OXPHOS gene expression and control in mitochondrial disorders.

  title={OXPHOS gene expression and control in mitochondrial disorders.},
  author={Fimmie Reinecke and Jan A M Smeitink and Francois Hendrikus van der Westhuizen},
  journal={Biochimica et biophysica acta},
  volume={1792 12},
The cellular consequences of deficiencies of the mitochondrial OXPHOS system include a variety of direct and secondary changes in metabolite homeostasis, such as ROS, Ca(2+), ADP/ATP, and NAD/NADH. The adaptive responses to these changes include the transcriptional responses of nuclear and mitochondrial genes that are mediated by these metabolites, control of the mitochondria permeability transition pore, and a great variety of secondary signalling elements. Among the transcriptional responses… CONTINUE READING


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