Vamsi K. Mootha

TL;DR: It is demonstrated that, in the absence of MICU1, both Mn2+ and Ca2+ can pass through the uniporter, as evidenced by mitochondrial Mn2- uptake assays, mitochondrial membrane potential measurements, and mitoplast electrophysiology.

TL;DR: A high-throughput imaging assay is developed that tracks both the per cell mitochondrial content and the cell size in confluent human umbilical vein endothelial cells and demonstrates that one compound, BRD6897, increases the cellular content of mitochondria as evidenced by fluorescence microscopy, mitochondrial protein content, and respiration, even after rigorous correction for cell size, cell volume, or total protein content.

TL;DR: Yeast mutants defective in their ability to synthesize different phospholipids are used to identify a specific requirement of cardiolipin (CL) in the stability and function of the mitochondrial uniporter, raising the hypothesis that impaired mitochondrial calcium transport contributes to the pathogenesis of Barth syndrome.

TL;DR: Four individuals from unrelated families where biallelic mutations in C1QBP cause a defect in mitochondrial energy metabolism are reported, representing an important mitochondrial disorder associated with a clinical spectrum ranging from infantile lactic acidosis to childhood (cardio)myopathy and late-onset progressive external ophthalmoplegia.

TL;DR: In this article , the authors used phage-assisted non-continuous and continuous evolution to evolve DddA variants with improved activity and expanded targeting scope, which substantially increase the effectiveness and applicability of all-protein base editing.