PMID: 11334790May 4, 2001Paper

Lack of dystrophin is associated with altered integration of the mitochondria and ATPases in slow-twitch muscle cells of MDX mice

Biochimica Et Biophysica Acta
U BraunE K Seppet

Abstract

The potential role of dystrophin-mediated control of systems integrating mitochondria with ATPases was assessed in muscle cells. Mitochondrial distribution and function in skinned cardiac and skeletal muscle fibers from dystrophin-deficient (MDX) and wild-type mice were compared. Laser confocal microscopy revealed disorganized mitochondrial arrays in m. gastrocnemius in MDX mice, whereas the other muscles appeared normal in this group. Irrespective of muscle type, the absence of dystrophin had no effect on the maximal capacity of oxidative phosphorylation, nor on coupling between oxidation and phosphorylation. However, in the myocardium and m. soleus, the coupling of mitochondrial creatine kinase to adenine nucleotide translocase was attenuated as evidenced by the decreased effect of creatine on the Km for ADP in the reactions of oxidative phosphorylation. In m. soleus, a low Km for ADP compared to the wild-type counterpart was found, which implies increased permeability for that nucleotide across the mitochondrial outer membrane. In normal cardiac fibers 35% of the ADP flux generated by ATPases was not accessible to the external pyruvate kinase-phosphoenolpyruvate system, which suggests the compartmentalized (direct) channelin...Continue Reading

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Citations

Mar 17, 2011·European Journal of Applied Physiology·D Gardan-SalmonJ T Selsby
Jul 1, 2009·International Journal of Molecular Sciences·Enn SeppetFrank N Gellerich
Jan 19, 2010·Muscle & Nerve·Lisiane TuonJoão Quevedo
Jun 16, 2004·Journal of Neuroscience Methods·Dzhamilja SafiulinaEnn Seppet
Apr 22, 2008·Biochimica Et Biophysica Acta·Margus EimreEnn Seppet
May 28, 2014·Molecular Therapy : the Journal of the American Society of Gene Therapy·Karima RelizaniHelge Amthor
Jun 28, 2014·Proceedings of the National Academy of Sciences of the United States of America·Helena M ViolaLivia C Hool
Jun 15, 2007·Free Radical Biology & Medicine·Jan J KaczorMark A Tarnopolsky
Dec 17, 2005·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·Margus EimreEnn K Seppet
Oct 28, 2011·American Journal of Physiology. Cell Physiology·Martin PicardYan Burelle
Jul 23, 2002·Journal of Applied Physiology·Jon F WatchkoEric P Hoffman
Mar 29, 2005·Molecular and Cellular Biochemistry·Evelin SeppetEnn K Seppet
May 17, 2002·Journal of Applied Physiology·Yan Burelle, Peter W Hochachka
Feb 7, 2016·American Journal of Physiology. Heart and Circulatory Physiology·Zachary StelterDeWayne Townsend

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