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.

This study shows that sphingolipid metabolism influences mitochondrial morphology changes in aging yeast cells and during oxidative stress.

This study investigates the distinct roles of VDAC1, VDAC2, and VDAC3 in mitochondrial respiration and cancer cell metabolism through isoform-specific knockouts in HeLa cells.

This study shows that AOX from Ciona intestinalis accelerates Drosophila larval growth and development by reconfiguring the mitochondrial ETS without increasing body heat.

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 identifies MRPL47 as an amplified and overexpressed gene in NSCLC that promotes tumor progression by disrupting mitochondrial function and ROS signaling.

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.

The paper uses single-cell and bulk RNA sequencing to compare pathological and physiological cardiac hypertrophy, finding that mitochondrial fission genes drive pathological progression.

This study shows that disrupting mitochondrial fission (DRP1) leads to liver injury and tumorigenesis, while loss of fusion proteins has less impact, and triple knockout mice show reduced tumor development.

This study shows EFhd2 promotes mitochondrial spare capacity in B cells during immune synapse formation by stabilizing microtubules.

This study investigates how germline and somatic genetic variations affect mitochondrial DNA content, mutation patterns, and gene expression in liver tumors across four mouse strains.

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 stressed astrocytes can restore mitochondrial function through fusion with mesenchymal stromal cells, as evidenced by mitochondrial DNA transfer and long-term functional recovery.

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 introduces a framework to quantify mitochondrial specialization and plasticity using transcriptomic data across various contexts.

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.

This study identifies Mdm38/LETM1 as a protein that links ion homeostasis and proteostasis in the mitochondrial inner membrane in yeast.

This study examines mitochondrial changes during atrial fibrillation in animals and humans, showing early hyperfunction and damage, and demonstrates that mitochondrial-targeted therapy can prevent adverse remodeling and AF sustainability.

This study shows that reduced Opa1 in endothelial cells of old mice worsens vascular function and increases kidney oxidative stress.

This study shows that mitochondrial dysfunction in monocytes of severe alcohol-related hepatitis patients contributes to immune deficiency.

The paper identifies NHR-180 as a regulator of mitochondrial function and protein synthesis that can restore mitochondrial function in atfs-1(null) worms and mammalian models.