The mitochondrial Ca2+ uniporter

Mitochondrial Ca2+ uptake plays a fundamental role in the regulation of energy production and cell survival. Under physiological conditions, mitochondrial Ca2+ uptake occurs by a uniport mechanism driven electrophoretically by the membrane potential created by the respiratory chain. The activity and the biochemical properties of the mitochondrial calcium uniporter (MCU) were extensively characterized for decades but the molecular identity of the channel has remained elusive. Here, we review the recent discovery of the mitochondria Ca2+ uniporter that represents a groundbreaking result for the molecular understanding of mitochondrial Ca2+ homeostasis and will provide insight into the role of mitochondrial Ca2+ deregulation in the pathogenesis of human disorders.