Mitochondrial Function and Pathology

The Structure of the Drp1 Lattice on Membrane

This study determines the structure of Drp1 on lipid membranes, revealing a locally ordered lattice and conformational changes that suggest a ratchet mechanism for mitochondrial constriction.

The MFN2 Q367H variant reveals a novel pathomechanism connected to mtDNA-mediated inflammation

The paper identifies a novel MFN2 variant, Q367H, linked to mtDNA-mediated inflammation in a patient with distal myopathy, revealing a pathomechanism connecting MFN2 dysfunction to inflammatory signaling.

Brain-body mitochondrial distribution patterns lack coherence and point to tissue-specific and individualized regulatory mechanisms

The paper examines mitochondrial distribution patterns across tissues and finds they lack coherence, suggesting tissue-specific and individualized regulatory mechanisms.

Mpf1 is a novel factor that affects the dual distribution of tail-anchored proteins between mitochondria and peroxisomes

This study identifies Mpf1 as a novel factor influencing the dual targeting of tail-anchored proteins between mitochondria and peroxisomes in yeast.

Mechanism of MCUB-dependent inhibition of mitochondrial calcium uptake

This study identifies three factors contributing to MCUB-dependent inhibition of mitochondrial calcium uptake using cell lines and live-cell imaging.

Endothelial MICU1 protects against vascular inflammation and atherosclerosis by inhibiting mitochondrial calcium uptake

This study shows that endothelial MICU1 prevents vascular inflammation and atherosclerosis by maintaining mitochondrial calcium homeostasis and reducing mitochondrial reactive oxygen species.

Oxidative Injury to Lung Mitochondrial DNA is a Key Contributorfor the Development of Chemical Lung Injury

This study shows that oxidative injury to mitochondrial DNA contributes to acute and chronic lung injury, and that targeting OGG1 can mitigate this damage.

An Msp1-Protease Chimera Captures Transient AAA+ Interactions and Unveils Ost4 Mislocalization Errors

This study creates an Msp1-protease chimera to capture transient AAA+ interactions, identifies Ost4 as a novel Msp1 substrate, and reveals mislocalization errors in Ost4.

A Human Brain Map of Mitochondrial Respiratory Capacity and Diversity

The paper creates a brain-wide map of mitochondrial phenotypes by combining physical voxelization with biochemical profiling, revealing regional differences in mitochondrial density and function.

The Volume of Mitochondria Inherited Impacts mtDNA Homeostasis in Budding Yeast

The paper shows that the volume of mitochondria inherited by daughter cells in budding yeast affects mtDNA homeostasis, with reduced mitochondrial volume increasing mtDNA loss and requiring copy number upregulation to maintain function.

COX6B1 secures a redox-sensitive step in early cytochrome c oxidase assembly

The paper investigates the role of COX6B1 in cytochrome c oxidase assembly, finding it essential for both early redox-sensitive steps and late stabilization of the complex.

H2S remodels mitochondrial ultrastructure and destabilizes respiratory supercomplexes

This study shows that chronic hydrogen sulfide exposure alters mitochondrial structure and function in human cells by disrupting cristae and respiratory supercomplexes.

In-cell architecture of the mitochondrial respiratory chain

The paper uses cryo-electron tomography to visualize mitochondrial respiratory complexes in Chlamydomonas reinhardtii, revealing their organization into distinct membrane domains and a respirasome structure with cytochrome c binding.

The Silent Saboteur: How Mitochondria Shape the Long-Term Fate of the Injured Brain.

This study identifies mitochondrial dysfunction in neurons and glia as an early driver of neurodegeneration following traumatic brain injury, linking it to Alzheimer's disease pathology.

The Mia40 substrate Mix17 exposes its N-terminus to the cytosolic side of the mitochondrial outer membrane

The paper identifies Mix17 as a Mia40 substrate in yeast that spans the mitochondrial outer membrane and exposes its N-terminus to the cytosol, mediated by its interaction with Tom40.

An Algebraic Approach to Representing Mitochondrial Dynamics

This paper introduces an algebraic framework to model mitochondrial dynamics as groupoids and defines a distance metric for comparing mitochondrial structures based on morphological operations.

A key role of the EMC complex for mitochondrial respiration and quiescence in fission yeasts

This study shows that EMC complex dysfunction in fission yeast impairs mitochondrial respiration and quiescence, with rescue via membrane fluidity and sterol composition changes.

Metabolic remodelling in hiPSC-derived myofibres carrying the m.3243A>G mutation

This study generates hiPSC-derived myofibres with the m.3243A>G mutation to model mitochondrial disease, showing metabolic and physiological changes related to mutation load.

Miro1 expression alters global gene expression, ERK1/2 phosphorylation, oxidation, and cell cycle progression

Skeletal Muscle Specific PolG Dysfunction Activates the Integrated Stress Response and Promotes a Cachexia like Phenotype

This study shows that muscle-specific PolG dysfunction causes mitochondrial dysfunction and cachexia by activating the mtISR and altering the mitochondrial folate cycle.

Biallelic variants in MRPL49 cause variable clinical presentations, including sensorineural hearing loss, leukodystrophy, and ovarian insufficiency.

A hepatocyte-specific cytochrome c oxidase deficiency in mice leads to a lymphopenia owing to deficiencies in bone marrow progenitors

This study shows that hepatocyte-specific cytochrome c oxidase deficiency in mice causes lymphopenia due to bone marrow progenitor defects and early autoimmunity.

Kingdom-specific lipid unsaturation shapes up sequence evolution in membrane arm subunits of eukaryotic respiratory complexes

The paper investigates how lipid-protein interactions constrain sequence evolution in membrane subunits of eukaryotic respiratory complexes, finding that lipid-exposed surfaces show non-PPI disease mutations and kingdom-specific lipid unsaturation affects stability.

Sphingolipid metabolism drives mitochondria remodeling during aging and oxidative stress

This study shows that sphingolipid metabolism influences mitochondrial morphology changes in aging yeast cells and during oxidative stress.

Unlocking the Distinct Roles of the Three Mammalian VDAC Isoforms in Mitochondrial Respiration and Cancer Cell Metabolism

This study investigates the distinct roles of VDAC1, VDAC2, and VDAC3 in mitochondrial respiration and cancer cell metabolism through isoform-specific knockouts in HeLa cells.

The alternative oxidase reconfigures the larval mitochondrial electron transport system to accelerate growth and development in Drosophila melanogaster

This study shows that AOX from Ciona intestinalis accelerates Drosophila larval growth and development by reconfiguring the mitochondrial ETS without increasing body heat.

Recognizing the Evolution of Clinical Syndrome Spectrum Progression in Individuals with Single Large-Scale mitochondrial DNA deletion syndromes (SLSMDS)

The paper characterizes the clinical progression of SLSMDS by analyzing electronic health records and identifies key genetic and clinical features associated with disease evolution.

The curse of the red pearl: a fibroblast specific pearl-necklace mitochondrial phenotype caused by phototoxicity

This study identifies a fibroblast-specific mitochondrial phenotype caused by phototoxicity from red fluorescent dyes during live cell imaging.

Exploring mitonuclear interactions in the regulation of cell physiology: insights from interspecies cybrids

The paper investigates mitonuclear incompatibilities in hybrid hares by generating cybrids and analyzing their cellular fitness and metabolic effects.

MRPL47 Deficiency Drives Mitochondrial Dysfunction via ROS/p38-MAPK/CDKN1A Signaling in Non-Small Cell Lung Cancer

This study identifies MRPL47 as an amplified and overexpressed gene in NSCLC that promotes tumor progression by disrupting mitochondrial function and ROS signaling.