Mitochondrial Function and Pathology

This study shows that hepatocyte-specific cytochrome c oxidase deficiency in mice causes lymphopenia due to bone marrow progenitor defects and early autoimmunity.

The paper investigates how lipid-protein interactions constrain sequence evolution in membrane subunits of eukaryotic respiratory complexes, finding that lipid-exposed surfaces show non-PPI disease mutations and kingdom-specific lipid unsaturation affects stability.

The paper characterizes the clinical progression of SLSMDS by analyzing electronic health records and identifies key genetic and clinical features associated with disease evolution.

This study identifies a fibroblast-specific mitochondrial phenotype caused by phototoxicity from red fluorescent dyes during live cell imaging.

The paper investigates mitonuclear incompatibilities in hybrid hares by generating cybrids and analyzing their cellular fitness and metabolic effects.

This study uses computational simulations to examine ATP transport in axons, showing that spontaneous neuronal firing stabilizes ATP levels and that a single mitochondrion can supply ATP to neighboring boutons, but ATP concentration declines with more empty boutons.

This study identifies a charged glutamate on VDAC1 and VDAC2 that interacts with HKI's N-terminal helix, revealing structural and mechanistic insights into their complex formation.

This study identifies Berberine as a mitochondrial calcium uniporter inhibitor that disrupts MCU-EMRE assembly and reduces mitochondrial calcium overload in mice.

This study shows that MCU regulates synaptic plasticity specifically at CA2 distal dendrites by maintaining mitochondrial morphology and distribution, with loss of MCU causing LTP deficits and mitochondrial fragmentation.

This study shows that progranulin (PGRN) is essential for vascular contractility by regulating mitochondrial function, mitophagy, and redox signaling in vascular smooth muscle cells.

The paper examines how physical activity affects mitochondrial function, ROS production, and calcium handling in skeletal muscle across the human lifespan, finding that aging does not reduce mitochondrial respiration but decreases calcium retention capacity.

The paper investigates how mtDNA damage affects polymerase gamma activity and mtDNA stability in yeast, showing that exonuclease activity drives damage-induced mtDNA clearance.

The paper uses cryo-electron tomography to study cytoplasmic ribosome orientation and clustering on mitochondria in yeast, revealing their role in co-translational protein import.

The paper uses in-situ Cryo-ET to study mitochondrial budding in BS-C-1 cells, finding no Drp1 or ER involvement and identifying new mitochondrial-vesicle interactions.

This study shows CDK4 inhibition in TNBC cells reduces apoptosis sensitivity and disrupts mitochondrial calcium signaling through MERCs, contributing to resistance to CDK4/6 inhibitors.

The paper identifies a COA5 missense variant causing isolated mitochondrial complex IV assembly impairment in two siblings and validates its role in early CIV assembly using patient-derived cells and CRISPR/Cas9 knockout models.

This paper introduces a low-cost respirometry assay for C. elegans using optical oxygen sensors and fluorescence readers, showing it can accurately measure OCR and identify issues with sequential respiratory inhibitor use.

The paper identifies a transcriptional network regulating mitochondrial biogenesis in Drosophila by combining RNAi screening and ChIP-seq analysis, revealing key factors like CG1603 and YL-1.

The paper creates a mitochondrial respiration atlas across 33 tissues in young and old mice, revealing age and sex effects on mitochondrial function.

The paper shows Mitoregulin self-associates into hexameric pore-like structures through co-immunoprecipitation, modeling, and gel analysis.

This study shows that reducing DRP1 activity in pediatric sarcomas does not improve drug sensitivity, suggesting DRP1 is not a key driver of chemoresistance in these cancers.

The paper identifies Mar26 as a determinant in cytochrome bc1 complex assembly, showing it connects assembly intermediates to MICOS to coordinate complex III maturation at crista junctions.

The paper analyzes ultrastructural data from C. elegans neurons to assess mitochondrial distribution relative to synapses, finding limited sub-cellular control over mitochondrial placement in fine caliber neurons.

This study investigates how OPA1 and its disease-causing mutants affect mitochondrial nucleoid distribution using high-resolution microscopy and a novel model.

This study characterizes mitochondrial bioenergetics in C. elegans across larval stages, revealing that OCR changes depend on normalization methods and showing an earlier shift to oxidative metabolism than previously reported.

This study identifies SLP2 as an auxiliary MICOS subunit that interacts with MIC13 to seed MIC60 assembly and promote crista junction formation.

This study identifies CCDC51 as a human ortholog of yeast Mdm33, showing they both regulate mitochondrial fission and are conserved in function.

This study shows that pyruvate supports neuronal viability without glucose and that mitochondrial pyruvate uptake regulates synaptic transmission in hippocampal terminals.

This study shows that inducible SOD2 knockdown in mouse skeletal muscle reduces metabolic flexibility by impairing pyruvate oxidation, with reversible effects upon recovery.