Increased mitochondrial mass in mitochondrial myopathy mice.

  title={Increased mitochondrial mass in mitochondrial myopathy mice.},
  author={Anna Wredenberg and Rolf Wibom and Hans Wilhelmsson and Caroline Graff and Heidi H. Wiener and Steven J Burden and Anders Oldfors and H{\aa}kan Westerblad and Nils-G{\"o}ran Larsson},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  volume={99 23},
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondrial transcription factor A (Tfam) in skeletal muscle of the mouse. The knockout animals developed a myopathy with ragged-red muscle fibers, accumulation of abnormally appearing mitochondria, and progressively deteriorating respiratory chain function in skeletal muscle. Enzyme histochemistry, electron micrographs, and citrate synthase activity revealed a substantial increase in mitochondrial mass in… CONTINUE READING