bims-miptne Biomed News
on Mitochondrial permeability transition pore-dependent necrosis
Issue of 2026–04–26
six papers selected by
Oluwatobi Samuel Adegbite, University of Liverpool



  1. J Biol Chem. 2026 Apr 17. pii: S0021-9258(26)00345-5. [Epub ahead of print] 111473
      Mitochondrial Ca2+ uptake via the mitochondrial Ca2+ uniporter (MCU) following store-operated Ca2+ entry (SOCE) supports cellular bioenergetics, yet how mitochondria shape SOCE and cytosolic Ca2+ signaling remains incompletely understood. Combining gene deletion and functional Ca2+ imaging techniques with a rigorous transcriptomic filter, we find larger cytosolic Ca2+ signals in CRISPR/Cas9-generated Mcu knockout cells. This increase arises primarily from slower cytosolic Ca2+ clearance rather than increased store-operated Ca2+ release-activated Ca2+ (CRAC) channel activity. Compensatory upregulation of cytosolic Ca2+ regulators, such as the plasma membrane Ca2+ ATPase (PMCA) pump that extrudes excess cytosolic Ca2+, is insufficient to restore normal Ca2+ homeostasis. Re-expression of wild-type MCU restored the cytosolic Ca2+ dynamics but a channel pore-dead MCU mutant did not. Deletion of Mcu resulted in major alterations in the transcriptome and re-expression of the protein significantly restored 15% of more than 200 common genes that showed differential expression in two independent knockout clones. Our results identify a set of candidate MCU-dependent genes that may contribute to the regulation of cellular Ca2+ signaling, and show how cytosolic Ca2+ signals can be enhanced in the absence of MCU without an increase in CRAC channel activity.
    Keywords:  Ca(2+) release-activated Ca(2+) (CRAC) channel; Calcium homeostasis; Cytosolic calcium clearance; Mitochondrial calcium uniporter (MCU); Plasma membrane Ca(2+) ATPase (PMCA); Store-operated calcium entry (SOCE); Transcriptomics
    DOI:  https://doi.org/10.1016/j.jbc.2026.111473
  2. Am J Physiol Heart Circ Physiol. 2026 Apr 21.
      Heart failure (HF) affects over 60 million people worldwide, with increasing prevalence as HF with preserved ejection fraction (HFpEF) among adults. Although metabolic remodeling and mitochondrial dysfunction are central features of HFpEF, the direct mechanistic link between altered cardiac metabolism and mitochondrial impairment remains elusive. Here, we investigated how cardiac metabolic remodeling drives mitochondrial impairment, leading to diastolic dysfunction in HFpEF, independent of extracardiac metabolic syndrome. Infusion of angiotensin-II (1.5 μg/g/day) and phenylephrine (50 μg/g/day) in 8-10-week-old male and female mice reproduced hallmark HFpEF features, including preserved EF, elevated E/E' ratio, reduced physical endurance, and impaired lung function. Cardiac mitochondria showed markedly reduced respiration, diminished complex II abundance, and impaired mitochondrial supercomplexes, accompanied by a ~20% reduction in mitochondrial calcium retention capacity and increased susceptibility to opening of the mitochondrial permeability transition pore (mPTP). Metabolomic analysis suggests a shift in mitochondrial metabolism from fatty acid (FA) to the utilization of alternative glucose substrates, characterized by reduced mitochondrial FA trafficking despite increased FA translocase. Dicarbonyl and glycative stress were substantially elevated, with mitochondrial protein glycation increased by 7-fold. Mass spectrometry identified 18 mitochondrial proteins present in a significantly glycated form, with potential implications for impairing metabolic flexibility, reducing electron transport efficiency, and promoting susceptibility to mPTP opening. Our findings demonstrate that metabolic remodeling contributes to dicarbonyl and glycative stress, which in turn compromises the integrity of mitochondrial electron transport complexes, respiratory function, and calcium retention capacity in the HFpEF heart, highlighting mitochondrial dicarbonyl detoxification and anti-glycation strategies as promising therapeutic avenues.
    Keywords:  Heart failure with preserved ejection fraction; metabolic remodeling; mitochondrial health; mitochondrial respiration
    DOI:  https://doi.org/10.1152/ajpheart.00029.2026
  3. Circ J. 2026 Apr 18.
       BACKGROUND: In patients with pulmonary arterial hypertension, death due to ventricular arrhythmias accounts for 8-26% of total deaths, so in this study we investigated whether mitochondrial Ca2+uptake affects ventricular arrhythmias in right ventricular hypertrophy (RVH).
    METHODS AND RESULTS: A total of 70 rats were subcutaneously injected with monocrotaline (MCT-rats) or solvent; 8 mice underwent pulmonary artery banding (PAB) surgery. At 4 weeks after MCU injection or PAB surgery, trabeculae were dissected from the RVs. Levels of mitochondrial Ca2+, cytoplasmic Ca2+, and reactive oxygen species (ROS) were measured using fluorescence dyes. Mitochondrial calcium uniporter (MCU) expression was measured by Western blotting. Ca2+waves and arrhythmias were induced by electrical stimulation (24℃). Both MCT-rats and PAB-mice showed RVH. Ru360, an MCU inhibitor, improved arrhythmias in trabeculae from severe RVH, whereas it worsened them in trabeculae from milder RVH in MCT-rats. Depending on the degree of RVH, Ru360 decreased rhod-2 fluorescence in both MCT-rats and PAB-mice, and decreased Ca2+wave velocity, the 2',7'-dichlorofluorescein fluorescence slope, and MitoSox Red fluorescence in the MCT-rats. MCU expression increased with the degree of RVH.
    CONCLUSIONS: Inhibition of mitochondrial Ca2+uptake improved arrhythmias in severe RVH, but worsened them in milder RVH, due to differences in mitochondrial Ca2+uptake, ROS production, and MCU expression.
    Keywords:  Arrhythmia; Calcium; Mitochondria; Right ventricular hypertension
    DOI:  https://doi.org/10.1253/circj.CJ-25-0856
  4. J Biol Chem. 2026 Apr 16. pii: S0021-9258(26)00327-3. [Epub ahead of print] 111455
      Mitochondrial translation is crucial for maintaining cellular respiration, energy balance, calcium signaling, apoptosis, immune surveillance, and the regulation of inflammatory responses. This specialized process, involving mitochondrial rRNAs, tRNAs, mitoribosomes, and nuclear-encoded translation factors, ensures the synthesis of mitochondrially encoded proteins that support oxidative phosphorylation. The mitochondrial translation cycle is tightly regulated by RNA-binding proteins, mitochondrial unfolded protein response, and stress-responsive pathways such as mTOR, particularly during metabolic shifts and immune activation. Emerging evidence highlights mitochondrial translation as a critical modulator of inflammation. In this review, we describe the alteration in mitochondrial-specific translation dynamics in immune cells, its adaptation to stress, and its interplay with organelle-wide signaling via mito-nuclear and mito-cytosolic communication. We focus on the alterations in mitochondrial translation machinery including mitoribosomal proteins, rRNA, tRNA synthetases or other regulatory factors linked to inflammatory diseases, including neurodegeneration, IBD, metabolic and cardiovascular disorders. We further examine how mitochondrial translation influences immune responses through mitochondrial DNA/RNA release, activation of mitochondrial damage-associated molecular patterns, and inflammasomes such as NLRP3. Collectively, mitochondrial translation functions as an immune centric-checkpoint that presents promising therapeutic target for intervention in inflammation-driven diseases.
    DOI:  https://doi.org/10.1016/j.jbc.2026.111455
  5. Front Oncol. 2026 ;16 1775602
       Background: Mitochondrial permeability transition-driven necrosis (MPT-DN) is a therapeutic target and critical driver of prostate adenocarcinoma (PRAD) progression. We investigated MPT-DN-related prognostic features in PRAD.
    Methods: PRAD transcriptomics and MPT-DN-RGs were sourced from public databases. WGCNA, differential expression, Cox regression, and machine learning identified BCR-FS prognostic genes. These genes built a risk model, revealing independent prognostic factors. Patients were stratified into high/low-risk groups. Pathways, immune microenvironment, and drug sensitivities were analyzed between groups. Finally, protein expression was validated in PCa versus normal tissues.
    Results: TREM2, FNDC1, and S100A8 were identified as prognostic genes. The developed risk model demonstrated strong predictive capabilities in BCR-FS, and subsequent analysis confirmed risk score, Gleason, T stage, and prostate specific antigen (PSA) as independent prognostic factors. The majority of the enrichment pathways in the high-risk group (HRG) and low-risk group (LRG) were related to the metabolism. Moreover, it was found that HRG and LRG displayed distinct immune landscapes, with HRG exhibiting immune exclusion and stronger immune evasion capabilities. Lastly, analysis of drug sensitivity showed significant differences for 6 drugs, with all values being lower in the HRG.
    Conclusion: This study identified TREM2, FNDC1, and S100A8 as key MPT-driven necrosis-related genes predicting biochemical recurrence in PRAD. The risk model effectively stratified patients, revealing immune exclusion and drug resistance in high-risk cases, offering prognostic and therapeutic insights.
    Keywords:  biochemical recurrence-free survival; mitochondrial permeability transition-driven necrosis; prognostic genes; prostate adenocarcinoma; risk score
    DOI:  https://doi.org/10.3389/fonc.2026.1775602
  6. Nat Commun. 2026 Apr 22.
      Mitochondrial dysfunction is a major contributor to myocardial ischemia-reperfusion injury, and limits cardiac recovery after blood flow is restored. Although mitochondria transplantation may help restore cellular energy metabolism, its therapeutic benefit is reduced by extracellular calcium-induced mitochondrial damage. Here we show that a thermosensitive phase-separated hydrogel made of gelatin and PEG can condense, protect and deliver freshly isolated mitochondria. Compared with conventional single-phase hydrogels, this system remains injectable at physiological temperature and enables rapid mitochondria release after transplantation. Furthermore, the phase-separated structure improves mitochondrial packing and preserves activity through spatial confinement and calcium chelation by gelatin. In vitro, condensed mitochondria show improved membrane potential and ATP production. In vivo, transplanted mitochondria are efficiently internalized by cardiomyocytes, improving cardiac function and reducing tissue injury after myocardial ischemia-reperfusion. These findings identify phase-separated hydrogels as a promising platform for mitochondria transplantation.
    DOI:  https://doi.org/10.1038/s41467-026-71765-6