Mitochondrial Function and Pathology

Mutant CHCHD10 disrupts cytochrome c oxidation and activates retrograde signaling in a model of cardiomyopathy.

This study shows that mutant CHCHD10 causes mitochondrial dysfunction and cardiomyopathy by disrupting cytochrome c oxidation and activating retrograde signaling.

The Cancer/Testis Antigen FATE1 Antagonizes Fission and Preserves Mitochondrial Network Integrity under Cytotoxic Stress

This study identifies FATE1 as a mitochondrial fusion-promoting factor that antagonizes fission and protects against fragmentation under cytotoxic stress.

Rewriting nuclear epigenetic scripts in mitochondrial diseases as a strategy for heteroplasmy control

This study shows that targeting nuclear DNA methylation can selectively reduce heteroplasmy in mitochondrial diseases by impairing high-heteroplasmy cells.

Mitochondrial DNA mutations in human oocytes undergo frequency-dependent selection but do not increase with age

The paper detects mtDNA mutations in oocytes, blood, and saliva across ages and finds mutations increase in blood and saliva but not in oocytes, with age-related differences in mutation frequency distribution.

IP3-mediated Ca2+ transfer from ER to mitochondria stimulates ATP synthesis in primary hippocampal neurons

The paper shows that IP3-mediated Ca2+ transfer from ER to mitochondria enhances mitochondrial ATP production in hippocampal neurons during activity.

Pathogenic Morphological Signatures of Perturbations in Mitochondrial-Related Genes Revealed by Pooled Imaging Assay

The paper identifies mitochondrial-related genes critical for morphology using a pooled imaging assay and reveals pathogenic variants associated with mitochondrial aggregation.

Small molecule oxybutynin rescues proliferative capacity of complex III-defective MPCs

This study shows that oxybutynin rescues proliferative capacity and metabolic function in Complex III-defective mitochondrial progenitor cells.

Various Optimization Strategies for the Isolation of Mitochondria from Sprague-Dawley Rat Liver Tissue

The paper tests optimization strategies for isolating mitochondria from rat liver tissue but finds that modifications do not significantly improve mitochondrial quality.

Metabolic dysfunction promoted by mitochondrial DNA mutation burden drives retinal degeneration

This study shows that mtDNA mutations in mice lead to metabolic dysfunction in the retina and RPE, impairing retinal function through altered energy metabolism and glucose transport.

Alterations in Lipid Saturation Trigger Remodeling of the Outer Mitochondrial Membrane

This study shows that increased unsaturated fatty acids remodel the mitochondrial outer membrane by promoting the formation of mitochondrial-derived compartments and impairing TOM complex assembly.

MNP33 is a novel mitochondrial protein that reprograms cellular bioenergetics and lipid metabolism

This study identifies MNP33, a novel mitochondrial microprotein, and shows it regulates bioenergetics, lipid metabolism, and stress responses by interacting with ANT2 and modulating mitochondrial function.

Saturated lipid stress attenuates mitochondrial genome synthesis in human cells

The paper shows that saturated lipid stress reduces mtDNA synthesis in human cells by disrupting ER-mitochondria contact sites and fatty acid trafficking, which is reversed by monounsaturated fatty acids.

An updated inventory of genes essential for oxidative phosphorylation identifies a mitochondrial origin in familial Meniere disease

Aetokthonotoxin, the causative agent of vacuolar myelinopathy, uncouples oxidative phosphorylation due to protonophore activity

This study identifies Aetokthonotoxin as a protonophore that uncouples oxidative phosphorylation, leading to ATP depletion and neurotoxic effects in wildlife.

The mammalian protein MTCH1 can function as an insertase

The paper shows MTCH1 can function as an insertase in mitochondria, compensating for the loss of the yeast MIM complex and restoring mitochondrial biogenesis and morphology.

A priority code in presequences: mitochondrial targeting signals assign specific import characteristics to precursor proteins

The paper classifies mitochondrial presequences into seven groups and identifies group A presequences as enhancing import efficiency through a specific targeting mechanism involving TOMM34.

Triphenylphosphonium is an effective targeting moiety for plants mitochondria

The paper demonstrates that triphenylphosphonium (TPP) targets plant mitochondria, enabling specific manipulation of mitochondrial DNA gyrase and triggering a retrograde response.

Structures of Chaetomium thermophilum TOM complexes with bound preproteins

The paper presents four structures of the TOM complex from Chaetomium thermophilum, showing its interaction with preproteins and revealing dynamic receptor conformations.

Drp1 Proteins Released from Hydrolysis-driven Scaffold Disassembly Trigger Nucleotide-dependent Membrane Remodeling to Promote Scission

The paper investigates the mechanism of mitochondrial fission by Drp1, showing that GTP hydrolysis enables free Drp1 to induce membrane negative Gaussian curvature, promoting scission.

Dynamins combine mechano-constriction and membrane remodeling to enable two-step mitochondrial fission via a snap-through instability.

This study shows how dynamin proteins use mechano-constriction and membrane curvature to drive mitochondrial fission through a snap-through instability.

Dose Modeling for Mitochondrial Transplantation in Barrier-Limited Tissues

The paper develops a computational model to estimate mitochondrial transplantation doses for barrier-limited tissues, finding route-adjusted requirements differ from cardiac estimates.

Mitochondrial fission controls astrocyte morphogenesis and organization in the cortex

This study shows that mitochondrial fission is essential for astrocyte morphogenesis and organization in the cortex, with Drp1 loss causing morphological and functional deficits.

The Core Subunit NDUFS2 in Mitochondrial Electron Transport Chain Complex I is Critical to Oxygen Responsiveness of Human Ductus Arteriosus Smooth Muscle Cells

This paper shows that NDUFS2 in mitochondrial Complex I is critical for oxygen responsiveness in human DASMCs, affecting calcium, cell shortening, and mROS without altering respiration.

Development of a Quantitative Systems Pharmacology Model to Interrogate Mitochondrial Metabolism in Heart Failure

This paper develops a quantitative systems pharmacology model to study mitochondrial metabolism in heart failure and predicts how changes in metabolic pathways affect exercise capacity.

Demonstration of Beat-to-Beat, On-Demand ATP Synthesis in Ventricular Myocytes Reveals Sex-Specific Mitochondrial and Cytosolic Dynamics.

This paper visualizes beat-to-beat ATP dynamics in ventricular myocytes of male and female mice, revealing sex-specific mitochondrial and cytosolic ATP production strategies.

Clu1/Clu form mitochondria-associated granules upon metabolic transitions and regulate mitochondrial protein translation via ribosome interactions

This study shows that Clu/Clu1 proteins form mitochondrial-associated granules and regulate mitochondrial protein translation by interacting with ribosomes and mRNAs during metabolic transitions.

Ubp2 modulates DJ-1-mediated redox-dependent mitochondrial dynamics in Saccharomyces cerevisiae

This study identifies a functional interaction between Ubp2 and DJ-1 paralogs in Saccharomyces cerevisiae that regulates mitochondrial homeostasis and oxidative stress resistance.

A constricted mitochondrial morphology optimizes respiration.

The paper identifies a new mitochondrial morphology in yeast called Ringo that enhances respiration by ensuring proper OXPHOS complex assembly.

Running on empty: Mitochondria without mtDNA exhibit differential motility and connectivity

The paper investigates mitochondrial behavior in Arabidopsis thaliana, showing that mitochondria lacking mtDNA exhibit altered motility and reduced connectivity.

An integrative modelling approach to the mitochondrial cristae

The paper presents a workflow to create a comprehensive model of a mitochondrial crista junction using a coarse-grained representation and molecular modelling.