Mitochondrial Calcium Uniporter Deficiency in Zebrafish Causes Cardiomyopathy With Arrhythmia.

Mitochondrial Ca uptake influences energy production, cell survival, and Ca signaling. The mitochondrial calcium uniporter, MCU, is the primary route for uptake of Ca into the mitochondrial matrix. We have generated a zebrafish mutant that survives to adulthood and exhibits dramatic cardiac phenotypes resembling cardiomyopathy and sinus arrest.