Mitochondrial Function and Pathology

Mitoregulin self-associates to form likely homo-oligomeric pore-like structures

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

Depletion of chemoresponsive mitochondrial fission mediator DRP1 does not mitigate sarcoma resistance

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.

Platelet bioenergetics correlate with skeletal muscle metabolism in C57BL/6J mice

The paper shows that platelet mitochondrial bioenergetics correlate with skeletal muscle mitochondrial function in mice, suggesting platelets as a non-invasive surrogate for muscle metabolism assessment.

Ultrastructural analysis reveals mitochondrial placement independent of synapse placement in fine caliber C. elegans neurons

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.

Bioenergetic signature of Synaptic mitochondria

The paper identifies a distinct bioenergetic profile of synaptic mitochondria that enables them to adapt to the dynamic energy demands of synaptic transmission.

Comprehensive characterization of mitochondrial bioenergetics at different larval stages reveals novel insights about the developmental metabolism of Caenorhabditis elegans

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.

Human CCDC51 and yeast Mdm33 are functionally conserved mitochondrial inner membrane proteins that demarcate a subset of organelle fission events

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

Mitochondrial pyruvate transport regulates presynaptic metabolism and neurotransmission

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

Inducible and reversible SOD2 knockdown in mouse skeletal muscle drives impaired pyruvate oxidation and reduced metabolic flexibility

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

Significantly reduced, but balanced, rates of mitochondrial fission and fusion are sufficient to maintain the integrity of yeast mitochondrial DNA

The paper shows that balanced, reduced rates of mitochondrial fission and fusion maintain mtDNA integrity in yeast, and increased mtDNA copy number reduces respiratory loss in mutants.

The mitochondrial mRNA stabilizing protein, SLIRP, regulates skeletal muscle mitochondrial structure and respiration by exercise-recoverable mechanisms

This study identifies SLIRP as a mitochondrial mRNA stabilizing protein that regulates mitochondrial structure and respiration in skeletal muscle, with exercise improving mitochondrial function through increased mitoribosome capacity.

Acute Triclosan Exposure Alters Mitochondrial Form and Function in Xenopus laevis Tadpoles, Including the Developing Brain

This study investigates triclosan's effects on mitochondrial form and function in Xenopus laevis tadpole brains, finding dose-dependent impairments in mitochondrial membrane potential and metabolic function.

The mitochondrial-targeted peptide therapeutic elamipretide improves cardiac and skeletal muscle function during aging without detectable changes in tissue epigenetic or transcriptomic age

Stem cell models of TAFAZZIN deficiency reveal novel tissue-specific pathologies in Barth Syndrome

This study uses stem cell models to investigate tissue-specific pathologies in Barth Syndrome caused by TAFAZZIN deficiency, identifying mitophagy defects and potential therapeutic targets.

Mitochondrial cardiolipin metabolism controlled by tafazzin enables ferroptosis

The paper shows that tafazzin deficiency in Barth Syndrome protects against ferroptosis by altering mitochondrial cardiolipin metabolism, affecting VDAC function and reducing ROS availability for ferroptosis.

A Novel Cyanine-Based Fluorescent Dye for Targeted Mitochondrial Imaging in Neurotoxic Conditions and In Vivo Brain Studies

This paper introduces a novel mitochondrial-targeting dye, Cy5-PEG4, for in vivo brain imaging and neurodegenerative disease studies, showing improved BBB penetration and reduced toxicity.

Mitochondrial network branching enables rapid protein spread with slower mitochondrial dynamics

The paper models how mitochondrial network structure and dynamics affect protein spread, finding that branching networks enable faster spread with slower dynamics.

The large GTPase Guanylate-Binding Protein-1 (GBP-1) promotes mitochondrial fission in glioblastoma.

This study shows GBP-1 promotes glioblastoma progression by enhancing mitochondrial fission through interaction with Drp1.

Human iPSCs from aged donors retain their mitochondrial aging signature

This study shows that iPSCs from aged donors retain mitochondrial aging features like impaired bioenergetics and increased reactive oxygen species, suggesting they can model mitochondrial aging for drug screening.

Mitochondrial DNA Mutations Linking Leigh Syndrome and Carotid Atherosclerosis: A Study of Shared Genetic Pathways

This study investigates the link between mitochondrial DNA mutations and Leigh syndrome, carotid atherosclerosis, and MELAS syndrome, identifying shared genetic pathways and potential therapeutic targets.

Mitochondrial Dysfunction in Alzheimer's Disease Is Driven By Excess ER-Calcium Release in Patient-Derived Neurons

This study shows that excess ER-Ca2+ release via RyR contributes to mitochondrial dysfunction in AD neurons, leading to cellular pathology and that pharmacological inhibition of RyR-Ca2+ release can reverse these effects.

ER-mitochondria distance is a critical parameter for efficient mitochondrial Ca2+ uptake and oxidative metabolism.

The paper shows that an optimal ER-mitochondria distance of ~20 nm is critical for efficient Ca2+ transfer and mitochondrial oxidative metabolism, with reduced distances observed in Parkinson's disease astrocytes.

Cardiolipin deficiency disrupts electron transport chain and drives steatohepatitis

This study shows that mitochondrial cardiolipin deficiency disrupts electron transport chain function, leading to oxidative stress and promoting MASH in mice and humans.

Pseudohypoxia-stabilized HIF2α transcriptionally inhibits MNRR1, a druggable target in MELAS

The paper identifies nitazoxanide and tizoxanide as compounds that increase MNRR1 levels, improving mitochondrial function in MELAS by inhibiting HIF2α-mediated transcriptional suppression.

Metabolic Regulation of Mitochondrial Morphologies in Pancreatic Beta Cells: Bioenergetics-Mitochondrial Dynamics Coupling

The paper models mitochondrial dynamics in pancreatic beta cells to show that fission occurs under metabolic stress when proton leak exceeds ATP synthesis.

Sociality shapes mitochondrial adaptations supporting hypoxia tolerance

This study identifies mitochondrial adaptations in naked mole-rats that confer hypoxia resistance, linking social behavior to molecular evolution.

Calcium Homeostasis Modulator 2 Constitutes an ATP-regulation Pore in Mitochondria

This study identifies CALHM2 as an ATP-regulated mitochondrial channel, showing it affects ATP levels and mTOR pathway activity, with implications for aging and disease.

AFG3L2-mediated proteolysis restricts mitochondrial biogenesis and gene expression in hypoxia

This study identifies AFG3L2-mediated proteolysis as a mechanism that restricts mitochondrial biogenesis and gene expression under hypoxia by degrading key mitochondrial proteins.

Selection promotes age-dependent degeneration of the mitochondrial genome

The paper identifies age-dependent selection forces that drive mtDNA mutation accumulation in liver cells, leading to functional decline.

The relationship between brain activation and mitochondrial complex I protein levels during cognitive function in healthy humans: an BCPP-EF PET and functional MRI study of task switching

This study links mitochondrial complex I levels to task switching performance and BOLD response using PET and fMRI in healthy humans.