Mitochondrial Function and Pathology

The paper investigates the cellular response to mitochondrial dysfunction in mtDNA maintenance disorders using imaging mass cytometry, finding complex-specific upregulation and proteostasis activation.

This study shows that the AMPK activator A-769662 improves mitochondrial function and partially restores mtDNA levels in cells with mtDNA depletion.

This study investigates the role of TSPO in astrocyte metabolism and inflammation, finding that TSPO deficiency alters metabolic flexibility but not the inflammatory response to LPS.

Mirin prevents mtDNA breakage and immune response in MGME1-deficient cells through MRE11-independent mechanisms involving TOP3A and mtDNA replication termination.

This study identifies α-ketoglutarate dehydrogenase (KGDH) as a major source of mitochondrial hydrogen peroxide (mH2O2) during fatty acid oxidation, showing sex-independent effects.

The paper examines how SARS-CoV-2 spike protein RBD affects mitochondrial function and inflammation in three human cell types, finding increased mitoROS and cytokine expression in all, with HLMVEC showing the strongest response.

This paper develops a cerebral organoid model of POLG disease that recapitulates its molecular and pathological features, including mtDNA depletion and neuronal loss, and shows metformin can alleviate some abnormalities.

This paper establishes an immortalised murine desminopathy model and finds reduced mitochondrial proton leak and ADP/ATP translocase levels in desminopathy myotubes.

This study shows that Myo19 and Miro regulate mitochondria-endoplasmic reticulum contacts and inner mitochondrial membrane structure in mouse cells.

The paper shows that most axonal mitochondria in mammalian neurons lack mtDNA and do not produce ATP, contrasting with dendritic mitochondria.

The paper shows that Drp1 regulates follicle cell differentiation in Drosophila oogenesis by controlling mitochondrial morphology, ROS levels, and epithelial polarity.

The paper uses long-read single-molecule accessibility mapping to reveal that human mtDNA undergoes all-or-none global compaction, with most nucleoids in an inaccessible state.

This study uses a Drosophila model to show that varying levels of NDUFS1 knockdown lead to distinct phenotypic and metabolic outcomes in complex I deficiency.

This study shows how herpesvirus infection alters mitochondrial structure and metabolism during infection progression.

The paper investigates ROS generation during reperfusion, finding that Cx-I RET contributes less than previously thought, with other sources contributing significantly.

The paper identifies pathways controlling neurodegeneration and proteostasis in mitochondrial complex I deficiency using a Drosophila model and genetic screens.

The paper uses CRISPRi and FACS to compare effects of respiratory gene knockdown on ROS and ATP, identifying differential regulators of these processes.

This study shows that increased POLRMT levels enhance mitochondrial transcription initiation but do not increase OXPHOS biogenesis due to downstream regulatory limitations.

This study identifies a mitochondrial carrier in Blastocystis that transports glycolytic intermediates, linking cytosolic and mitochondrial glycolysis and highlighting it as a potential drug target.

This study shows that mitochondrial defects in desmin-mutated cardiomyocytes contribute to cardiomyopathy and can be reversed by transferring normal mitochondria.

This study uses iPSC-derived cortical organoids to model Alpers' disease and shows that nicotinamide riboside improves mitochondrial defects and neuronal loss.

This study demonstrates that mitochondrial transplantation can restore bioenergetics in diseased cells, with metabolic compatibility and mitochondrial activity influencing therapeutic outcomes.

The paper identifies dual regulation of SLC25A39 by AFG3L2 and iron, showing how this controls mitochondrial glutathione homeostasis in neurons.

The paper develops a genetically encoded tool to manipulate mitochondrial membrane potential and shows it drives the integrated stress response from ATP synthase dysfunction.

This study uses COX-SBFSEM to examine mitochondrial morphology and function in human muscle biopsies with mtDNA deletions, revealing distinct mitochondrial populations and spatial patterns of oxidative phosphorylation.

The paper identifies CLPP-deficiency-induced changes in mitochondrial translation and respiration, linking them to hearing loss in Perrault syndrome type 3.

The paper analyzes the mitochondrial mutation spectrum in chordates, identifying three main mutation signatures and their underlying causes, with implications for age-related disease prevention.

This study identifies structural and metabolic changes in human heart failure using SBF-SEM and metabolomic analyses.

The paper develops a method to deplete mitochondrial DNA in bronchial epithelial cells and shows the resulting metabolic changes.

This paper reveals a wave-shaped germline selection profile of mtDNA mutations by correcting for regression to the mean bias, showing how mutations are filtered in the germline.