Mitochondrial dysfunction, including decreased mitochondrial respiration and increased mitochondrial stress, is associated with many major human diseases and casual to many pathological defects.

ATP synthase is a universal molecular machine for energy conversion. By coupling to cellular respiration in mitochondria, it catalyzes conversion of chemical energy of cells. Mitochondrial ATP ...

Proceedings of the National Academy of Sciences of the United States of America, Vol. 118, No. 8 (February 23, 2021), pp. 1-9 (9 pages) The ATP synthase complexes in mitochondria make the ATP required ...

We describe the case of identical twin boys who presented with low body weight despite excessive caloric intake. An evaluation of their fibroblasts showed elevated oxygen consumption and decreased ...

A defect in mitochondrial tRNA modification impairs the proper formation of mitochondrial OXPHOS complexes, which results in iron transmigration from mitochondria to cytosol. Excess cytosolic iron ...