Mitochondrial Function and Pathology

Asymmetric distribution of mitochondrial Ca2+ regulators specifies compartment-specific mitochondrial function and neuronal development

The paper identifies compartment-specific mitochondrial Ca2+ dynamics in neurons and their molecular regulators, showing how these differences affect neuronal development.

Elevated cholesterol in APOE4 astrocytes drives mitochondrial cristae collapse and ATP synthase dysfunction

This study shows that elevated cholesterol in APOE4 astrocytes causes mitochondrial cristae collapse and ATP synthase dysfunction, linking cholesterol imbalance to mitochondrial disease.

Mitochondrial phosphate carrier-dependence of mitochondrial calcium chelation and respiration in skeletal muscle

This study shows that mitochondrial phosphate carrier (PiC) depletion increases matrix Ca2+ uptake and alters exercise performance in mice.

A Modular Platform for Purification of Organelle-associated Mitochondria Reveals Functional Specialization at Organelle Contact Sites

The paper introduces ORCA, a method to isolate mitochondria associated with specific organelles, revealing distinct proteomes and functional roles at organelle contact sites.

Recurrent Single-Nucleotide Insertions in the Mitochondrial Second Light-Strand Promoter Cause Tubulointerstitial Kidney Disease

The paper identifies recurrent mtDNA variants, particularly in the LSP2 promoter, as a cause of tubulointerstitial kidney disease in families with maternal inheritance.

Exploring Heteroplasmic Variants in mtDNA: Insights from Single-Cell Transcriptomics

This study uses single-cell RNA sequencing to analyze mtDNA heteroplasmy in fibroblasts, revealing cell-to-cell variability and sample-specific transcriptional responses.

Combinatorial sequence elements fine-tune mitochondrial protein import to facilitate dual localization

This study identifies sequence elements that enable dual localization of mitochondrial proteins, showing how combinatorial features regulate import and function across compartments.

Succinate and its carrier Sfc1 mediate metabolic control of mitochondrial protein import by the TIM23 translocase

The paper shows that succinate and its carrier Sfc1 regulate mitochondrial protein import via interaction with Tim23, affecting enzyme localization between mitochondria and cytosol.

Mitochondrial control of amino acid catabolism by a fasting-inducible mitochondrial carrier

This study identifies SLC25A34 as a fasting-inducible mitochondrial carrier that mediates PEP import, linking amino acid catabolism to muscle preservation during fasting.

Maternal iron deficiency elicits divergent cardiac mitochondrial responses in normotensive and hypertensive pregnancy

This study examines how maternal iron deficiency affects cardiac mitochondrial function in hypertensive and normotensive pregnancies, finding altered mitochondrial structure and respiration with no apoptosis.

SARS-CoV-2 ORF7a Drives Mitochondrial Dysfunction via PDK4 Activation and Complex I Inhibition

This study shows that SARS-CoV-2 ORF7a causes mitochondrial dysfunction by activating PDK4 and inhibiting complex I, altering host metabolism.

Bioenergetic dysfunction and inflammation in hiPSC-derived astrocytes from m.14484T>C Leber's Hereditary Optic Neuropathy

This study investigates the role of astrocytes in LHON pathology using iPSC-derived astrocytes, finding bioenergetic dysfunction and differences between recovery and non-recovery astrocytes.

Bioenergetic profiling of fresh human kidney tissue reveals compensatory metabolic adaptation and intrinsic mitochondrial dysfunction in diabetes

This study profiles bioenergetic changes in fresh human kidney tissue from diabetic patients with preserved function, revealing compensatory metabolic adaptation and intrinsic mitochondrial dysfunction.

Spatial control of mitochondrial retrograde signaling by nuclear pore-associated contact sites

The paper shows that perinuclear clustering of mitochondria forms contact sites that facilitate GPS2 nuclear entry, essential for retrograde signaling, while TSPO-driven MNCS are not required.

Targeted modulation of IGFBP5/IGF1, THPO, and P38 MAPK signaling are potent therapeutic strategies generalizable for mitochondrial respiratory chain disease and osteosarcoma

The paper identifies THPO, IGF1, and MAPK as therapeutic targets for mitochondrial respiratory chain disease and osteosarcoma, showing their modulation can replicate the benefits of CHX treatment.

NDUFV2P1-driven modulation of mitochondrial and neuronal activities; implications to schizophrenia

This study shows that NDUFV2P1 modulates mitochondrial and neuronal function in schizophrenia by interfering with NDUFV2, suggesting therapeutic potential for targeting PG in bioenergetic disorders.

The blood proteome predicts the impact of circulating factors on age-related mitochondrial health

The paper develops a proteomic signature, MitoAge, that predicts age-related mitochondrial health based on circulating factors in serum.

PINK1 stabilizes RMDN3-VAPB interaction without altering the landscape of mito-ER contact sites

The paper investigates how PINK1 stabilizes the RMDN3-VAPB interaction at mitochondrial-ER contact sites during depolarization without changing the proteomic landscape.

The ribosome-associated quality control factor Vms1 protects mitochondrial import and homeostasis during translation stress

The paper shows that Vms1 protects mitochondrial import and homeostasis during translation stress by interacting with Msp1.

Mathematical Modelling of the Mitochondrial Dicarboxylate Carrier (SLC25A10)

The paper develops a mechanistically derived model of SLC25A10 transport, incorporating competitive binding and electroneutrality, and validates it with experimental data.

Integrative structure determination of a human mitochondrial contact site and cristae organizing system (MICOS) sub-assembly

The paper determines the structure of a MICOS sub-assembly using integrative methods, revealing novel interfaces and mutation hotspots.

Large-scale single-cell mapping of concealed mitochondrial ageing

The paper maps concealed mitochondrial DNA mutations across 2.7 million cells from 311 individuals, finding that low-prevalence mutations accumulate with age and predict human age.

CBR4 is essential for mice but not for skeletal muscle function

This study shows that CBR4 is essential in mammals but not for skeletal muscle function, as muscle-specific Cbr4 knockout in mice did not cause measurable defects.

MTERF1 loss buffers against pathogenic mtDNA deletions through transcriptional regulation

The paper identifies MTERF1 as a suppressor of mtDNA deletion pathologies by showing its loss enhances mitochondrial function through transcriptional relief.

The Disease Gene THAP12 is a Transcriptional Regulator of Mitochondrial ETC Complex I

This study identifies THAP12 as a transcriptional regulator of mitochondrial complex I biogenesis and shows its role in neurodevelopmental disorders.

Mitochondrial structural and functional defects in the Drosophila melanogaster model of PLA2G6 Associated Neurodegeneration (PLAN)

This study examines mitochondrial defects in Drosophila melanogaster iPLA2-VIA mutants, showing structural and functional impairments across tissues and age.

Loss of CHCHD2 and CHCHD10 reveals differential vulnerability to bioenergetic failure in cardiac and skeletal muscle

This study shows that loss of CHCHD2 and CHCHD10 causes tissue-specific bioenergetic defects in cardiac and skeletal muscle, leading to distinct pathological outcomes.

Mitochondrial inner membrane partners of Aurora kinase A/AURKA and PHB2 shape organelle metabolic heterogeneity.

The paper identifies mitochondrial inner membrane proteins interacting with AURKA and PHB2, showing their role in shaping metabolic heterogeneity and mitochondrial morphology in cancer cells.

Reactivation of DRP1 plays a functional role in resistance to MEK inhibition in pancreatic cancer cells

This study identifies DRP1 reactivation as a mechanism of resistance to MEK inhibition in pancreatic cancer cells and shows that inhibiting DRP1 can restore sensitivity to trametinib.

Adenine nucleotide translocase 2 (ANT2) deficiency reprograms ferroptosis in alveolar progenitor cells to promote emphysema

This study shows that ANT2 deficiency in AT2 cells promotes ferroptosis and emphysema by impairing bioenergetics and self-renewal.