Calcium and ATP handling in human NADH:ubiquinone oxidoreductase deficiency

Federica Valsecchi , John J. Esseling , Werner J.H. Koopman , Peter H.G.M. Willems

Proper cell functioning requires precise coordination between mitochondrial ATP production and local energy demand. Ionic calcium (Ca) plays a central role in this coupling because it activates mitochondrial oxidative phosphorylation (OXPHOS) during hormonal and electrical cell stimulation. To determine how mitochondrial dysfunction affects cytosolic and mitochondrial Ca/ATP handling, we performed life-cell quantification of these parameters in fibroblast cell lines derived from healthy subjects and patients with isolated deficiency of the first OXPHOS complex (CI).

Mots clés: [ATP]; CI; [Ca]; ERCa; ETC; HG; MIM; MOM; MCU; NDUF; OXPHOS; PM; PMCA; PMF; PTP; ROS; SERCA; VDAC

Federica Valsecchi, John J. Esseling, Werner J.H. Koopman, Peter H.G.M. Willems. Calcium and ATP handling in human NADH:ubiquinone oxidoreductase deficiency. Biochimica et Biophysica Acta - Molecular Basis of Disease, 2009, 1792 (12), pp.1130. ⟨10.1016/j.bbadis.2009.01.001⟩. ⟨hal-00537249⟩