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Mitochondrial disease in superoxide dismutase 2 mutant mice

Oxidative stress has been implicated in many diseases. The chief source of reactive oxygen species within the cell is the mitochondrion. We have characterized a variety of the biochemical and metabolic effects of inactivation of the mouse gene for the mitochondrial superoxide dismutase (CD1-Sod2(tm1...

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Main Authors:	Melov, Simon, Coskun, Pinar, Patel, Manisha, Tuinstra, Robbyn, Cottrell, Barbara, Jun, Albert S., Zastawny, Tomsz H., Dizdaroglu, Miral, Goodman, Stephen I., Huang, Ting-Ting, Miziorko, Henry, Epstein, Charles J., Wallace, Douglas C., Breckenridge, David G., Watanabe, Yoh-ichi, Greenwood, Spencer J., Gray, Michael W., Schnare, Murray N., Zhang, Xue-Nong, Zhu, Jingya, Spudich, John L., Russ, William P., Engelman, Donald M., Perbal, Bernard, Martinerie, Cécile, Sainson, Richard, Werner, Michel, He, Bin, Roizman, Bernard, Ramesh, Vaidyanathan, Mayer, Christine, Dyson, Michael R., Gite, Sadanand, RajBhandary, Uttam L., Liu, Qiyong, Wang, James C., Hassouni, Mohammed El, Chambost, Jean Pierre, Expert, Dominique, Van Gijsegem, Frederique, Barras, Frederic, Ha, Taekjip, Ting, Alice Y., Liang, Joy, Caldwell, W. Brett, Deniz, Ashok A., Chemla, Daniel S., Schultz, Peter G., Weiss, Shimon, Farver, Ole, Lu, Yi, Ang, Majorie C., Pecht, Israel, Siedlecka, Monika, Goch, Grażyna, Ejchart, Andrzej, Sticht, Heinrich, Bierzyǹski, Andrzej, Jäntti, Jussi, Lahdenranta, Johanna, Olkkonen, Vesa M., Söderlund, Hans, Keränen, Sirkka, Chatterton, Jon E., Hirsch, David, Schwartz, John J., Bickel, Perry E., Rosenberg, Robert D., Lodish, Harvey F., Krieger, Monty, Rotsch, Christian, Jacobson, Ken, Radmacher, Manfred, Tailor, Chetankumar S., Nouri, Ali, Lee, Chun G., Kozak, Christine, Kabat, David, Wan, Katherine M., Nickerson, Jeffrey A., Krockmalnic, Gabriela, Penman, Sheldon, Zhang, Yinghua, Gardina, Paul J., Kuebler, Ann S., Kang, Hui S., Christopher, Jon A., Manson, Michael D., Kasai, Haruo, Kishimoto, Takuya, Liu, Ting-Ting, Miyashita, Yasushi, Podini, Paola, Grohovaz, Fabio, Meldolesi, Jacopo, Tevosian, Sergei G., Deconinck, Anne E., Cantor, Alan B., Rieff, Heather I., Fujiwara, Yuko, Corfas, Gabriel, Orkin, Stuart H., Svensson, Eric C., Tufts, Rachel L., Polk, Christine E., Leiden, Jeffrey M., Huang, Haochu, Marsh-Armstrong, Nicholas, Brown, Donald D., Herman, Tory, Hartwieg, Erika, Horvitz, H. Robert, Mazza, Carlos A., Zavala, Jorge, Scopel, Ana L., Ballaré, Carlos L., Hansen, Thomas F., Stenseth, Nils C., Henttonen, Heikki, Tast, Johan, Avise, John C., Walker, DeEtte, Engel, Isaac, Murre, Cornelis, Brugarolas, James, Moberg, Ken, Boyd, Scott D., Taya, Yoichi, Jacks, Tyler, Lees, Jacqueline A., Stephan, Dietrich A., Gillanders, Elizabeth, Vanderveen, Deborah, Freas-Lutz, Diana, Wistow, Graeme, Baxevanis, Andreas D., Robbins, Christiane M., Van Auken, Ann, Quesenberry, Matthew I., Bailey-Wilson, Joan, Juo, Suh-Hang Hank, Trent, Jeffrey M., Smith, Lois, Brownstein, Michael J., Baron, Udo, Schnappinger, Dirk, Helbl, Vera, Gossen, Manfred, Hillen, Wolfgang, Bujard, Hermann, González-Cabrero, Jesús, Wise, Catherine J., Latchman, Yvette, Freeman, Gordon J., Sharpe, Arlene H., Reiser, Hans, Yamashita, Masakatsu, Kimura, Motoko, Kubo, Masato, Shimizu, Chiori, Tada, Tomio, Perlmutter, Roger M., Nakayama, Toshinori, Stranford, Sharon A., Skurnick, Joan, Louria, Donald, Osmond, Dennis, Chang, Sheng-Yung, Sninsky, John, Ferrari, Guido, Weinhold, Kent, Lindquist, Craig, Levy, Jay A., Pulendran, B., Smith, J. L., Caspary, G., Brasel, K., Pettit, D., Maraskovsky, E., Maliszewski, C. R., Li, Yongan, Kang, Jian, Friedman, Joshua, Tarassishin, Leonid, Ye, Jianjiang, Kovalenko, Andrei, Wallach, David, Horwitz, Marshall S., Vemuri, Ramesh, Lankford, Edward B., Poetter, Karl, Hassanzadeh, Shahin, Takeda, Kazuyo, Yu, Zu-Xi, Ferrans, Victor J., Epstein, Neal D., Bank, Ruud A., Robins, Simon P., Wijmenga, Cisca, Breslau-Siderius, Liesbeth J., Bardoel, Alfons F. J., Van der Sluijs, Hans A., Pruijs, Hans E. H., TeKoppele, Johan M., Dhabhar, Firdaus S., McEwen, Bruce S., Waugh, J. M., Kattash, M., Li, J., Yuksel, E., Kuo, M. D., Lussier, M., Weinfeld, A. B., Saxena, R., Rabinovsky, E. D., Thung, S., Woo, S. L. C., Shenaq, S. M., Lin, Wei, Fullner, Karla Jean, Clayton, Rebecca, Sexton, Jessica A., Rogers, Marc B., Calia, Kerstin E., Calderwood, Stephen B., Fraser, Claire, Mekalanos, John J., Dawid, Suzanne, Barenkamp, Stephen J., St. Geme, Joseph W., Blais, Brian S., Shouval, Harel Z., Cooper, Leon N., Tanaka, M., Cummins, T. R., Ishikawa, K., Black, J. A., Ibata, Y., Waxman, S. G., Ashery, Uri, Koch, Henriette, Scheuss, Volker, Brose, Nils, Rettig, Jens, Song, Ji-Ying, Ichtchenko, Konstantin, Südhof, Thomas C., Villa, Alessandro E. P., Tetko, Igor V., Hyland, Brian, Najem, Abdellatif, Wells, David G., McKechnie, Beth A., Kelkar, Samir, Fallon, Justin R., Vogt, Kaspar E., Nicoll, Roger A., Fleming, Ingrid, Bauersachs, Johann, Schäfer, Andreas, Scholz, Dimitri, Aldershvile, Jan, Busse, Rudi, Brooks, George A., Dubouchaud, Hervé, Brown, Marcia, Sicurello, James P., Butz, C. Eric
Formato:	Artigo
Idioma:	English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English
Publicado em:	The National Academy of Sciences 1999
Assuntos:	Biological Sciences
Acesso em linha:	https://ncbi.nlm.nih.gov/pmc/articles/PMC15313/ https://ncbi.nlm.nih.gov/pubmed/9927656 https://ncbi.nlm.nih.gov/pmc/articles/PMC15314/ https://ncbi.nlm.nih.gov/pubmed/9927657 https://ncbi.nlm.nih.gov/pmc/articles/PMC15315/ https://ncbi.nlm.nih.gov/pubmed/9927658 https://ncbi.nlm.nih.gov/pmc/articles/PMC15316/ https://ncbi.nlm.nih.gov/pubmed/9927659 https://ncbi.nlm.nih.gov/pmc/articles/PMC15317/ https://ncbi.nlm.nih.gov/pubmed/9927660 https://ncbi.nlm.nih.gov/pmc/articles/PMC15318/ https://ncbi.nlm.nih.gov/pubmed/9927661 https://ncbi.nlm.nih.gov/pmc/articles/PMC15319/ https://ncbi.nlm.nih.gov/pubmed/9927662 https://ncbi.nlm.nih.gov/pmc/articles/PMC15320/ https://ncbi.nlm.nih.gov/pubmed/9927663 https://ncbi.nlm.nih.gov/pmc/articles/PMC15321/ https://ncbi.nlm.nih.gov/pubmed/9927664 https://ncbi.nlm.nih.gov/pmc/articles/PMC15322/ https://ncbi.nlm.nih.gov/pubmed/9927665 https://ncbi.nlm.nih.gov/pmc/articles/PMC15323/ https://ncbi.nlm.nih.gov/pubmed/9927666 https://ncbi.nlm.nih.gov/pmc/articles/PMC15324/ https://ncbi.nlm.nih.gov/pubmed/9927667 https://ncbi.nlm.nih.gov/pmc/articles/PMC15325/ https://ncbi.nlm.nih.gov/pubmed/9927668 https://ncbi.nlm.nih.gov/pmc/articles/PMC15326/ https://ncbi.nlm.nih.gov/pubmed/9927669 https://ncbi.nlm.nih.gov/pmc/articles/PMC15327/ https://ncbi.nlm.nih.gov/pubmed/9927670 https://ncbi.nlm.nih.gov/pmc/articles/PMC15328/ https://ncbi.nlm.nih.gov/pubmed/9927671 https://ncbi.nlm.nih.gov/pmc/articles/PMC15329/ https://ncbi.nlm.nih.gov/pubmed/9927672 https://ncbi.nlm.nih.gov/pmc/articles/PMC15330/ https://ncbi.nlm.nih.gov/pubmed/9927673 https://ncbi.nlm.nih.gov/pmc/articles/PMC15331/ https://ncbi.nlm.nih.gov/pubmed/9927674 https://ncbi.nlm.nih.gov/pmc/articles/PMC15332/ https://ncbi.nlm.nih.gov/pubmed/9927675 https://ncbi.nlm.nih.gov/pmc/articles/PMC15333/ https://ncbi.nlm.nih.gov/pubmed/9927676 https://ncbi.nlm.nih.gov/pmc/articles/PMC15334/ https://ncbi.nlm.nih.gov/pubmed/9927677 https://ncbi.nlm.nih.gov/pmc/articles/PMC15335/ https://ncbi.nlm.nih.gov/pubmed/9927678 https://ncbi.nlm.nih.gov/pmc/articles/PMC15336/ https://ncbi.nlm.nih.gov/pubmed/9927679 https://ncbi.nlm.nih.gov/pmc/articles/PMC15337/ https://ncbi.nlm.nih.gov/pubmed/9927680 https://ncbi.nlm.nih.gov/pmc/articles/PMC15338/ https://ncbi.nlm.nih.gov/pubmed/9927681 https://ncbi.nlm.nih.gov/pmc/articles/PMC15339/ https://ncbi.nlm.nih.gov/pubmed/9927682 https://ncbi.nlm.nih.gov/pmc/articles/PMC15340/ https://ncbi.nlm.nih.gov/pubmed/9927683 https://ncbi.nlm.nih.gov/pmc/articles/PMC15341/ https://ncbi.nlm.nih.gov/pubmed/9927684 https://ncbi.nlm.nih.gov/pmc/articles/PMC15342/ https://ncbi.nlm.nih.gov/pubmed/9927685 https://ncbi.nlm.nih.gov/pmc/articles/PMC15343/ https://ncbi.nlm.nih.gov/pubmed/9927686 https://ncbi.nlm.nih.gov/pmc/articles/PMC15344/ https://ncbi.nlm.nih.gov/pubmed/9927687 https://ncbi.nlm.nih.gov/pmc/articles/PMC15345/ https://ncbi.nlm.nih.gov/pubmed/9927688 https://ncbi.nlm.nih.gov/pmc/articles/PMC15346/ https://ncbi.nlm.nih.gov/pubmed/9927689 https://ncbi.nlm.nih.gov/pmc/articles/PMC15347/ https://ncbi.nlm.nih.gov/pubmed/9927690 https://ncbi.nlm.nih.gov/pmc/articles/PMC15348/ https://ncbi.nlm.nih.gov/pubmed/9927691 https://ncbi.nlm.nih.gov/pmc/articles/PMC15349/ https://ncbi.nlm.nih.gov/pubmed/9927692 https://ncbi.nlm.nih.gov/pmc/articles/PMC15350/ https://ncbi.nlm.nih.gov/pubmed/9927693 https://ncbi.nlm.nih.gov/pmc/articles/PMC15351/ https://ncbi.nlm.nih.gov/pubmed/9927694 https://ncbi.nlm.nih.gov/pmc/articles/PMC15352/ https://ncbi.nlm.nih.gov/pubmed/9927695 https://ncbi.nlm.nih.gov/pmc/articles/PMC15353/ https://ncbi.nlm.nih.gov/pubmed/9927696 https://ncbi.nlm.nih.gov/pmc/articles/PMC15354/ https://ncbi.nlm.nih.gov/pubmed/9927697 https://ncbi.nlm.nih.gov/pmc/articles/PMC15355/ https://ncbi.nlm.nih.gov/pubmed/9927698 https://ncbi.nlm.nih.gov/pmc/articles/PMC15356/ https://ncbi.nlm.nih.gov/pubmed/9927699 https://ncbi.nlm.nih.gov/pmc/articles/PMC15357/ https://ncbi.nlm.nih.gov/pubmed/9927700 https://ncbi.nlm.nih.gov/pmc/articles/PMC15358/ https://ncbi.nlm.nih.gov/pubmed/9927701 https://ncbi.nlm.nih.gov/pmc/articles/PMC15359/ https://ncbi.nlm.nih.gov/pubmed/9927702 https://ncbi.nlm.nih.gov/pmc/articles/PMC15360/ https://ncbi.nlm.nih.gov/pubmed/9927703 https://ncbi.nlm.nih.gov/pmc/articles/PMC15361/ https://ncbi.nlm.nih.gov/pubmed/9927704 https://ncbi.nlm.nih.gov/pmc/articles/PMC15362/ https://ncbi.nlm.nih.gov/pubmed/9927705
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id	pubmed-15313
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spelling	pubmed-153132001-03-07 Mitochondrial disease in superoxide dismutase 2 mutant mice Melov, Simon Coskun, Pinar Patel, Manisha Tuinstra, Robbyn Cottrell, Barbara Jun, Albert S. Zastawny, Tomsz H. Dizdaroglu, Miral Goodman, Stephen I. Huang, Ting-Ting Miziorko, Henry Epstein, Charles J. Wallace, Douglas C. Proc Natl Acad Sci U S A Biological Sciences Oxidative stress has been implicated in many diseases. The chief source of reactive oxygen species within the cell is the mitochondrion. We have characterized a variety of the biochemical and metabolic effects of inactivation of the mouse gene for the mitochondrial superoxide dismutase (CD1-Sod2(tm1Cje)). The Sod2 mutant mice exhibit a tissue-specific inhibition of the respiratory chain enzymes NADH-dehydrogenase (complex I) and succinate dehydrogenase (complex II), inactivation of the tricarboxylic acid cycle enzyme aconitase, development of a urine organic aciduria in conjunction with a partial defect in 3-hydroxy-3-methylglutaryl-CoA lyase, and accumulation of oxidative DNA damage. These results indicate that the increase in mitochondrial reactive oxygen species can result in biochemical aberrations with features reminiscent of mitochondrial myopathy, Friedreich ataxia, and 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. The National Academy of Sciences 1999-02-02 /pmc/articles/PMC15313/ /pubmed/9927656 Text en Copyright © 1999, The National Academy of Sciences
institution	US National Library of Medicine
collection	PubMed Central
language	English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English English
format	Article
topic	Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences
spellingShingle	Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Melov, Simon Coskun, Pinar Patel, Manisha Tuinstra, Robbyn Cottrell, Barbara Jun, Albert S. Zastawny, Tomsz H. Dizdaroglu, Miral Goodman, Stephen I. Huang, Ting-Ting Miziorko, Henry Epstein, Charles J. Wallace, Douglas C. Breckenridge, David G. Watanabe, Yoh-ichi Greenwood, Spencer J. Gray, Michael W. Schnare, Murray N. Zhang, Xue-Nong Zhu, Jingya Spudich, John L. Russ, William P. Engelman, Donald M. Perbal, Bernard Martinerie, Cécile Sainson, Richard Werner, Michel He, Bin Roizman, Bernard Ramesh, Vaidyanathan Mayer, Christine Dyson, Michael R. Gite, Sadanand RajBhandary, Uttam L. Liu, Qiyong Wang, James C. Hassouni, Mohammed El Chambost, Jean Pierre Expert, Dominique Van Gijsegem, Frederique Barras, Frederic Ha, Taekjip Ting, Alice Y. Liang, Joy Caldwell, W. Brett Deniz, Ashok A. Chemla, Daniel S. Schultz, Peter G. Weiss, Shimon Farver, Ole Lu, Yi Ang, Majorie C. Pecht, Israel Siedlecka, Monika Goch, Grażyna Ejchart, Andrzej Sticht, Heinrich Bierzyǹski, Andrzej Jäntti, Jussi Lahdenranta, Johanna Olkkonen, Vesa M. Söderlund, Hans Keränen, Sirkka Chatterton, Jon E. Hirsch, David Schwartz, John J. Bickel, Perry E. Rosenberg, Robert D. Lodish, Harvey F. Krieger, Monty Rotsch, Christian Jacobson, Ken Radmacher, Manfred Tailor, Chetankumar S. Nouri, Ali Lee, Chun G. Kozak, Christine Kabat, David Wan, Katherine M. Nickerson, Jeffrey A. Krockmalnic, Gabriela Penman, Sheldon Zhang, Yinghua Gardina, Paul J. Kuebler, Ann S. Kang, Hui S. Christopher, Jon A. Manson, Michael D. Kasai, Haruo Kishimoto, Takuya Liu, Ting-Ting Miyashita, Yasushi Podini, Paola Grohovaz, Fabio Meldolesi, Jacopo Tevosian, Sergei G. Deconinck, Anne E. Cantor, Alan B. Rieff, Heather I. Fujiwara, Yuko Corfas, Gabriel Orkin, Stuart H. Svensson, Eric C. Tufts, Rachel L. Polk, Christine E. Leiden, Jeffrey M. Huang, Haochu Marsh-Armstrong, Nicholas Brown, Donald D. Herman, Tory Hartwieg, Erika Horvitz, H. Robert Herman, Tory Horvitz, H. Robert Mazza, Carlos A. Zavala, Jorge Scopel, Ana L. Ballaré, Carlos L. Hansen, Thomas F. Stenseth, Nils C. Henttonen, Heikki Tast, Johan Avise, John C. Walker, DeEtte Engel, Isaac Murre, Cornelis Brugarolas, James Moberg, Ken Boyd, Scott D. Taya, Yoichi Jacks, Tyler Lees, Jacqueline A. Stephan, Dietrich A. Gillanders, Elizabeth Vanderveen, Deborah Freas-Lutz, Diana Wistow, Graeme Baxevanis, Andreas D. Robbins, Christiane M. Van Auken, Ann Quesenberry, Matthew I. Bailey-Wilson, Joan Juo, Suh-Hang Hank Trent, Jeffrey M. Smith, Lois Brownstein, Michael J. Baron, Udo Schnappinger, Dirk Helbl, Vera Gossen, Manfred Hillen, Wolfgang Bujard, Hermann González-Cabrero, Jesús Wise, Catherine J. Latchman, Yvette Freeman, Gordon J. Sharpe, Arlene H. Reiser, Hans Yamashita, Masakatsu Kimura, Motoko Kubo, Masato Shimizu, Chiori Tada, Tomio Perlmutter, Roger M. Nakayama, Toshinori Stranford, Sharon A. Skurnick, Joan Louria, Donald Osmond, Dennis Chang, Sheng-Yung Sninsky, John Ferrari, Guido Weinhold, Kent Lindquist, Craig Levy, Jay A. Pulendran, B. Smith, J. L. Caspary, G. Brasel, K. Pettit, D. Maraskovsky, E. Maliszewski, C. R. Li, Yongan Kang, Jian Friedman, Joshua Tarassishin, Leonid Ye, Jianjiang Kovalenko, Andrei Wallach, David Horwitz, Marshall S. Vemuri, Ramesh Lankford, Edward B. Poetter, Karl Hassanzadeh, Shahin Takeda, Kazuyo Yu, Zu-Xi Ferrans, Victor J. Epstein, Neal D. Bank, Ruud A. Robins, Simon P. Wijmenga, Cisca Breslau-Siderius, Liesbeth J. Bardoel, Alfons F. J. Van der Sluijs, Hans A. Pruijs, Hans E. H. TeKoppele, Johan M. Dhabhar, Firdaus S. McEwen, Bruce S. Waugh, J. M. Kattash, M. Li, J. Yuksel, E. Kuo, M. D. Lussier, M. Weinfeld, A. B. Saxena, R. Rabinovsky, E. D. Thung, S. Woo, S. L. C. Shenaq, S. M. Lin, Wei Fullner, Karla Jean Clayton, Rebecca Sexton, Jessica A. Rogers, Marc B. Calia, Kerstin E. Calderwood, Stephen B. Fraser, Claire Mekalanos, John J. Dawid, Suzanne Barenkamp, Stephen J. St. Geme, Joseph W. Blais, Brian S. Shouval, Harel Z. Cooper, Leon N. Tanaka, M. Cummins, T. R. Ishikawa, K. Black, J. A. Ibata, Y. Waxman, S. G. Ashery, Uri Koch, Henriette Scheuss, Volker Brose, Nils Rettig, Jens Song, Ji-Ying Ichtchenko, Konstantin Südhof, Thomas C. Brose, Nils Villa, Alessandro E. P. Tetko, Igor V. Hyland, Brian Najem, Abdellatif Wells, David G. McKechnie, Beth A. Kelkar, Samir Fallon, Justin R. Vogt, Kaspar E. Nicoll, Roger A. Fleming, Ingrid Bauersachs, Johann Schäfer, Andreas Scholz, Dimitri Aldershvile, Jan Busse, Rudi Brooks, George A. Dubouchaud, Hervé Brown, Marcia Sicurello, James P. Butz, C. Eric Mitochondrial disease in superoxide dismutase 2 mutant mice
topic_facet	Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences Biological Sciences
description	Oxidative stress has been implicated in many diseases. The chief source of reactive oxygen species within the cell is the mitochondrion. We have characterized a variety of the biochemical and metabolic effects of inactivation of the mouse gene for the mitochondrial superoxide dismutase (CD1-Sod2(tm1Cje)). The Sod2 mutant mice exhibit a tissue-specific inhibition of the respiratory chain enzymes NADH-dehydrogenase (complex I) and succinate dehydrogenase (complex II), inactivation of the tricarboxylic acid cycle enzyme aconitase, development of a urine organic aciduria in conjunction with a partial defect in 3-hydroxy-3-methylglutaryl-CoA lyase, and accumulation of oxidative DNA damage. These results indicate that the increase in mitochondrial reactive oxygen species can result in biochemical aberrations with features reminiscent of mitochondrial myopathy, Friedreich ataxia, and 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
author	Melov, Simon Coskun, Pinar Patel, Manisha Tuinstra, Robbyn Cottrell, Barbara Jun, Albert S. Zastawny, Tomsz H. Dizdaroglu, Miral Goodman, Stephen I. Huang, Ting-Ting Miziorko, Henry Epstein, Charles J. Wallace, Douglas C. Breckenridge, David G. Watanabe, Yoh-ichi Greenwood, Spencer J. Gray, Michael W. Schnare, Murray N. Zhang, Xue-Nong Zhu, Jingya Spudich, John L. Russ, William P. Engelman, Donald M. Perbal, Bernard Martinerie, Cécile Sainson, Richard Werner, Michel He, Bin Roizman, Bernard Ramesh, Vaidyanathan Mayer, Christine Dyson, Michael R. Gite, Sadanand RajBhandary, Uttam L. Liu, Qiyong Wang, James C. Hassouni, Mohammed El Chambost, Jean Pierre Expert, Dominique Van Gijsegem, Frederique Barras, Frederic Ha, Taekjip Ting, Alice Y. Liang, Joy Caldwell, W. Brett Deniz, Ashok A. Chemla, Daniel S. Schultz, Peter G. Weiss, Shimon Farver, Ole Lu, Yi Ang, Majorie C. Pecht, Israel Siedlecka, Monika Goch, Grażyna Ejchart, Andrzej Sticht, Heinrich Bierzyǹski, Andrzej Jäntti, Jussi Lahdenranta, Johanna Olkkonen, Vesa M. Söderlund, Hans Keränen, Sirkka Chatterton, Jon E. Hirsch, David Schwartz, John J. Bickel, Perry E. Rosenberg, Robert D. Lodish, Harvey F. Krieger, Monty Rotsch, Christian Jacobson, Ken Radmacher, Manfred Tailor, Chetankumar S. Nouri, Ali Lee, Chun G. Kozak, Christine Kabat, David Wan, Katherine M. Nickerson, Jeffrey A. Krockmalnic, Gabriela Penman, Sheldon Zhang, Yinghua Gardina, Paul J. Kuebler, Ann S. Kang, Hui S. Christopher, Jon A. Manson, Michael D. Kasai, Haruo Kishimoto, Takuya Liu, Ting-Ting Miyashita, Yasushi Podini, Paola Grohovaz, Fabio Meldolesi, Jacopo Tevosian, Sergei G. Deconinck, Anne E. Cantor, Alan B. Rieff, Heather I. Fujiwara, Yuko Corfas, Gabriel Orkin, Stuart H. Svensson, Eric C. Tufts, Rachel L. Polk, Christine E. Leiden, Jeffrey M. Huang, Haochu Marsh-Armstrong, Nicholas Brown, Donald D. Herman, Tory Hartwieg, Erika Horvitz, H. Robert Herman, Tory Horvitz, H. Robert Mazza, Carlos A. Zavala, Jorge Scopel, Ana L. Ballaré, Carlos L. Hansen, Thomas F. Stenseth, Nils C. Henttonen, Heikki Tast, Johan Avise, John C. Walker, DeEtte Engel, Isaac Murre, Cornelis Brugarolas, James Moberg, Ken Boyd, Scott D. Taya, Yoichi Jacks, Tyler Lees, Jacqueline A. Stephan, Dietrich A. Gillanders, Elizabeth Vanderveen, Deborah Freas-Lutz, Diana Wistow, Graeme Baxevanis, Andreas D. Robbins, Christiane M. Van Auken, Ann Quesenberry, Matthew I. Bailey-Wilson, Joan Juo, Suh-Hang Hank Trent, Jeffrey M. Smith, Lois Brownstein, Michael J. Baron, Udo Schnappinger, Dirk Helbl, Vera Gossen, Manfred Hillen, Wolfgang Bujard, Hermann González-Cabrero, Jesús Wise, Catherine J. Latchman, Yvette Freeman, Gordon J. Sharpe, Arlene H. Reiser, Hans Yamashita, Masakatsu Kimura, Motoko Kubo, Masato Shimizu, Chiori Tada, Tomio Perlmutter, Roger M. Nakayama, Toshinori Stranford, Sharon A. Skurnick, Joan Louria, Donald Osmond, Dennis Chang, Sheng-Yung Sninsky, John Ferrari, Guido Weinhold, Kent Lindquist, Craig Levy, Jay A. Pulendran, B. Smith, J. L. Caspary, G. Brasel, K. Pettit, D. Maraskovsky, E. Maliszewski, C. R. Li, Yongan Kang, Jian Friedman, Joshua Tarassishin, Leonid Ye, Jianjiang Kovalenko, Andrei Wallach, David Horwitz, Marshall S. Vemuri, Ramesh Lankford, Edward B. Poetter, Karl Hassanzadeh, Shahin Takeda, Kazuyo Yu, Zu-Xi Ferrans, Victor J. Epstein, Neal D. Bank, Ruud A. Robins, Simon P. Wijmenga, Cisca Breslau-Siderius, Liesbeth J. Bardoel, Alfons F. J. Van der Sluijs, Hans A. Pruijs, Hans E. H. TeKoppele, Johan M. Dhabhar, Firdaus S. McEwen, Bruce S. Waugh, J. M. Kattash, M. Li, J. Yuksel, E. Kuo, M. D. Lussier, M. Weinfeld, A. B. Saxena, R. Rabinovsky, E. D. Thung, S. Woo, S. L. C. Shenaq, S. M. Lin, Wei Fullner, Karla Jean Clayton, Rebecca Sexton, Jessica A. Rogers, Marc B. Calia, Kerstin E. Calderwood, Stephen B. Fraser, Claire Mekalanos, John J. Dawid, Suzanne Barenkamp, Stephen J. St. Geme, Joseph W. Blais, Brian S. Shouval, Harel Z. Cooper, Leon N. Tanaka, M. Cummins, T. R. Ishikawa, K. Black, J. A. Ibata, Y. Waxman, S. G. Ashery, Uri Koch, Henriette Scheuss, Volker Brose, Nils Rettig, Jens Song, Ji-Ying Ichtchenko, Konstantin Südhof, Thomas C. Brose, Nils Villa, Alessandro E. P. Tetko, Igor V. Hyland, Brian Najem, Abdellatif Wells, David G. McKechnie, Beth A. Kelkar, Samir Fallon, Justin R. Vogt, Kaspar E. Nicoll, Roger A. Fleming, Ingrid Bauersachs, Johann Schäfer, Andreas Scholz, Dimitri Aldershvile, Jan Busse, Rudi Brooks, George A. Dubouchaud, Hervé Brown, Marcia Sicurello, James P. Butz, C. Eric
author_facet	Melov, Simon Coskun, Pinar Patel, Manisha Tuinstra, Robbyn Cottrell, Barbara Jun, Albert S. Zastawny, Tomsz H. Dizdaroglu, Miral Goodman, Stephen I. Huang, Ting-Ting Miziorko, Henry Epstein, Charles J. Wallace, Douglas C. Breckenridge, David G. Watanabe, Yoh-ichi Greenwood, Spencer J. Gray, Michael W. Schnare, Murray N. Zhang, Xue-Nong Zhu, Jingya Spudich, John L. Russ, William P. Engelman, Donald M. Perbal, Bernard Martinerie, Cécile Sainson, Richard Werner, Michel He, Bin Roizman, Bernard Ramesh, Vaidyanathan Mayer, Christine Dyson, Michael R. Gite, Sadanand RajBhandary, Uttam L. Liu, Qiyong Wang, James C. Hassouni, Mohammed El Chambost, Jean Pierre Expert, Dominique Van Gijsegem, Frederique Barras, Frederic Ha, Taekjip Ting, Alice Y. Liang, Joy Caldwell, W. Brett Deniz, Ashok A. Chemla, Daniel S. Schultz, Peter G. Weiss, Shimon Farver, Ole Lu, Yi Ang, Majorie C. Pecht, Israel Siedlecka, Monika Goch, Grażyna Ejchart, Andrzej Sticht, Heinrich Bierzyǹski, Andrzej Jäntti, Jussi Lahdenranta, Johanna Olkkonen, Vesa M. Söderlund, Hans Keränen, Sirkka Chatterton, Jon E. Hirsch, David Schwartz, John J. Bickel, Perry E. Rosenberg, Robert D. Lodish, Harvey F. Krieger, Monty Rotsch, Christian Jacobson, Ken Radmacher, Manfred Tailor, Chetankumar S. Nouri, Ali Lee, Chun G. Kozak, Christine Kabat, David Wan, Katherine M. Nickerson, Jeffrey A. Krockmalnic, Gabriela Penman, Sheldon Zhang, Yinghua Gardina, Paul J. Kuebler, Ann S. Kang, Hui S. Christopher, Jon A. Manson, Michael D. Kasai, Haruo Kishimoto, Takuya Liu, Ting-Ting Miyashita, Yasushi Podini, Paola Grohovaz, Fabio Meldolesi, Jacopo Tevosian, Sergei G. Deconinck, Anne E. Cantor, Alan B. Rieff, Heather I. Fujiwara, Yuko Corfas, Gabriel Orkin, Stuart H. Svensson, Eric C. Tufts, Rachel L. Polk, Christine E. Leiden, Jeffrey M. Huang, Haochu Marsh-Armstrong, Nicholas Brown, Donald D. Herman, Tory Hartwieg, Erika Horvitz, H. Robert Herman, Tory Horvitz, H. Robert Mazza, Carlos A. Zavala, Jorge Scopel, Ana L. Ballaré, Carlos L. Hansen, Thomas F. Stenseth, Nils C. Henttonen, Heikki Tast, Johan Avise, John C. Walker, DeEtte Engel, Isaac Murre, Cornelis Brugarolas, James Moberg, Ken Boyd, Scott D. Taya, Yoichi Jacks, Tyler Lees, Jacqueline A. Stephan, Dietrich A. Gillanders, Elizabeth Vanderveen, Deborah Freas-Lutz, Diana Wistow, Graeme Baxevanis, Andreas D. Robbins, Christiane M. Van Auken, Ann Quesenberry, Matthew I. Bailey-Wilson, Joan Juo, Suh-Hang Hank Trent, Jeffrey M. Smith, Lois Brownstein, Michael J. Baron, Udo Schnappinger, Dirk Helbl, Vera Gossen, Manfred Hillen, Wolfgang Bujard, Hermann González-Cabrero, Jesús Wise, Catherine J. Latchman, Yvette Freeman, Gordon J. Sharpe, Arlene H. Reiser, Hans Yamashita, Masakatsu Kimura, Motoko Kubo, Masato Shimizu, Chiori Tada, Tomio Perlmutter, Roger M. Nakayama, Toshinori Stranford, Sharon A. Skurnick, Joan Louria, Donald Osmond, Dennis Chang, Sheng-Yung Sninsky, John Ferrari, Guido Weinhold, Kent Lindquist, Craig Levy, Jay A. Pulendran, B. Smith, J. L. Caspary, G. Brasel, K. Pettit, D. Maraskovsky, E. Maliszewski, C. R. Li, Yongan Kang, Jian Friedman, Joshua Tarassishin, Leonid Ye, Jianjiang Kovalenko, Andrei Wallach, David Horwitz, Marshall S. Vemuri, Ramesh Lankford, Edward B. Poetter, Karl Hassanzadeh, Shahin Takeda, Kazuyo Yu, Zu-Xi Ferrans, Victor J. Epstein, Neal D. Bank, Ruud A. Robins, Simon P. Wijmenga, Cisca Breslau-Siderius, Liesbeth J. Bardoel, Alfons F. J. Van der Sluijs, Hans A. Pruijs, Hans E. H. TeKoppele, Johan M. Dhabhar, Firdaus S. McEwen, Bruce S. Waugh, J. M. Kattash, M. Li, J. Yuksel, E. Kuo, M. D. Lussier, M. Weinfeld, A. B. Saxena, R. Rabinovsky, E. D. Thung, S. Woo, S. L. C. Shenaq, S. M. Lin, Wei Fullner, Karla Jean Clayton, Rebecca Sexton, Jessica A. Rogers, Marc B. Calia, Kerstin E. Calderwood, Stephen B. Fraser, Claire Mekalanos, John J. Dawid, Suzanne Barenkamp, Stephen J. St. Geme, Joseph W. Blais, Brian S. Shouval, Harel Z. Cooper, Leon N. Tanaka, M. Cummins, T. R. Ishikawa, K. Black, J. A. Ibata, Y. Waxman, S. G. Ashery, Uri Koch, Henriette Scheuss, Volker Brose, Nils Rettig, Jens Song, Ji-Ying Ichtchenko, Konstantin Südhof, Thomas C. Brose, Nils Villa, Alessandro E. P. Tetko, Igor V. Hyland, Brian Najem, Abdellatif Wells, David G. McKechnie, Beth A. Kelkar, Samir Fallon, Justin R. Vogt, Kaspar E. Nicoll, Roger A. Fleming, Ingrid Bauersachs, Johann Schäfer, Andreas Scholz, Dimitri Aldershvile, Jan Busse, Rudi Brooks, George A. Dubouchaud, Hervé Brown, Marcia Sicurello, James P. Butz, C. Eric
author_sort	Melov, Simon
title	Mitochondrial disease in superoxide dismutase 2 mutant mice
title_short	Mitochondrial disease in superoxide dismutase 2 mutant mice
title_full	Mitochondrial disease in superoxide dismutase 2 mutant mice
title_fullStr	Mitochondrial disease in superoxide dismutase 2 mutant mice
title_full_unstemmed	Mitochondrial disease in superoxide dismutase 2 mutant mice
title_sort	mitochondrial disease in superoxide dismutase 2 mutant mice
publisher	The National Academy of Sciences
publisher_facet	The National Academy of Sciences
publishDate	1999
url	https://ncbi.nlm.nih.gov/pmc/articles/PMC15313/ https://ncbi.nlm.nih.gov/pubmed/9927656 https://ncbi.nlm.nih.gov/pmc/articles/PMC15314/ https://ncbi.nlm.nih.gov/pubmed/9927657 https://ncbi.nlm.nih.gov/pmc/articles/PMC15315/ https://ncbi.nlm.nih.gov/pubmed/9927658 https://ncbi.nlm.nih.gov/pmc/articles/PMC15316/ https://ncbi.nlm.nih.gov/pubmed/9927659 https://ncbi.nlm.nih.gov/pmc/articles/PMC15317/ https://ncbi.nlm.nih.gov/pubmed/9927660 https://ncbi.nlm.nih.gov/pmc/articles/PMC15318/ https://ncbi.nlm.nih.gov/pubmed/9927661 https://ncbi.nlm.nih.gov/pmc/articles/PMC15319/ https://ncbi.nlm.nih.gov/pubmed/9927662 https://ncbi.nlm.nih.gov/pmc/articles/PMC15320/ https://ncbi.nlm.nih.gov/pubmed/9927663 https://ncbi.nlm.nih.gov/pmc/articles/PMC15321/ https://ncbi.nlm.nih.gov/pubmed/9927664 https://ncbi.nlm.nih.gov/pmc/articles/PMC15322/ https://ncbi.nlm.nih.gov/pubmed/9927665 https://ncbi.nlm.nih.gov/pmc/articles/PMC15323/ https://ncbi.nlm.nih.gov/pubmed/9927666 https://ncbi.nlm.nih.gov/pmc/articles/PMC15324/ https://ncbi.nlm.nih.gov/pubmed/9927667 https://ncbi.nlm.nih.gov/pmc/articles/PMC15325/ https://ncbi.nlm.nih.gov/pubmed/9927668 https://ncbi.nlm.nih.gov/pmc/articles/PMC15326/ https://ncbi.nlm.nih.gov/pubmed/9927669 https://ncbi.nlm.nih.gov/pmc/articles/PMC15327/ https://ncbi.nlm.nih.gov/pubmed/9927670 https://ncbi.nlm.nih.gov/pmc/articles/PMC15328/ https://ncbi.nlm.nih.gov/pubmed/9927671 https://ncbi.nlm.nih.gov/pmc/articles/PMC15329/ https://ncbi.nlm.nih.gov/pubmed/9927672 https://ncbi.nlm.nih.gov/pmc/articles/PMC15330/ https://ncbi.nlm.nih.gov/pubmed/9927673 https://ncbi.nlm.nih.gov/pmc/articles/PMC15331/ https://ncbi.nlm.nih.gov/pubmed/9927674 https://ncbi.nlm.nih.gov/pmc/articles/PMC15332/ https://ncbi.nlm.nih.gov/pubmed/9927675 https://ncbi.nlm.nih.gov/pmc/articles/PMC15333/ https://ncbi.nlm.nih.gov/pubmed/9927676 https://ncbi.nlm.nih.gov/pmc/articles/PMC15334/ https://ncbi.nlm.nih.gov/pubmed/9927677 https://ncbi.nlm.nih.gov/pmc/articles/PMC15335/ https://ncbi.nlm.nih.gov/pubmed/9927678 https://ncbi.nlm.nih.gov/pmc/articles/PMC15336/ https://ncbi.nlm.nih.gov/pubmed/9927679 https://ncbi.nlm.nih.gov/pmc/articles/PMC15337/ https://ncbi.nlm.nih.gov/pubmed/9927680 https://ncbi.nlm.nih.gov/pmc/articles/PMC15338/ https://ncbi.nlm.nih.gov/pubmed/9927681 https://ncbi.nlm.nih.gov/pmc/articles/PMC15339/ https://ncbi.nlm.nih.gov/pubmed/9927682 https://ncbi.nlm.nih.gov/pmc/articles/PMC15340/ https://ncbi.nlm.nih.gov/pubmed/9927683 https://ncbi.nlm.nih.gov/pmc/articles/PMC15341/ https://ncbi.nlm.nih.gov/pubmed/9927684 https://ncbi.nlm.nih.gov/pmc/articles/PMC15342/ https://ncbi.nlm.nih.gov/pubmed/9927685 https://ncbi.nlm.nih.gov/pmc/articles/PMC15343/ https://ncbi.nlm.nih.gov/pubmed/9927686 https://ncbi.nlm.nih.gov/pmc/articles/PMC15344/ https://ncbi.nlm.nih.gov/pubmed/9927687 https://ncbi.nlm.nih.gov/pmc/articles/PMC15345/ https://ncbi.nlm.nih.gov/pubmed/9927688 https://ncbi.nlm.nih.gov/pmc/articles/PMC15346/ https://ncbi.nlm.nih.gov/pubmed/9927689 https://ncbi.nlm.nih.gov/pmc/articles/PMC15347/ https://ncbi.nlm.nih.gov/pubmed/9927690 https://ncbi.nlm.nih.gov/pmc/articles/PMC15348/ https://ncbi.nlm.nih.gov/pubmed/9927691 https://ncbi.nlm.nih.gov/pmc/articles/PMC15349/ https://ncbi.nlm.nih.gov/pubmed/9927692 https://ncbi.nlm.nih.gov/pmc/articles/PMC15350/ https://ncbi.nlm.nih.gov/pubmed/9927693 https://ncbi.nlm.nih.gov/pmc/articles/PMC15351/ https://ncbi.nlm.nih.gov/pubmed/9927694 https://ncbi.nlm.nih.gov/pmc/articles/PMC15352/ https://ncbi.nlm.nih.gov/pubmed/9927695 https://ncbi.nlm.nih.gov/pmc/articles/PMC15353/ https://ncbi.nlm.nih.gov/pubmed/9927696 https://ncbi.nlm.nih.gov/pmc/articles/PMC15354/ https://ncbi.nlm.nih.gov/pubmed/9927697 https://ncbi.nlm.nih.gov/pmc/articles/PMC15355/ https://ncbi.nlm.nih.gov/pubmed/9927698 https://ncbi.nlm.nih.gov/pmc/articles/PMC15356/ https://ncbi.nlm.nih.gov/pubmed/9927699 https://ncbi.nlm.nih.gov/pmc/articles/PMC15357/ https://ncbi.nlm.nih.gov/pubmed/9927700 https://ncbi.nlm.nih.gov/pmc/articles/PMC15358/ https://ncbi.nlm.nih.gov/pubmed/9927701 https://ncbi.nlm.nih.gov/pmc/articles/PMC15359/ https://ncbi.nlm.nih.gov/pubmed/9927702 https://ncbi.nlm.nih.gov/pmc/articles/PMC15360/ https://ncbi.nlm.nih.gov/pubmed/9927703 https://ncbi.nlm.nih.gov/pmc/articles/PMC15361/ https://ncbi.nlm.nih.gov/pubmed/9927704 https://ncbi.nlm.nih.gov/pmc/articles/PMC15362/ https://ncbi.nlm.nih.gov/pubmed/9927705
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Mitochondrial disease in superoxide dismutase 2 mutant mice

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