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Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase gamma.

Lewis W, Day BJ, Kohler JJ, Hosseini SH, Chan SS, Green EC, Haase CP, Keebaugh ES, Long R, Ludaway T, Russ R, Steltzer J, Tioleco N, Santoianni R, Copeland WC.

Lab Invest. 2007 Apr;87(4):326-35. Epub 2006 Feb 19.


Mitochondrial POLG mutation, mtDNA depletion, and cardiomyopathy.

Lasota J.

Lab Invest. 2007 Apr;87(4):316. No abstract available.


Cardiac-targeted transgenic mutant mitochondrial enzymes: mtDNA defects, antiretroviral toxicity and cardiomyopathy.

Kohler JJ, Hosseini SH, Green E, Hoying-Brandt A, Cucoranu I, Haase CP, Russ R, Srivastava J, Ivey K, Ludaway T, Kapoor V, Abuin A, Shapoval A, Santoianni R, Saada A, Elpeleg O, Lewis W.

Cardiovasc Toxicol. 2008 Summer;8(2):57-69. doi: 10.1007/s12012-008-9015-1. Epub 2008 Apr 30.


Transgenic mouse model with deficient mitochondrial polymerase exhibits reduced state IV respiration and enhanced cardiac fibrosis.

Koczor CA, Torres RA, Fields E, Qin Q, Park J, Ludaway T, Russ R, Lewis W.

Lab Invest. 2013 Feb;93(2):151-8. doi: 10.1038/labinvest.2012.146. Epub 2012 Oct 22.


Murine cardiac mtDNA: effects of transgenic manipulation of nucleoside phosphorylation.

Kohler JJ, Hosseini SH, Cucoranu I, Hoying-Brandt A, Green E, Johnson D, Wittich B, Srivastava J, Ivey K, Fields E, Russ R, Raper CM, Santoianni R, Lewis W.

Lab Invest. 2009 Feb;89(2):122-30. doi: 10.1038/labinvest.2008.121. Epub 2008 Dec 15.


Antiretroviral nucleosides, deoxynucleotide carrier and mitochondrial DNA: evidence supporting the DNA pol gamma hypothesis.

Lewis W, Kohler JJ, Hosseini SH, Haase CP, Copeland WC, Bienstock RJ, Ludaway T, McNaught J, Russ R, Stuart T, Santoianni R.

AIDS. 2006 Mar 21;20(5):675-84.


Transgenic mitochondrial superoxide dismutase and mitochondrially targeted catalase prevent antiretroviral-induced oxidative stress and cardiomyopathy.

Kohler JJ, Cucoranu I, Fields E, Green E, He S, Hoying A, Russ R, Abuin A, Johnson D, Hosseini SH, Raper CM, Lewis W.

Lab Invest. 2009 Jul;89(7):782-90. doi: 10.1038/labinvest.2009.39. Epub 2009 Apr 27.


Genetic and chemical rescue of the Saccharomyces cerevisiae phenotype induced by mitochondrial DNA polymerase mutations associated with progressive external ophthalmoplegia in humans.

Baruffini E, Lodi T, Dallabona C, Puglisi A, Zeviani M, Ferrero I.

Hum Mol Genet. 2006 Oct 1;15(19):2846-55. Epub 2006 Aug 29.


Mitochondrial DNA polymerase-gamma and human disease.

Hudson G, Chinnery PF.

Hum Mol Genet. 2006 Oct 15;15 Spec No 2:R244-52. Review.


Defects in mitochondrial DNA replication and oxidative damage in muscle of mtDNA mutator mice.

Kolesar JE, Safdar A, Abadi A, MacNeil LG, Crane JD, Tarnopolsky MA, Kaufman BA.

Free Radic Biol Med. 2014 Oct;75:241-51. doi: 10.1016/j.freeradbiomed.2014.07.038. Epub 2014 Aug 12.


Association of novel POLG mutations and multiple mitochondrial DNA deletions with variable clinical phenotypes in a Spanish population.

González-Vioque E, Blázquez A, Fernández-Moreira D, Bornstein B, Bautista J, Arpa J, Navarro C, Campos Y, Fernández-Moreno MA, Garesse R, Arenas J, Martín MA.

Arch Neurol. 2006 Jan;63(1):107-11.


mip1 containing mutations associated with mitochondrial disease causes mutagenesis and depletion of mtDNA in Saccharomyces cerevisiae.

Stumpf JD, Bailey CM, Spell D, Stillwagon M, Anderson KS, Copeland WC.

Hum Mol Genet. 2010 Jun 1;19(11):2123-33. doi: 10.1093/hmg/ddq089. Epub 2010 Feb 25.


Increase in mitochondrial DNA mutations impairs retinal function and renders the retina vulnerable to injury.

Kong YX, Van Bergen N, Trounce IA, Bui BV, Chrysostomou V, Waugh H, Vingrys A, Crowston JG.

Aging Cell. 2011 Aug;10(4):572-83. doi: 10.1111/j.1474-9726.2011.00690.x. Epub 2011 Apr 7.


Targeted transgenic overexpression of mitochondrial thymidine kinase (TK2) alters mitochondrial DNA (mtDNA) and mitochondrial polypeptide abundance: transgenic TK2, mtDNA, and antiretrovirals.

Hosseini SH, Kohler JJ, Haase CP, Tioleco N, Stuart T, Keebaugh E, Ludaway T, Russ R, Green E, Long R, Wang L, Eriksson S, Lewis W.

Am J Pathol. 2007 Mar;170(3):865-74.


Age-dependent cardiomyopathy in mitochondrial mutator mice is attenuated by overexpression of catalase targeted to mitochondria.

Dai DF, Chen T, Wanagat J, Laflamme M, Marcinek DJ, Emond MJ, Ngo CP, Prolla TA, Rabinovitch PS.

Aging Cell. 2010 Aug;9(4):536-44. doi: 10.1111/j.1474-9726.2010.00581.x. Epub 2010 Apr 29.


Mutant POLG2 disrupts DNA polymerase gamma subunits and causes progressive external ophthalmoplegia.

Longley MJ, Clark S, Yu Wai Man C, Hudson G, Durham SE, Taylor RW, Nightingale S, Turnbull DM, Copeland WC, Chinnery PF.

Am J Hum Genet. 2006 Jun;78(6):1026-34. Epub 2006 May 4.


Active site mutation in DNA polymerase gamma associated with progressive external ophthalmoplegia causes error-prone DNA synthesis.

Ponamarev MV, Longley MJ, Nguyen D, Kunkel TA, Copeland WC.

J Biol Chem. 2002 May 3;277(18):15225-8. Epub 2002 Mar 15.


Mitochondrial DNA depletion and fatal infantile hepatic failure due to mutations in the mitochondrial polymerase γ (POLG) gene: a combined morphological/enzyme histochemical and immunocytochemical/biochemical and molecular genetic study.

Müller-Höcker J, Horvath R, Schäfer S, Hessel H, Müller-Felber W, Kühr J, Copeland WC, Seibel P.

J Cell Mol Med. 2011 Feb;15(2):445-56. doi: 10.1111/j.1582-4934.2009.00819.x.


Targeted myocardial transgenic expression of HIV Tat causes cardiomyopathy and mitochondrial damage.

Raidel SM, Haase C, Jansen NR, Russ RB, Sutliff RL, Velsor LW, Day BJ, Hoit BD, Samarel AM, Lewis W.

Am J Physiol Heart Circ Physiol. 2002 May;282(5):H1672-8.

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