**5.6 Calcium deregulation in Pyruvate Dehydrogenase deficiency**

Aerobic metabolism may also affect mitochondrial Ca2+ homeostasis. Thus, deregulation of Ca2+ handling was also reported in human fibroblasts from a patient with an inherited defect in pyruvate dehydrogenase (PDH). Indeed, these cells show a decrease ability to sequester cytosolic Ca2+ into mitochondria without affecting basal cytosolic and mitochondrial Ca2+ levels. It was postulated that reduced mitochondrial uptake is linked to decreased mitochondrial potential (Padua et al., 1998).
