Mitochondrial Function and Pathology

Infrared light stimulates the mitochondrial large-conductance calcium-activated potassium channel in guinea pig cardiomyocytes.

This study shows that 820 nm infrared light enhances mitochondrial BKCa channel activity in guinea pig cardiomyocytes through a redox-dependent mechanism.

The clinical utility of functional testing in fibroblasts to diagnose primary mitochondrial disease

Four mitochondrial functional tests in fibroblasts show good sensitivity and excellent specificity for diagnosing primary mitochondrial disease.

Engineered flavonoid disrupts mitochondrial AIF/CHCHD4 complex for targeted cancer therapy

This paper identifies M30-E05, an engineered flavonoid that disrupts the AIF/CHCHD4 mitochondrial complex, reducing cancer cell viability and tumor growth in xenograft models.

Retrograde mitochondrial transport is required for mitochondrial biogenesis in zebrafish neurons

The paper shows that retrograde mitochondrial transport is necessary for mitochondrial biogenesis in zebrafish neurons, linking transport to nuclear gene expression.

Endosomal mRNA transport coordinates local mitochondrial bioenergetics during polar fungal growth

This study shows that endosomal mRNA transport coordinates mitochondrial bioenergetics in fungal hyphae, with Rrm4 essential for mitochondrial protein import and function.

Lipid mediated ER-stress contributes to the pathogenesis of mitochondrial myopathies

This study identifies lipid-mediated ER stress as a driver of muscle dysfunction in mitochondrial myopathies and shows that ceramide inhibition improves symptoms in a mouse model.

Pathogenic PTCD1 variants cause mitochondrial protein aggregation and cardiomyopathy

This study identifies pathogenic PTCD1 variants causing mitochondrial protein aggregation and cardiomyopathy by disrupting mitoribosome biogenesis and proteostasis.

Electron transport chain complex I and mitochondrial fusion regulate ROS for differentiation in Drosophila neural stem cells

This study shows that complex I and mitochondrial fusion regulate ROS levels, which in turn affect differentiation in Drosophila neuroblasts.

Individualised mapping of living human brain mitochondria by MRI reveals signatures of bioenergetic defects.

This paper introduces a neuroimaging framework to map brain mitochondria non-invasively and finds age-related declines in mitochondrial density and respiratory capacity, along with disease-specific alterations and links to systemic stress and cognition.

Mitochondrial-derived compartments buffer outer membrane protein load during acute mitochondrial adaptation

The paper shows that mitochondrial-derived compartments (MDCs) form during metabolic stress to buffer outer membrane protein load during mitochondrial adaptation.

Label-free real-time imaging of mitochondrial matrix volume changes and permeability transition in living cells

This study uses dark-field imaging to measure mitochondrial matrix volume changes in living cells, showing volume increases with K+ influx and swelling during permeability transition.

Complex-phase stochastic modeling of mitochondrial heteroplasmy

This paper introduces a complex-phase stochastic model to quantify mitochondrial disease risk by incorporating selection, genetic drift, and tissue-specific dynamics.

Tracking gene expression of single mitochondria in live neurons using nanotweezers

The paper tracks mitochondrial gene expression in live neurons using nanotweezers and finds downregulation of MT-ND1 and MT-ATP6 after exposure to α-synuclein aggregates.

Identification and small molecule rescue of mitochondrial dysfunction phenotype which converges across Leigh Syndrome and Huntington's Disease patient fibroblasts.

This study identifies a common mitochondrial dysfunction phenotype in Leigh syndrome and Huntington's disease patient fibroblasts and finds that the AMPK activator A769662 rescues this phenotype in both conditions.

Copper transport to mitochondria by SLC25A3 contributes to skeletal myoblast differentiation and is required for survival of differentiated myotubes

This study shows that SLC25A3-mediated copper transport to mitochondria is essential for skeletal myoblast differentiation and survival of mature myotubes.

Disease-causing MFN2 mutants impair mitochondrial fission dynamics by distinct DRP1 dysregulation

This study shows that MFN2 mutations in CMT2A patients alter mitochondrial fission and metabolism through distinct DRP1 dysregulation mechanisms.

Single-nucleoid imaging in whole cells defines the dynamics of the mtDNA life cycle

The paper uses advanced imaging techniques to study mtDNA dynamics in quiescent human cells, revealing rapid replication and degradation rates and spatial organization of mtDNA.

Iron Deficiency Impairs Mitochondrial Energetics and Early Axonal Growth and Branching in Developing Hippocampal Neurons

This study shows iron deficiency impairs mitochondrial function and early axon growth in hippocampal neurons, linking iron-dependent energy production to axonal development.

A conserved mechanism for regulation of mtDNA copy number in eukaryotes

The paper identifies a conserved mechanism regulating mtDNA copy number through compartmentalization of redox co-factors across eukaryotes.

Quantum transport in mitochondrial complex I is governed by a conserved structural bottleneck

The paper uses quantum walk analysis to show that a conserved structural bottleneck in mitochondrial complex I redistributes electron flux through alternative pathways across species.

Beyond copy number: The regulatory architecture of mitochondrial DNA gene expression

The paper investigates the relationship between mtDNA copy number and gene expression, finding they are largely decoupled and that mtDNA accessibility, not copy number, better explains expression variation.

Super-resolution complexome of human mitochondria elucidates translocase, morphology and OXPHOS networks

The paper maps the human mitochondrial complexome at super-resolution, revealing protein complexes, networks, and interactions involved in mitochondrial function and disease.

Full-Length Structural Modeling of Mitofusins with AlphaFold Reveals a Novel Cross-Type Dimerization and Insights into Oligomerization

The paper uses AlphaFold to generate full-length structural models of mitofusins, revealing a novel cross-type dimerization mode and a new hypothetical mechanism of outer mitochondrial membrane fusion.

Impact of aldehyde dehydrogenase 2 deficiency on tissue-specific mitochondrial metabolism in aging mice

This study investigates how ALDH2 deficiency impacts mitochondrial metabolism in aging mice across different tissues, revealing tissue-specific vulnerabilities and cognitive impairments.

Targeting the Mitochondrial Phenotype in Cockayne Syndrome Patient Cells: From Bioenergetic Fragility to Pharmacologic Rescue

This study identifies five compounds that improve mitochondrial function and survival in Cockayne syndrome patient fibroblasts by targeting oxidative stress, autophagy, and cellular resilience.

Inhibition of Mitochondrial Respiration Fragments ER Architecture and Remodels Organelle Contact Sites, as Revealed by FIB-SEM

This study uses FIB-SEM to show that mitochondrial dysfunction causes ER fragmentation and remodeling of contact sites in pancreatic β-cells.

Nuclear DNA Damage Response Triggers Reorganization of Mitochondrial Nucleic Acids

This study identifies mito-blobs as nuclear DNA damage-induced mitochondrial structures that concentrate immunogenic nucleic acids and influence inflammatory gene expression through autophagic clearance.

Mutations Causative of CPEO Differentially Engage Innate Immunity Sensors

This study shows that mutations in RNASEH1 and Twinkle trigger innate immune activation through different mitochondrial nucleic acid release mechanisms, leading to paracrine inflammation in CPEO.

The multistep quality control of mitochondrial β-barrel protein import

The paper identifies a quality control mechanism that removes faulty mitochondrial β-barrel proteins through cooperation between mitochondrial and cytosolic factors.

Adrenomedullin Restores Mitochondrial Bioenergetics and Rescues Interneuron Phenotypes in Human Models of 22q11.2 Deletion Syndrome

This study identifies mitochondrial dysfunction in 22q11.2DS interneurons and shows that Adrenomedullin rescues these defects through distinct signaling pathways.