Mitochondrial Function and Pathology

Robust activity-dependent mitochondrial calcium dynamics at the AIS is dispensable for action potential generation

The paper investigates the role of mitochondrial calcium dynamics at the axon initial segment (AIS) in action potential generation and finds they are dispensable for this function.

Astrocyte-neuron mitochondrial transfer via mitoEVs supports neuronal energy metabolism and is impaired in early Alzheimer's disease

This study identifies mitoEV-mediated mitochondrial transfer from astrocytes to neurons as a compromised pathway in early Alzheimer's disease, revealing its role in supporting neuronal energy metabolism.

Presenilin 1 (PS1) located at mitochondrial inner membrane regulates mitochondrial cristae junction proteins arrangement and cristae formation in HEK293 cells

This study shows PS1 localizes to mitochondrial inner membrane and regulates cristae structure by modulating protein interactions at cristae junctions, leading to mitochondrial dysfunction in Alzheimer's disease.

Organization of core mitochondrial replication components into multiphasic condensates

The paper investigates how mitochondrial replication components form multiphasic condensates and shows how ssDNA and dsDNA are segregated by architectural proteins.

PPR9 mediates mitochondrial nad transcript maturation required for complex I biogenesis and early plant development in Arabidopsis

This study identifies PPR9 as essential for mitochondrial RNA splicing, complex I biogenesis, and early Arabidopsis development.

MitoTracker transfers from astrocytes to neurons independently of mitochondria

The study shows MitoTracker transfers from astrocytes to neurons without requiring mitochondrial transfer.

Protein Phosphatase 2A Orchestrates Mitochondrial Dynamics via MAPK Signaling in heart

This study identifies PP2A's role in regulating mitochondrial dynamics via MAPK signaling in heart failure, showing hyperphosphorylated ERK2 drives fission and contributes to cardiomyopathy.

Stress adaptation of mitochondrial protein import by OMA1-mediated degradation of DNAJC15

The paper shows OMA1 regulates mitochondrial protein import and OXPHOS biogenesis under stress by degrading DNAJC15, affecting protein translocation and organelle communication.

The Cytochrome b m.14849T>C (S35P) Variant Induces Structural and Dynamic Alterations in the Heme bL Microenvironment in Multisystem Disease

This study investigates the structural and dynamic effects of the m.14849T>C variant in Cytochrome b, showing localized perturbations in the heme bL microenvironment that may contribute to multisystem mitochondrial disease.

Complex-I Preserves Mitochondrial Polarization during Infection of Human Macrophages by Secretion-competent Bacteria

This study identifies Complex I as essential for maintaining mitochondrial membrane potential during infection with virulent bacteria, revealing distinct strategies used by Legionella and Salmonella.

Systems Analysis of Carboxylate Transport and Oxidation Pathways in Cardiac Mitochondria

The paper uses experimental and computational methods to analyze mitochondrial energy metabolism in cardiac mitochondria, revealing new insights into substrate transport, TCA cycle kinetics, and oxidative phosphorylation.

Transcriptomic analysis of cells following decreased mitochondrial DNA-copy number reveals compensatory mechanisms in mitochondrial DNA replication and cellular energetics

The paper investigates transcriptomic and methylomic changes in HEK 293T cells with reduced mtDNA copy number, revealing compensatory mechanisms in mitochondrial replication and metabolic pathways.

Inferring Accumulation Times of Mitochondrial DNA Deletion Mutants from Cross-Sectional Single-Cell Data: Application to Tabula Muris Senis

The paper infers mtDNA deletion accumulation times from scRNAseq data in mouse muscle cells, finding mean accumulation times of ~3 months.

TargetMITO: A rule-based model for generating highly functional synthetic mitochondrial targeting sequence in yeast

The paper develops a rule-based model to generate synthetic mitochondrial targeting sequences in yeast and demonstrates their functionality in directing proteins to mitochondria.

Regulation of mitochondrial DNA homeostasis by a mitochondrial microprotein

This study identifies AltSLC35A4, an AltORF-encoded protein, as a regulator of mitochondrial DNA homeostasis by interacting with actomyosin cytoskeletal components and affecting mtDNA spatial distribution.

Multilevel impairment of mitochondrial respiration with sex-specific signatures in inclusion body myositis

This study profiles mitochondrial respiration in IBM muscle tissue, identifying sex-specific impairments in respiration and OXPHOS complexes, along with correlations with disease severity.

Disturbed mitochondrial maturation in cardiolipin remodeling-deficient cardiomyocytes

This study shows that TAFAZZIN deficiency disrupts mitochondrial maturation in cardiomyocytes, leading to structural and functional impairments associated with Barth syndrome cardiomyopathy.

Inducible Impairment of Polymerase Gamma Activity in Cardiomyocytes Promotes Severe Cardiomyopathy with Cardiac Hepatopathy

This study shows that impairing polymerase gamma activity in cardiomyocytes leads to mtDNA instability, activates the integrated stress response, and causes severe cardiomyopathy with hepatopathy in mice.

Accurate quantification of canine mitochondrial DNA copy number and its evaluation as a biomarker of brain injury

The paper develops an assay to quantify canine mitochondrial DNA copy number and evaluates its potential as a biomarker for acute brain injury.

Excessive Ca2+-dependent ER-mitochondrial contact stabilization by EFHD1 drives liver injury

This study identifies EFHD1 as a Ca2+-dependent stabilizer of ER-mitochondrial contacts that drives liver injury in MASH by promoting mitochondrial fragmentation and antiviral stress responses.

Energy dissipation via the glycerol phosphate shuttle: coupling glycolysis to mitochondrial thermogenesis in Drosophila melanogaster flight muscle

This study investigates the role of the glycerol phosphate shuttle in energy coupling and thermogenesis in Drosophila flight muscle, showing it enhances heat production while regenerating NAD+.

Vitamin D3 Regulates Mitochondrial Function and Redox Homeostasis in the Brain

This study shows that vitamin D3 protects brain mitochondria from calcium-induced damage, improves bioenergetics, and enhances redox balance in rats.

Enhanced intercellular transfer of mitochondria from nuclear respiratory factor 1 (NRF1)-primed mesenchymal stem cells: towards creation of superior mitochondrial delivery hubs

This study shows NRF1-primed MSCs enhance mitochondrial transfer to endothelial cells, improving mitochondrial function and reducing oxidative stress.

Cooperative Architecture of Mitochondrial Proteome Homeostasis

The paper investigates mitochondrial proteome homeostasis through multiomic analyses of gene perturbations, revealing post-transcriptional regulation and new protein functions.

Suppression of interferon signaling via small molecule modulation of TFAM

This study identifies small molecule modulators of TFAM that increase its levels and mtDNA copy number, reducing mtDNA stress and inflammation in disease models.

Reduced Complex III assembly dampens host inflammation as an adaptive response to viral infection

This study shows that reducing Complex III in macrophages during viral infection dampens inflammation, improving recovery from H1N1 influenza.

Cognate amino acid therapies provide preclinical benefit in 19 C. elegans models of ARS2 deficiency

This study shows that cognate amino acid treatment improves growth, neuromuscular function, and reduces stress in 19 C. elegans models of mt-ARS deficiency.

Live high-content imaging with automated analysis reveals mitochondrial changes during vascular calcification

This paper introduces H-MIP, a high-throughput platform for mitochondrial morphology analysis, and applies it to study mitochondrial changes in vascular calcification.

Transient protein structure guides surface diffusion pathways for electron transport in membrane supercomplexes

This study uses multiscale modeling and cryo-EM to show that transient protein structures guide electron transfer in mitochondrial supercomplexes, improving energy conversion efficiency by 30%.

Mitochondrial double-stranded RNA accumulation in brain aging and Alzheimer's disease

This study identifies mitochondrial double-stranded RNA (mt-dsRNA) accumulation as a driver of chronic inflammation in brain aging and Alzheimer's disease, correlating with cognitive decline and genetic risk factors.