Microscale oxygraphy reveals OXPHOS impairment in MRC mutant cells

  title={Microscale oxygraphy reveals OXPHOS impairment in MRC mutant cells},
  author={Federica Invernizzi and Ippolita D'Amato and P Jensen and Sabrina Ravaglia and Massimo Zeviani and Valeria Tiranti},
Given the complexity of the respiratory chain structure, assembly and regulation, the diagnostic workout for the identification of defects of oxidative phosphorylation (OXPHOS) is a major challenge. Spectrophotometric assays, that measure the activity of individual respiratory complexes in tissue and cell homogenates or isolated mitochondria, are highly specific, but their utilization is limited by the availability of sufficient biological material and intrinsic sensitivity. A further… CONTINUE READING