Mitochondrial Function and Pathology

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.

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

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

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.

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.

This study identifies a cold-induced conformation of respiratory complex CI:III2 using cryo-EM and simulations, revealing structural changes that enhance electron transfer efficiency in cold environments.

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.

The paper shows MIC26 regulates metabolic pathways by modulating mitochondrial metabolite exchange, with its role reversing under hyperglycemia.

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.

The paper shows that reduced CKMT2 impairs mitochondrial function in type 2 diabetes, independent of insulin action.

The paper identifies DELE1's role in promoting translation-associated homeostasis, growth, and survival in mitochondrial myopathy through the mt-ISR pathway.

This study models mitochondrial network dynamics using simulations and analytic models to show how local parameters control network morphology and remodeling rates.

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

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

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

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.

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

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

This study shows that mitochondrial fragmentation increases protein expression noise due to stochastic co-translational targeting, and that fission-fusion processes reduce this heterogeneity.

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

This study shows that pharmacologic activation of ISR kinases HRI or GCN2 prevents mitochondrial fragmentation in disease models.

This study finds that aging baboons show sex-specific declines in prefrontal cortex mitochondrial bioenergetics, with males experiencing greater ETC activity loss and a link between walking speed and respiration in males.

This study shows that restoring mitochondrial complex III function in the liver of CIII-deficient mice prevents hypothermia and improves survival by enabling normal thermoregulation.

This study identifies diet-induced mitochondrial DNA replication instability in Rad51c mutant mice as a molecular cause of sex-bias in anemia of inflammation, showing estrogen protects females by reducing oxidative stress.

This study shows that LDL exposure causes mitochondrial dysfunction and dynamic changes in hippocampal neuronal cells.

This study shows TBC1D24 regulates mitochondrial structure, activity, and ER-mitochondria contact sites, contributing to neurological disorders.

The paper identifies Lys716 in yeast mitofusin Fzo1 as a residue modulating dimer stability and fusion through molecular dynamics simulations and experimental validation.

The paper investigates how mitochondrial rRNA sequence variation contributes to insulin resistance and metabolic syndrome in rats fed a high-fat diet.

This study maps the MCU-mediated mitochondrial calcium signaling network using proteomics, identifying 89 new interactors and their roles in disease-related pathways.

The paper introduces novel genetic tools for live imaging of mitochondrial networks in C. elegans and compares them with existing methods, highlighting their advantages and limitations.