PMID: 9540845Apr 16, 1998Paper

Expression of mtDNA and nDNA encoded respiratory chain proteins in chemically and genetically-derived Rho0 human fibroblasts: a comparison of subunit proteins in normal fibroblasts treated with ethidium bromide and fibroblasts from a patient with mtDNA depletion syndrome

Biochimica Et Biophysica Acta
M F MarusichR A Capaldi

Abstract

Although much progress has been made in identifying genetic defects associated with mitochondrial diseases, the protein expression patterns of most disorders are poorly understood. Here we use immunochemical techniques to describe subunit expression patterns of respiratory chain enzyme complexes II (succinate dehydrogenase: SD) and IV (cytochrome c oxidase: COX) in cultured cells lacking mtDNA (Rho0 cells) derived either chemically by exposure of normal cells to ethidium bromide, or genetically in cells derived from a patient with mtDNA depletion syndrome. Both control cells and early passage patient-derived cells express a normal complement of SD and COX subunit proteins. Ethidium bromide treatment of normal cells and in vitro cell proliferation of patient-derived cells caused both populations to acquire identical Rho0 phenotypes. As expected, they lack mtDNA-encoded subunits COX-I and COX-II. In contrast, nDNA-encoded subunits are affected differentially, with some (COX-VIc) lacking and others (COX-IV, COX-Va, SD 30 and SD 70) maintained at somewhat reduced levels. We suggest that the differential stability of nDNA-encoded subunits in the absence of intact enzyme complexes is due to the ability of some, but not all, subunits ...Continue Reading

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Citations

Apr 8, 2004·Proceedings of the National Academy of Sciences of the United States of America·Jeffrey R GingrichMichael W Salter
Nov 6, 2008·PloS One·Corinne E GriguerG Yancey Gillespie
Apr 7, 2010·Molecular Interventions·Dean P JonesNeil Kaplowitz
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Feb 11, 2015·The International Journal of Biochemistry & Cell Biology·Umut Cagin, José Antonio Enriquez
Nov 20, 2015·Oxidative Medicine and Cellular Longevity·Fernanda Marques da CunhaAlicia J Kowaltowski
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Dec 7, 2005·Medicine and Science in Sports and Exercise·Beatrice ChabiDavid A Hood
Mar 30, 2001·The Journal of Biological Chemistry·B J HansonR A Capaldi

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