PMID: 9655182Jul 9, 1998Paper

Impaired mitochondrial oxidative phosphorylation in skeletal muscle of the dystrophin-deficient mdx mouse

Molecular and Cellular Biochemistry
A V KuznetsovW S Kunz

Abstract

The mdx mouse, an animal model of the Duchenne muscular dystrophy, was used for the investigation of changes in mitochondrial function associated with dystrophin deficiency. Enzymatic analysis of skeletal muscle showed an approximately 50% decrease in the activity of all respiratory chain-linked enzymes in musculus quadriceps of adult mdx mice as compared with controls, while in cardiac muscle no difference was observed. The activities of cytosolic and mitochondrial matrix enzymes were not significantly different from the control values in both cardiac and skeletal muscles. In saponin-permeabilized skeletal muscle fibers of mdx mice the maximal rates of mitochondrial respiration were about two times lower than those of controls. These changes were also demonstrated on the level of isolated mitochondria. Mdx muscle mitochondria had only 60% of maximal respiration activities of control mice skeletal muscle mitochondria and contained only about 60% of hemoproteins of mitochondrial inner membrane. Similar findings were observed in a skeletal muscle biopsy of a Duchenne muscular dystrophy patient. These data strongly suggest that a specific decrease in the amount of all mitochondrial inner membrane enzymes, most probably as result o...Continue Reading

Citations

Oct 13, 2000·The Journal of Biological Chemistry·V RobertT Pozzan
Nov 25, 2005·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·R TurkP A C 't Hoen
Dec 14, 2011·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·Rosemary A SchuhEspen E Spangenburg
Oct 10, 2013·British Journal of Pharmacology·J C Komen, D R Thorburn
Feb 7, 2016·American Journal of Physiology. Heart and Circulatory Physiology·Zachary StelterDeWayne Townsend
Jun 21, 2017·Proteomics. Clinical Applications·Stephanie J CarrAndreas Roos
Oct 6, 2018·British Journal of Clinical Pharmacology·Sam AminFinbar O'Callaghan
Mar 9, 2019·NMR in Biomedicine·Brittany Lee-McMullenGlenn A Walter
Aug 15, 2002·American Journal of Physiology. Cell Physiology·Karl RougerJean J Leger
Nov 15, 2001·Journal of Neuropathology and Experimental Neurology·S VielhaberW S Kunz
Nov 25, 2006·Sports Medicine·Sébastien RatelCraig A Williams
Aug 19, 2007·The Journal of Biological Chemistry·Christoph HandschinBruce M Spiegelman
Apr 25, 2006·Muscle & Nerve·Dawn A LoweRobert W Grange
Sep 26, 2008·Muscle & Nerve·Rachel M LandischDawn A Lowe
Jan 19, 2010·Muscle & Nerve·Lisiane TuonJoão Quevedo
Apr 4, 2015·Skeletal Muscle·Anna VainshteinDavid A Hood
Sep 20, 2015·Human Molecular Genetics·Thomas C RobertsMatthew J A Wood
Nov 12, 2016·Cell Death and Differentiation·Maria C VilaJyoti K Jaiswal
Jun 7, 2017·Physical Biology·Ryan D Welch, Colin A Flaveny
Nov 27, 2018·Human Molecular Genetics·Angus LindsayJames M Ervasti
Apr 24, 2009·Pflügers Archiv : European journal of physiology·Vyacheslav M ShkrylNatalia Shirokova
May 19, 2010·Proceedings of the National Academy of Sciences of the United States of America·Wei ChenRobert G Roeder
Jul 24, 2012·Molecular Neurobiology·Clara De Palma, Emilio Clementi
Nov 6, 2013·Proceedings of the National Academy of Sciences of the United States of America·Kelly Hyunju Oh, Hongkyun Kim

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