**7. Conclusion**

This literature analysis highlights the broad Ca2+ deregulation in different models of OXPHOS diseases and demonstrates the cross regulation between Ca2+ and bioenergetics in the development of mitochondrial and cellular pathologies. Some studies revealed also the potential use of Ca2+ modulating drugs to reveres mitochondrial pathology. These studies may encourage researcher to investigate systematically Ca2+ deregulation in OXPHOS and help to reveal new targets for the development of new or combined therapies to rescue mitochondrial pathology in these diseases.

## **8. Acknowledgements**

Work presented in this review has been supported by INSERM, CNRS, (AFM (11456 and 13291) and FRM (DEQ20071210550). We gratefully acknowledge INSERM for supporting the MD-PhD curriculum (Ecole de l'INSERM) of B. Oulès and the Italian Institute of Technology, Genova, Italy for supporting the PhD curriculum of Dolores Del Prete.
