Tissue-specific heteroplasmy segregation is accompanied by a sharp mtDNA decline in soma.

Mutations in the mitochondrial genome (mtDNA) can be pathogenic. Owing to the multi-copy nature of mtDNA, wild-type copies can compensate for the effects of mutant mtDNA. Wild-type copies available for compensation vary depending on the mutant load and the total copy number.

A tRNA processing enzyme is a key regulator of the mitochondrial unfolded protein response.

The mitochondrial unfolded protein response (UPR) has emerged as a predominant mechanism that preserves mitochondrial function. Consequently, multiple pathways likely exist to modulate UPR. We discovered that the tRNA processing enzyme, homolog of ELAC2 (HOE-1), is key to UPR regulation in .

Mitoflash biogenesis and its role in the autoregulation of mitochondrial proton electrochemical potential.

Respiring mitochondria undergo an intermittent electrical and chemical excitation called mitochondrial flash (mitoflash), which transiently uncouples mitochondrial respiration from ATP production. How a mitoflash is generated and what specific role it plays in bioenergetics remain incompletely understood. Here, we investigate mitoflash biogenesis in isolated cardiac mitochondria by varying the respiratory states and substrate supply and by dissecting the involvement of different electron transfer chain (ETC) complexes.

Mitochondrial Flashes: Elemental Signaling Events in Eukaryotic Cells.

Mitochondrial flashes (mitoflashes) are recently discovered mitochondrial activity which reflects chemical and electrical excitation of the organelle. Emerging evidence indicates that mitoflashes represent highly regulated, elementary signaling events that play important roles in physiological and pathophysiological processes in eukaryotes. Furthermore, they are regulated by mitochondrial ROS, Ca, and protons, and are intertwined with mitochondrial metabolic processes.