Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts.

@article{Onopiuk2009MutationID,
  title={Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts.},
  author={Marta Onopiuk and Wojciech Brutkowski and Katarzyna Wierzbicka and Sylwia Wojciechowska and Joanna Szczepanowska and Jan Fronk and Hanns Lochm{\"u}ller and Dariusz C G{\'o}recki and Krzysztof Zabłocki},
  journal={Biochemical and biophysical research communications},
  year={2009},
  volume={386 3},
  pages={
          463-6
        }
}
Duchenne Muscular Dystrophy is characterized by severe defects in differentiated muscle fibers, including abnormal calcium homeostasis and impaired cellular energy metabolism. Here we demonstrate that myoblasts derived from dystrophic (mdx) mouse exhibit reduced oxygen consumption, increased mitochondrial membrane potential, enhanced reactive oxygen species formation, stimulated glycolysis but unaffected total cellular ATP content. Moreover, reduced amounts of specific subunits of the… CONTINUE READING
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