Mitochondrial Function and Pathology

Differential Control of Inhibitory and Excitatory Nerve Terminal Function by Mitochondria

The paper identifies differences in mitochondrial function between inhibitory and excitatory neurons, showing inhibitory terminals have higher ATP levels and distinct Ca2+ handling mechanisms.

The iAAA-mitochondrial protease YME1L1 regulates the degradation of the short-lived mitochondrial transporter SLC25A38

The paper identifies SLC25A38 as a short-lived mitochondrial transporter degraded by YME1L1 and shows its degradation is regulated by mitochondrial membrane potential.

UPF1 localises to mitochondria and is linked to mitochondrial gene expression and function in Drosophila

This study shows UPF1 localizes to mitochondria in Drosophila and affects mitochondrial gene expression and function independently of its role in NMD.

High fat diet ameliorates the mitochondrial cardiomyopathy of CHCHD10 mutant mice

This study shows that a high fat diet improves mitochondrial cardiomyopathy in CHCHD10 mutant mice by enhancing fatty acid utilization and reducing protein aggregation.

Compartmental Differences in the Retinal Ganglion Cell Mitochondrial Proteome

The paper identifies compartment-specific mitochondrial proteins in retinal ganglion cells using mass spectrometry and immunofluorescence validation.

Mitochondria-derived Vesicles mediate Mito-nuclear Trafficking of Cargo

This study identifies mitochondria-derived vesicles (MDVs) as a novel pathway for transporting mitochondrial proteins to the nucleus in cardiomyocytes.

Stomatin-like protein 2 senses oxidative stress through the interaction with phosphatidic acid to promote mitochondrial unfolded protein response

This study identifies STL-1/SLP-2 as a sensor of oxidative stress that activates the mitochondrial unfolded protein response through interaction with phosphatidic acid.

Radiation resistance in cervical cancer: An insight on mitochondrial associated membranes and its related proteins.

This study investigates radiation resistance in cervical cancer by analyzing mitochondrial-associated membrane (MAM) alterations and identifies BNIP3L as a potential target for improving radiation therapy efficacy.

SIN-3 transcriptional coregulator maintains mitochondrial homeostasis and polyamine flux

This study shows that loss of SIN-3 in C. elegans disrupts mitochondrial function and metabolism, leading to mitochondrial fragmentation and altered metabolic flux.

Cytosolic Class I J-domain proteins aid mitochondrial protein import and influence homeostasis in Arabidopsis thaliana.

This study identifies two cytosolic Class I J-domain proteins in Arabidopsis thaliana that facilitate mitochondrial protein import and homeostasis by interacting with outer mitochondrial receptors.

A systematic bi-genomic split-GFP assay illuminates the mitochondrial matrix proteome and protein targeting routes

The paper uses a bi-genomic split-GFP assay to identify mitochondrial matrix and inner membrane proteins, revealing 50 previously unvisualized proteins and characterizing dually localized proteins.

Cytosolic Ribosomal Protein Haploinsufficiency affects Mitochondrial Morphology and Respiration

This study investigates how haploinsufficiency of ribosomal proteins affects mitochondrial morphology, respiration, and stress responses in C. elegans and human cells.

The long term effects of uncoupling interventions as a therapy for dementia in humans

This paper simulates the effects of an uncoupling agent on dementia by modeling mitochondrial deletion mutants in human neurons, showing reduced dementia severity but increased pre-onset cognitive dysfunction.

In-depth study of MPV17: a molecular travel unveiling a mitochondrial calcium regulation function

This study identifies MPV17 as a regulator of mitochondrial calcium and oxidative stress, showing its role in preventing mtDNA degradation through interactions with the MICOS complex.

Drp1-JNK knockdown mitigates Scribble loss induced cell proliferation, metastasis and lethality phenotypes in Drosophila

This study shows that reducing Drp1 activity in Scrib loss models decreases cell proliferation, metastasis, and lethality by restoring cell polarity and mitochondrial function.

COA8 is a COX10-binding protein involved in the early biogenesis of cytochrome c oxidase

This study identifies COA8 as a COX10-binding protein essential for COX10 stability and catalytic activity in human cytochrome c oxidase biogenesis.

SLC25A46 is in contact with lysosomes and plays a role in mitochondrial cholesterol homeostasis

This study shows SLC25A46 is required for stress-induced mitochondrial hyperfusion and involved in mitochondrial cholesterol homeostasis.

Mitochondria-Associated Membranes Are Not Altered In Immune Cells In T2D

This study shows that MAM proteins and their functions in human immune cells are not altered in T2D, suggesting they are unlikely to contribute to metaflammation.

The specific AMPK activator A-769662 ameliorates pathological phenotypes following mitochondrial DNA depletion

This study shows that the AMPK activator A-769662 improves mitochondrial function and partially restores mtDNA levels in cells with mtDNA depletion.

Regulation of astrocyte metabolism by mitochondrial translocator protein 18kDa

This study investigates the role of TSPO in astrocyte metabolism and inflammation, finding that TSPO deficiency alters metabolic flexibility but not the inflammatory response to LPS.

A small molecule inhibitor Mirin prevents TOP3A-dependent mtDNA breakage and segregation

Mirin prevents mtDNA breakage and immune response in MGME1-deficient cells through MRE11-independent mechanisms involving TOP3A and mtDNA replication termination.

Myosin-19 and Miro regulate mitochondria-endoplasmatic reticulum contacts and mitochondria inner membrane architecture

This study shows that Myo19 and Miro regulate mitochondria-endoplasmic reticulum contacts and inner mitochondrial membrane structure in mouse cells.

The majority of axonal mitochondria in mammalian neuronslack mitochondrial DNA and do not produce ATP

The paper shows that most axonal mitochondria in mammalian neurons lack mtDNA and do not produce ATP, contrasting with dendritic mitochondria.

Mitochondrial morphology dynamics and ROS regulate apical polarity and differentiation in Drosophila follicle cells

The paper shows that Drp1 regulates follicle cell differentiation in Drosophila oogenesis by controlling mitochondrial morphology, ROS levels, and epithelial polarity.

Single-nucleoid architecture reveals heterogeneous packaging of mitochondrial DNA

The paper uses long-read single-molecule accessibility mapping to reveal that human mtDNA undergoes all-or-none global compaction, with most nucleoids in an inaccessible state.

A Drosophila model of mitochondrial disease phenotypic heterogeneity

This study uses a Drosophila model to show that varying levels of NDUFS1 knockdown lead to distinct phenotypic and metabolic outcomes in complex I deficiency.

Pathways controlling neurotoxicity and proteostasis in mitochondrial complex I deficiency

The paper identifies pathways controlling neurodegeneration and proteostasis in mitochondrial complex I deficiency using a Drosophila model and genetic screens.

Systems-level analyses dissociate genetic regulators of reactive oxygen species and energy production

The paper uses CRISPRi and FACS to compare effects of respiratory gene knockdown on ROS and ATP, identifying differential regulators of these processes.

Increased mitochondrial transcription initiation does not promote oxidative phosphorylation

This study shows that increased POLRMT levels enhance mitochondrial transcription initiation but do not increase OXPHOS biogenesis due to downstream regulatory limitations.

The NAD+ Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease

This study uses iPSC-derived cortical organoids to model Alpers' disease and shows that nicotinamide riboside improves mitochondrial defects and neuronal loss.