Mitochondrial Function and Pathology

Pharmacologic Activation of Integrated Stress Response Kinases Inhibits Pathologic Mitochondrial Fragmentation

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

Sex-specific decline in prefrontal cortex mitochondrial bioenergetics in aging baboons correlates with walking speed

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.

Hepatic mitochondrial respiration is crucial for euthermia in complex III-deficient mice with impaired brown adipose tissue thermogenesis

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.

Diet induced mitochondrial DNA replication instability in Rad51c mutant mice drives sex-bias in anemia of inflammation

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.

LDL exposure disrupts mitochondrial function and dynamics in a hippocampal neuronal cell line

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

TBC1D24 regulates mitochondria and endoplasmic reticulum-mitochondria contact sites

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

Lys716 in the transmembrane domain of yeast mitofusin Fzo1 modulates anchoring and fusion

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

Novel Imaging Tools to Study Mitochondrial Dynamics in Caenorhabditis elegans

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.

Mitochondrial morphodynamics are modulated by physiological range of temperature and influence host cell outcomes during influenza infection

This study shows that temperature affects mitochondrial structure and function, which in turn influences host cell outcomes during influenza A virus infection.

A novel variant in MFN2 linked to a lethal disorder of neonatal onset

The paper identifies a novel homozygous MFN2 variant R334K linked to a lethal neonatal disorder and shows it causes severe mitochondrial dysfunction, confirmed through functional studies and Drosophila modeling.

Advancing Variant Phenotyping in Myelodysplastic Syndromes via Computational Genomics of Mitochondrial Enzyme Complexes

This study uses computational structural genomics to analyze mitochondrial enzyme complexes in MDS, showing improved variant phenotyping compared to conventional methods.

Mitochondrial DNA is a sensitive surrogate and oxidative stress target in oral cancer cells

This study develops a method to quantify mtDNA damage, repair, and copy number changes in oral cancer cells under oxidative stress and finds differential responses compared to normal cells.

Perturbations in mitochondrial metabolism associated with defective cardiolipin biosynthesis: An in-organello real-time NMR study

This study uses in-organello NMR to analyze how cardiolipin defects affect mitochondrial metabolism in yeast, showing altered metabolite kinetics and TCA cycle flux.

Mitochondrial Deletions and Healthspan: Lessons from the Common Repeat

The paper investigates the role of mitochondrial DNA repeats in aging and healthspan, finding that disrupting the common repeat reduces somatic deletions and improves healthspan.

Mitochondrial Protein Carboxyl-Terminal Alanine-Threonine Tailing Promotes Human Glioblastoma Growth by Regulating Mitochondrial Function

This study identifies msiCAT-tailing of mitochondrial proteins as a mechanism promoting glioblastoma growth by enhancing mitochondrial function and reducing apoptosis.

Somatic nuclear mitochondrial DNA insertions are prevalent in the human brain and accumulate over time in fibroblasts

The paper quantifies somatic mitochondrial DNA insertions (Numts) in human brain and fibroblasts, showing their accumulation over time and association with lifespan.

Evolutionary mismatch between nuclear and mitochondrial genomes does not promote reversion mutations in mtDNA.

The paper shows that apparent reversion mutations in mtDNA are not real but result from sample cross-contamination and NUMTs.

Development of a signal-integrating reporter to monitor mitochondria-ER contacts

The paper develops a new MERCS reporter system using split LacZ complementation to dynamically track mitochondria-ER contacts and validate it with drug and genetic perturbations.

Biallelic variants in DAP3 result in reduced assembly of the mitoribosomal small subunit with altered intrinsic and extrinsic apoptosis and a Perrault syndrome-spectrum phenotype

This study identifies biallelic DAP3 variants causing mitoribosomal dysfunction, leading to combined oxidative phosphorylation deficiency and Perrault syndrome-like features.

RNA promotes mitochondrial import of F1-ATP synthase subunit alpha (ATP5A1)

The paper shows that specific cytosolic RNAs bind ATP5A1 precursor proteins and promote their mitochondrial import.

ER stress and mitochondrial dynamics: a tale of a wandering phosphatase DUSP28

This study identifies DUSP28 as a phosphatase regulating mitochondrial dynamics during ER stress by promoting fission and inhibiting mitophagy.

Cell-free chromatin particles damage genomic DNA of healthy cells via an ROS-independent mechanism

The paper shows that cell-free chromatin particles cause genomic DNA damage in an ROS-independent manner, suggesting new therapeutic targets for aging and degenerative diseases.

MIRO1 controls energy production and proliferation of vascular smooth muscle cells

This study shows MIRO1 regulates VSMC proliferation and neointima formation by controlling mitochondrial positioning, ATP production, and ETC function.

A cleaved cytosolic FOXG1 promotes excitatory neurogenesis by enhancing mitochondrial translation.

The paper shows that a cleaved cytosolic FOXG1 enhances mitochondrial translation and promotes excitatory neurogenesis, offering a new therapeutic target for mitochondrial disorders.

Inherited mitochondrial dysfunction triggered by OPA1 mutation impacts the sensory innervation fibre identity, functionality and regenerative potential in the cornea

This study shows that OPA1 mutation causes corneal innervation issues, including reduced sensitivity and impaired regeneration, despite normal epithelial function.

Heterogeneous brain region-specific responses to astrocytic mitochondrial DNA damage in mice

This study shows brain-region-specific differences in astrocytic responses to mitochondrial DNA damage using a novel AAV-based tool in mice.

Mfn2 promotes NCLX-mediated Ca2+ release from mitochondria

This study identifies Mfn2's role in promoting NCLX-mediated Ca2+ release from mitochondria, linking mitochondrial ROS to mitophagy via Ca2+ signaling.

Disruption of mitochondrial folate metabolism leads to mitochondrial DNA leakage and activation of apoptotic or inflammatory pathways

This study shows that reduced Shmt2 expression causes mtDNA leakage into the cytosol, triggering different downstream effects like apoptosis or activation of the cGAS/STING pathway in different cell types.

The role of the synaptic vesicle protein SV2A in regulating mitochondrial morphology and autophagy.

This paper identifies SV2A as a mitochondrial outer membrane protein that regulates mitochondrial morphology and autophagy through interactions with DRP1 and ATG9A.

MitoNEET reduces the mitochondrial oxidative phosphorylation during epithelial-mesenchymal transition

This study shows that mitoNEET downregulation during EMT impairs OXPHOS and cell motility in mouse mammary epithelial cells.