Mitochondrial Function and Pathology

This study shows that yeast cells use local mitochondrial physiology to select for functional mtDNA, with purifying selection against specific mtDNA mutations under fermentative conditions.

The paper shows that wild-type cortical neurons form and extrude calcium-phosphate sheets associated with mitochondria, suggesting a role in pathological calcification in neurological disorders.

The paper shows TMBIM5 and SLC8B1 work together in tissue-specific mitochondrial calcium regulation in zebrafish, with TMBIM5 acting as a calcium efflux pathway.

This study shows that cancer cells surviving cisplatin chemotherapy increase OMA1 activity and mitochondrial fragmentation to resist oxidative stress.

The paper develops a computational model of mitochondrial bioenergetics and integrates it with experimental data from Parkinson's Disease models to predict molecular defects and provide a resource for interpreting bioenergetic experiments.

The paper profiles mitochondrial phenotypes in immune cells of mitochondrial disease patients and finds they are largely preserved and not strongly linked to disease severity.

This study investigates age-related mitochondrial cristae structural changes in human and mouse hearts, linking Opa1 downregulation to reduced OXPHOS capacity without affecting core subunit levels.

This study uses mouse models to investigate mitochondrial metabolism in specific kidney tubular cell types and finds differences in mitochondrial function under fasting conditions.

This study identifies TMEM65 as a key component of the mitochondrial Ca2+ efflux machinery, showing its role in maintaining Ca2+ homeostasis and preventing mitochondrial dysfunction.

This study quantifies presequence strength in mitochondrial protein import and shows its impact on mitochondrial function and cellular fitness.

This study identifies MISO as a regulator of mitochondrial dynamics and mtDNA homeostasis by forming SMEM subdomains in response to IMM stresses.

This study ablates Miro1 in cortical excitatory neurons and finds it leads to mitochondrial mislocalization, reduced brain size, and anxiety-like behaviors in adult mice.

The paper analyzes mitochondrial morphology and spatial organization in Drosophila neurons to identify systematic rules governing their positioning and cell-type specificity.

The paper investigates OPA1's role in cardiac function and metabolism using in silico and in vivo approaches, finding that OPA1 overexpression improves cardiac function and maintains metabolic markers in a mouse model.

The paper shows that mice reduce pathogenic mtDNA mutations in oocytes during early folliculogenesis, maintaining mitochondrial integrity across generations.

This paper develops an inducible mtDNA mutator mouse model to study mitochondrial dysfunction with spatial and temporal control, showing it recapitulates the original model's phenotype.

This study identifies GFAP as a novel mediator of mitochondrial fission by surrounding fission sites and modulating Drp1 recruitment, revealing a new mechanism in mitochondrial plasticity.

The paper identifies two compounds that activate the HRI pathway, improving mitochondrial function in MFN2-deficient cells.

This study shows that mitochondrial dysfunction contributes to aortic aging and that treatment with elamipretide improves mitochondrial function and reduces inflammation in aged mice.

This study shows reduced mitochondrial ketone body oxidation in insulin resistant states across multiple organs in humans and mice.

This study shows STX4 is essential for mitochondrial homeostasis in skeletal muscle by regulating mitochondrial biogenesis and mitophagy.

This study identifies mitochondrial complex III-derived ROS as a key driver of immunometabolic changes in astrocytes that contribute to dementia pathology.

This paper identifies TMX2 as a regulator of ER-mitochondria contacts by preventing TOM70 sulfenylation, linking its dysfunction to neurodevelopmental disorders.

The paper shows that Euglenozoa replaced the Mic60 subunit of the MICOS complex with two mitofilin-containing proteins, Mic34 and Mic40, which have distinct structural and functional properties.

This study reveals the mechanism of UCP1-mediated proton and fatty acid transport using molecular simulations and mutagenesis, showing how proton transfer and FA movement are facilitated by specific residues and conformational changes.

This paper introduces a no-wash, high-throughput toxicity assay using a novel fluorescent dye to measure mitochondrial membrane potential in primary cells and validates it with a compound screening.

The paper shows that disrupting mitochondrial fusion in Drosophila neurons leads to a Warburg-like metabolic response that protects against neurodegeneration.

The paper identifies Pth2 as a key player in mitoRQC by modulating protein translocation and shows that delayed translocation protects mitochondria from toxic CAT-tailed proteins.

The paper shows that mitochondrial function influences cell growth rates to maintain mitochondrial volume homeostasis across cell divisions in yeast.

The paper investigates the pathogenic mutation m.10191T>C in mitochondrial DNA, showing it leads to reduced complex I function and NADH accumulation, contributing to Leigh syndrome.