity.

Jin LW, Horiuchi M, Wulff H, Liu XB, Cortopassi GA, Erickson JD, Maezawa I.

J Neurosci. 2015 Feb 11;35(6):2516-29. doi: 10.1523/JNEUROSCI.2778-14.2015.


Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse.

Yu AK, Song L, Murray KD, van der List D, Sun C, Shen Y, Xia Z, Cortopassi GA.

Hum Mol Genet. 2015 May 15;24(10):2848-60. doi: 10.1093/hmg/ddv045. Epub 2015 Feb 4.


Mitochondrial complex I defects increase ubiquitin in substantia nigra.

Song L, Cortopassi G.

Brain Res. 2015 Jan 12;1594:82-91. doi: 10.1016/j.brainres.2014.11.013. Epub 2014 Nov 13.


Shc depletion stimulates brown fat activity in vivo and in vitro.

Tomilov A, Bettaieb A, Kim K, Sahdeo S, Tomilova N, Lam A, Hagopian K, Connell M, Fong J, Rowland D, Griffey S, Ramsey J, Haj F, Cortopassi G.

Aging Cell. 2014 Dec;13(6):1049-58. doi: 10.1111/acel.12267. Epub 2014 Sep 26.


Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.

Sahdeo S, Scott BD, McMackin MZ, Jasoliya M, Brown B, Wulff H, Perlman SL, Pook MA, Cortopassi GA.

Hum Mol Genet. 2014 Dec 20;23(25):6848-62. doi: 10.1093/hmg/ddu408. Epub 2014 Aug 11.


Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia.

Hayashi G, Shen Y, Pedersen TL, Newman JW, Pook M, Cortopassi G.

Hum Mol Genet. 2014 Dec 20;23(25):6838-47. doi: 10.1093/hmg/ddu407. Epub 2014 Aug 7.


High-throughput screening of FDA-approved drugs using oxygen biosensor plates reveals secondary mitofunctional effects.

Sahdeo S, Tomilov A, Komachi K, Iwahashi C, Datta S, Hughes O, Hagerman P, Cortopassi G.

Mitochondrion. 2014 Jul;17:116-25. doi: 10.1016/j.mito.2014.07.002. Epub 2014 Jul 14.


The influence of Shc proteins on life span in mice.

Ramsey JJ, Tran D, Giorgio M, Griffey SM, Koehne A, Laing ST, Taylor SL, Kim K, Cortopassi GA, Lloyd KC, Hagopian K, Tomilov AA, Migliaccio E, Pelicci PG, McDonald RB.

J Gerontol A Biol Sci Med Sci. 2014 Oct;69(10):1177-85. doi: 10.1093/gerona/glt198. Epub 2013 Dec 14.


Effects of alkyl side chain modification of coenzyme Q10 on mitochondrial respiratory chain function and cytoprotection.

Fash DM, Khdour OM, Sahdeo SJ, Goldschmidt R, Jaruvangsanti J, Dey S, Arce PM, Collin VC, Cortopassi GA, Hecht SM.

Bioorg Med Chem. 2013 Apr 15;21(8):2346-2354. doi: 10.1016/j.bmc.2013.01.075. Epub 2013 Feb 13.


Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model.

Shan Y, Schoenfeld RA, Hayashi G, Napoli E, Akiyama T, Iodi Carstens M, Carstens EE, Pook MA, Cortopassi GA.

Antioxid Redox Signal. 2013 Nov 1;19(13):1481-93. doi: 10.1089/ars.2012.4537. Epub 2013 Mar 28.


OPA1 mutation and late-onset cardiomyopathy: mitochondrial dysfunction and mtDNA instability.

Chen L, Liu T, Tran A, Lu X, Tomilov AA, Davies V, Cortopassi G, Chiamvimonvat N, Bers DM, Votruba M, Knowlton AA.

J Am Heart Assoc. 2012 Oct;1(5):e003012. doi: 10.1161/JAHA.112.003012. Epub 2012 Oct 25.


Development of an HTS assay for EPHX2 phosphatase activity and screening of nontargeted libraries.

Morisseau C, Sahdeo S, Cortopassi G, Hammock BD.

Anal Biochem. 2013 Mar 1;434(1):105-11. doi: 10.1016/j.ab.2012.11.017. Epub 2012 Dec 3.


Mutant Twinkle increases dopaminergic neurodegeneration, mtDNA deletions and modulates Parkin expression.

Song L, Shan Y, Lloyd KC, Cortopassi GA.

Hum Mol Genet. 2012 Dec 1;21(23):5147-58. doi: 10.1093/hmg/dds365. Epub 2012 Sep 4.


Shc proteins influence the activities of enzymes involved in fatty acid oxidation and ketogenesis.

Hagopian K, Tomilov AA, Tomilova N, Kim K, Taylor SL, Lam AK, Cortopassi GA, McDonald RB, Ramsey JJ.

Metabolism. 2012 Dec;61(12):1703-13. doi: 10.1016/j.metabol.2012.05.007. Epub 2012 Jun 7.


Hepatic Src homology phosphatase 2 regulates energy balance in mice.

Nagata N, Matsuo K, Bettaieb A, Bakke J, Matsuo I, Graham J, Xi Y, Liu S, Tomilov A, Tomilova N, Gray S, Jung DY, Ramsey JJ, Kim JK, Cortopassi G, Havel PJ, Haj FG.

Endocrinology. 2012 Jul;153(7):3158-69. doi: 10.1210/en.2012-1406. Epub 2012 May 22.


HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis.

Shan Y, Cortopassi G.

Hum Mol Genet. 2012 Apr 1;21(7):1457-69. doi: 10.1093/hmg/ddr582. Epub 2011 Dec 13.

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