bims-mikwok Biomed News
on Mitochondrial quality control
Issue of 2025–02–16
48 papers selected by
Gavin McStay, Liverpool John Moores University



  1. Trends Cell Biol. 2025 Feb 07. pii: S0962-8924(25)00003-0. [Epub ahead of print]
      The selective removal of mitochondria by mitophagy proceeds via multiple mechanisms and is essential for human well-being. The PINK1/Parkin and NIX/BNIP3 pathways are strongly linked to mitochondrial dysfunction and hypoxia, respectively. Both are regulated by ubiquitylation and mitochondrial import. Recent studies have elucidated how the ubiquitin kinase PINK1 acts as a sensor of mitochondrial import stress through stable interaction with a mitochondrial import supercomplex. The stability of BNIP3 and NIX is regulated by the SCFFBXL4 ubiquitin ligase complex. Substrate recognition requires an adaptor molecule, PPTC7, whose availability is limited by mitochondrial import. Unravelling the functional implications of each mode of mitophagy remains a critical challenge. We propose that mitochondrial import stress prompts a switch between these two pathways.
    Keywords:  BNIP3; FBXL4; PINK1; PPTC7; mitophagy; ubiquitin
    DOI:  https://doi.org/10.1016/j.tcb.2025.01.003
  2. Stem Cell Rev Rep. 2025 Feb 12.
      FBXL4- related mitochondrial depletion syndrome is a very rare inherited disorder characterized by global developmental delays, hypotonia, seizures, growth failure, and early onset lactic acidosis. Often, it is associated with structural brain and heart defects, and facial dysmorphism suggesting an embryogenesis defect. FBXL4 encodes F-box and leucine-rich repeat protein 4 (FBXL4) which is involved in mitochondrial quality control and maintenance by regulating mitophagy. A recent study suggests that FBXL4 deficiency leads to increased mitophagy. Fine tuning of mitophagy is essential for stem cell differentiation during embryogenesis. The disruption of this process is the likely explanation of developmental defects in FBXL4- related mitochondrial depletion syndrome.
    Keywords:  Embryogenesis; FBXL4; Mitochondria; Mitophagy; Stem cell Differentiation
    DOI:  https://doi.org/10.1007/s12015-025-10854-3
  3. Int J Mol Sci. 2025 Feb 03. pii: 1290. [Epub ahead of print]26(3):
      Neurotrophic keratopathy (NK) is a degenerative corneal disease characterized by impaired corneal sensitivity and epithelial repair that is often linked to sensory nerve dysfunction. To establish a clinically relevant model and explore the mechanisms underlying NK pathogenesis, we developed a novel mouse model through partial transection of the ciliary nerve. This approach mimics the progressive nature of NK, reproducing key clinical features such as corneal epithelial defects, reduced sensitivity, diminished tear secretion, and delayed wound healing. Using this model, we investigated how disruptions in mitochondrial dynamics contribute to corneal epithelial dysfunction and impaired repair in NK. Our findings revealed substantial disruptions in mitochondrial dynamics, including reduced expression of fusion proteins (OPA1), downregulation of fission regulators (FIS1 and MFF), and impaired mitochondrial transport, as evidenced by decreased expression of Rhot1 and Kif5b. Additionally, the downregulation of mitophagy-related genes (Pink1 and Prkn) contributed to the accumulation of dysfunctional mitochondria, leading to DNA damage and impaired corneal epithelial repair. These mitochondrial abnormalities were accompanied by increased γH2AX staining, indicative of DNA double-strand breaks and cellular stress. This study highlights the pivotal role of mitochondrial dynamics in corneal epithelial health and repair, suggesting that therapeutic strategies aimed at restoring mitochondrial function, enhancing mitophagy, and mitigating oxidative stress may offer promising avenues for treating NK.
    Keywords:  corneal epithelial repair; corneal nerve; mitochondrial dynamics; neurotrophic keratopathy
    DOI:  https://doi.org/10.3390/ijms26031290
  4. Neurobiol Dis. 2025 Feb 10. pii: S0969-9961(25)00054-3. [Epub ahead of print] 106838
       BACKGROUND: Despite the availability of various antiepileptic treatments, approximately 30 % of epilepsy patients remain refractory to conventional therapies, underscoring the need for neuroprotective strategies. This study investigates the role of GrpEL1 in modulating the mitochondrial unfolded protein response (UPRmt) and its potential protective effects on hippocampal neurons following experimental status epilepticus (SE).
    METHODS: The effects of GrpEL1 were assessed in vivo using a Lithium-pilocarpine rat model of SE and in vitro with glutamate-treated HT22 hippocampal cells. Protein expression and interactions were analyzed by Western blot, immunofluorescence, and co-immunoprecipitation. Neuronal survival was evaluated through Nissl staining. Mitochondrial function was evaluated aggresome formation, mitochondrial membrane potential (MMP) assays, mitochondrial oxygen consumption rate (OCR) measurements, and behavioral assessments using the Morris water maze.
    RESULTS: In the SE rat model, mtHSP70 levels were significantly upregulated in mitochondria, while GrpEL1 expression remained relatively stable. Overexpression of GrpEL1 led to a reduction in neuronal damage and improved functional recovery post-SE. In vitro, GrpEL1 overexpression enhanced the GrpEL1-mtHSP70 interaction, reduced the accumulation of misfolded proteins, and decreased neuronal apoptosis. Furthermore, GrpEL1 overexpression mitigated mitochondrial dysfunction by preserving MMP and improving mitochondrial bioenergetics, as evidenced by enhanced mitochondrial OCR.
    CONCLUSION: GrpEL1 plays a crucial role in maintaining mitochondrial proteostasis and mitigating hippocampal neuronal injury following SE by regulating UPRmt. These findings suggest that GrpEL1 may represent a promising target for therapeutic intervention to protect against seizure-induced neurodegeneration.
    Keywords:  GrpEL1; Hippocampal neurons; Mitochondrial dysfunction; Mitochondrial unfolded protein response (UPRmt); Neuronal protection; Status epilepticus
    DOI:  https://doi.org/10.1016/j.nbd.2025.106838
  5. Elife. 2025 Feb 12. pii: RP100541. [Epub ahead of print]13
      Excessive mitochondrial fragmentation is associated with the pathologic mitochondrial dysfunction implicated in the pathogenesis of etiologically diverse diseases, including many neurodegenerative disorders. The integrated stress response (ISR) - comprising the four eIF2α kinases PERK, GCN2, PKR, and HRI - is a prominent stress-responsive signaling pathway that regulates mitochondrial morphology and function in response to diverse types of pathologic insult. This suggests that pharmacologic activation of the ISR represents a potential strategy to mitigate pathologic mitochondrial fragmentation associated with human disease. Here, we show that pharmacologic activation of the ISR kinases HRI or GCN2 promotes adaptive mitochondrial elongation and prevents mitochondrial fragmentation induced by the calcium ionophore ionomycin. Further, we show that pharmacologic activation of the ISR reduces mitochondrial fragmentation and restores basal mitochondrial morphology in patient fibroblasts expressing the pathogenic D414V variant of the pro-fusion mitochondrial GTPase MFN2 associated with neurological dysfunctions, including ataxia, optic atrophy, and sensorineural hearing loss. These results identify pharmacologic activation of ISR kinases as a potential strategy to prevent pathologic mitochondrial fragmentation induced by disease-relevant chemical and genetic insults, further motivating the pursuit of highly selective ISR kinase-activating compounds as a therapeutic strategy to mitigate mitochondrial dysfunction implicated in diverse human diseases.
    Keywords:  cell biology; human; integrated stress response; mitochondrial fragmentation; mitochondrial morphology; mouse; stress signaling
    DOI:  https://doi.org/10.7554/eLife.100541
  6. FASEB J. 2025 Feb 28. 39(4): e70397
      Myocardial dysfunction is a significant complication of sepsis that is associated with elevated mortality rates. Transmembrane BAX inhibitor motif containing 1 (TMBIM1), a stress-responsive protein, has garnered interest in the field of cardiovascular disease for its cardioprotective properties. Nevertheless, the role of TMBIM1 on sepsis-induced cardiac dysfunction (SICD) remains unknown. Here, our findings revealed a significant elevation in TMBIM1 expression within the myocardium following endotoxin challenge and further demonstrate the cardioprotective effects of TMBIM1 through adenovirus-mediated gene manipulation. Notably, lipopolysaccharide exposure markedly induced mitochondrial dysfunction in cardiomyocytes, which was effectively alleviated by TMBIM1 overexpression, while TMBIM1 knockdown exacerbated this dysfunction. Moreover, in cardiomyocytes subjected to endotoxin challenge, TMBIM1 was observed to interact with Parkin, facilitating its translocation from the cytosol to damaged mitochondria. This interaction enhanced the activation of mitophagy, thereby promoting the clearance of dysfunctional mitochondria and subsequently mitigating cellular injury. Hence, targeting TMBIM1 could be a novel therapeutic strategy for treating SICD.
    Keywords:  Parkin; TMBIM1; mitophagy; myocardial injury; sepsis
    DOI:  https://doi.org/10.1096/fj.202402599RR
  7. PLoS One. 2025 ;20(2): e0317080
       BACKGROUND: Chronic stress has emerged as a prevalent facet of contemporary existence, significantly jeopardizing overall bodily health. The liver, a pivotal organ responsible for metabolic equilibrium, is particularly vulnerable to its adverse effects. This study delves into the hepatoprotective properties of extended consumption of HRW in mice subjected to chronic stress.
    METHODS: Mice subjected to chronic stress via CUMS and HRW administration for seven months underwent liver pathological examination. Key liver function indicators (AST, ALT), oxidative stress markers (SOD, CAT, GSH), and markers related to lipid peroxidation and ferroptosis (MDA, Fe) were measured using standard kits. ELISA determined corticosterone and 4-HNE levels. Immunofluorescence evaluated ROS, Nrf2, and apoptosis in liver tissues. Western blotting analyzed markers for ferroptosis (GPX4, SLC7A11, HO-1, Nrf2), apoptosis (Bax, Bcl-2, Cytc, Caspase-3, Caspase-8), mitochondrial biogenesis (Nrf1, PGC-1α, Tfam), and quality control (Drp1, Fis1, Mfn1, Mfn2, OPA1, PINK1, Parkin, LC3 I/II).
    RESULTS: The findings indicate a noteworthy improvement in liver health among mice exposed to HRW, as evidenced by histological analysis. Furthermore, the consumption of HRW exhibited hepatoprotection, as evidenced by the normalization of AST and ALT levels. Mechanistically, our results indicate that HRW elevates the levels of SOD, CAT, and GSH, while effectively clearing ROS within mitochondria. It was observed led to a regulation in the expression of mitochondrial quality control proteins, consequently improving mitochondrial biogenesis (Nrf1, PGC-1α, Tfam), and increasing ATP production. Furthermore, HRW decreased Cytc, Bax, Caspase-3, and Caspase-8 levels, and increasing the expression of Bcl-2. Additionally, HRW reduced MDA and 4-HNE levels, alleviating ferroptosis through the Nrf2/HO-1 pathway, and upregulating the expression of GPX4 and SLC7A11. By mitigating hepatocyte death through the aforementioned mechanisms, HRW fulfills its crucial role in safeguarding liver health.
    CONCLUSIONS: This study reveals that long-term hydrogen-rich water (HRW) consumption provides significant hepatoprotection in mice under chronic stress. HRW normalizes liver enzyme levels, enhances antioxidant capacity, and reduces lipid peroxidation and ferroptosis. It improves mitochondrial biogenesis, function, and ATP production, and attenuates apoptosis by modulating related proteins. Behavioral tests show HRW alleviates stress-induced anxiety and enhances exploratory behavior. These findings suggest HRW is a promising non-invasive intervention for preventing and treating stress-related liver disorders by targeting oxidative stress and mitochondrial dysfunction.
    DOI:  https://doi.org/10.1371/journal.pone.0317080
  8. Int J Mol Sci. 2025 Feb 04. pii: 1325. [Epub ahead of print]26(3):
      Dysfunctional mitochondria are present in many neurodegenerative diseases, such as spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD). SCA3/MJD, the most frequent neurodegenerative ataxia worldwide, is caused by the abnormal expansion of the polyglutamine tract (polyQ) at ataxin-3. This protein is known to deubiquitinate key proteins such as Parkin, which is required for mitophagy. Ataxin-3 also interacts with Beclin1 (essential for initiating autophagosome formation adjacent to mitochondria), as well as with the mitochondrial cristae protein TBK1. To identify other proteins of the mitophagy pathway (according to the KEGG database) that can interact with ataxin-3, here we developed a pipeline for in silico analyses of protein-protein interactions (PPIs), called auto-p2docking. Containerized in Docker, auto-p2docking ensures reproducibility and reduces the number of errors through its simplified configuration. Its architecture consists of 22 modules, here used to develop 12 protocols but that can be specified according to user needs. In this work, we identify 45 mitophagy proteins as putative ataxin-3 interactors (53% are novel), using ataxin-3 interacting regions for validation. Furthermore, we predict that ataxin-3 interactors from both Parkin-independent and -dependent mechanisms are affected by the polyQ expansion.
    Keywords:  SCA3/MJD; in silico; mitophagy; pipeline
    DOI:  https://doi.org/10.3390/ijms26031325
  9. J Cell Mol Med. 2025 Feb;29(3): e70419
      Lipotoxicity plays a crucial role in the progression of diabetic kidney disease (DKD), yet the dynamic changes in renal lipid composition from diabetes to early-stage DKD remain unclear. Free fatty acids, lactosylceramides and cardiolipin (CL) were identified as the most significantly altered lipids by quantitatively comparing targeted lipids in the renal cortex of the classic spontaneous diabetic db/db mice using high-coverage targeted lipidomics. Further investigation into the causes and effects of decreased CL, which is a unique mitochondrial phospholipid, was conducted in mitochondria-rich renal proximal tubular cells by using western blotting, real-time PCR, immunohistochemistry and transmission electron microscopy. Reduced expression of cardiolipin synthase, a key enzyme in the CL synthesis pathway, and inhibition of CL-related mitophagy were confirmed under high glucose conditions. In addition, the protective effect of CL-targeted Szeto-Schiller 31 in preserving mitophagy was demonstrated in both in vivo and in vitro studies. These findings provide new insights into the pathogenesis of early-stage DKD from a lipid perspective and offer a theoretical basis for discovering new treatments.
    Keywords:  Szeto‐Schiller 31; cardiolipin; cardiolipin synthase; diabetic kidney disease; free fatty acid; lipidomics; mitophagy; proximal tubule; sphingolipid
    DOI:  https://doi.org/10.1111/jcmm.70419
  10. Nat Commun. 2025 Feb 10. 16(1): 1501
      Mitochondria are essential organelles and their functional state dictates cellular proteostasis. However, little is known about the molecular gatekeepers involved, especially in absence of external stress. Here we identify a role of MFN2 in quality control independent of its function in organellar shape remodeling. MFN2 ablation alters the cellular proteome, marked for example by decreased levels of the import machinery and accumulation of the kinase PINK1. Moreover, MFN2 interacts with the proteasome and cytosolic chaperones, thereby preventing aggregation of newly translated proteins. Similarly to MFN2-KO cells, patient fibroblasts with MFN2-disease variants recapitulate excessive protein aggregation defects. Restoring MFN2 levels re-establishes proteostasis in MFN2-KO cells and rescues fusion defects of MFN1-KO cells. In contrast, MFN1 loss or mitochondrial shape alterations do not alter protein aggregation, consistent with a fusion-independent role of MFN2 in cellular homeostasis. In sum, our findings open new possibilities for therapeutic strategies by modulation of MFN2 levels.
    DOI:  https://doi.org/10.1038/s41467-025-56673-5
  11. Mol Nutr Food Res. 2025 Feb 09. e202400699
       SCOPE: Polysaccharides from Tremella fuciformis have gained significant interest due to their diverse biological activities. This study focuses on characterizing a purified polysaccharide, TPSP2, extracted from T. fuciformis and evaluating its antiobesity effect and underlying mechanisms in vivo.
    METHODS AND RESULTS: Structural analysis revealed that TPSP2, with a molecular weight of 1.51 × 103 kDa, is composed of mannose, rhamnose, glucuronic acid, galactose, xylose, arabinose, and fucose in specific molar ratios. The primary linkages identified include t-Fuc(p), 1,2-Xyl(p), t-GlcA(p), 1,3-Man(p), and 1,2,3-Man(p), with their corresponding ratios being 12.987%, 11.404%, 16.050%, 16.527%, and 26.624%, respectively. In vivo experiments demonstrated that TPSP2 significantly alleviated high-fat diet-induced weight gain, hyperlipidemia, hepatic steatosis, hyperglycemia, and insulin resistance in mice. Mechanistically, TPSP2 was found to enhance AMPK/PINK1-PRKN-dependent mitophagy by upregulating the p-AMPK/AMPK ratio, LC3-II/I ratio, and expression of PINK1, PRKN, prohibitin 2 (PHB2), and LAMP2, while downregulating p62 and TOM20 expression.
    CONCLUSION: This study suggested that TPSP2 could be a promising candidate for addressing obesity-related metabolic disorders by targeting mitochondrial quality control mechanisms.
    Keywords:  PINK1/PRKN; Tremella fuciformis polysaccharides; antiobesity activity; mitophagy; structural characterization
    DOI:  https://doi.org/10.1002/mnfr.202400699
  12. Future Oncol. 2024 Dec 29. 1-11
      Breast cancer (BC) is a global women's health concern, with ongoing research to address diagnostic and treatment challenges. Understanding the developmental mechanisms is vital for improved clinical prevention and treatment strategies. Mitochondria, undergo dynamic processes like fission and fusion, regulated by proteins like Dynamic-related protein 1 (DRP1). DRP1 plays a key role in mitochondrial fission, a process associated with BC development. This study aims to explore the impact of DRP1 on BC growth, assessing its potential as a therapeutic target. The findings could contribute to a better understanding of mitochondria-related molecular mechanisms in BC development and guide the identification of clinical drug targets.
    Keywords:  Breast cancer; DRP1; fission; fusion; mitochondrial
    DOI:  https://doi.org/10.1080/14796694.2024.2447813
  13. Apoptosis. 2025 Feb 09.
      Subarachnoid hemorrhage (SAH), representing 5-10% of all stroke cases, is a cerebrovascular event associated with a high mortality rate and a challenging prognosis. The role of IRG1-regulated itaconate in bridging metabolism, inflammation, oxidative stress, and immune response is pivotal; however, its implications in the early brain injury following SAH remain elusive. The SAH nerve inflammation model was constructed by Hemin solution and BV2 cells. In vitro and in vivo SAH models were established by intravascular puncture and Hemin solution treatment of HT22 cells. To explore the relationship between IRG1 and neuroinflammation by interfering the expression of Irg1 in BV2 cells. By adding itaconate and its derivatives to explore the relationship between mitophagy and ferroptosis. IRG1 knockdown increased the expression of inflammatory factors and induced the transformation of microglia to pro-inflammatory phenotype after SAH; Itaconate and itaconate derivative 4-OI can reduce oxidative stress and lipid peroxidation level in neuron after SAH, and reduce EBI after SAH; IRG1/ itaconate promotes mitophagy through PINK1/Parkin signaling pathway to inhibit neuronal ferroptosis. IRG1 can improve nerve inflammation after SAH, M2 of microglia induced polarization. IRG1/ Itaconate participates in mitophagy through PINK1/Parkin to alleviate neuronal ferroptosis after SAH and play a neuroprotective role.
    Keywords:  Ferroptosis; IRG1; Itaconate; Mitophagy; Subarachnoid hemorrhage
    DOI:  https://doi.org/10.1007/s10495-025-02077-1
  14. Oral Dis. 2025 Feb 10.
       OBJECTIVES: Periodontal disease is a common chronic inflammatory condition affecting the tissues that support teeth, leading to their destruction. Mitophagy, a specialized form of autophagy responsible for degrading damaged mitochondria, plays a crucial role in maintaining cellular homeostasis. However, its role in periodontal disease progression remains poorly understood. This review aims to summarize recent research on mitophagy's role in periodontal disease pathogenesis.
    METHODS: A comprehensive literature review on mitophagy was conducted using PubMed, Scopus, and Web of Science databases, employing keywords related to periodontal disease such as "periodontal," "periodontitis," "gingiva," and "gingivitis."
    RESULTS: A review of 18 original studies revealed that mitophagy plays a crucial role in periodontal disease by regulating key pathophysiological mechanisms. Specifically, mitophagy modulates periodontal inflammation by influencing pro-inflammatory cytokines and mitochondrial reactive oxygen species. Additionally, it is essential for alveolar bone remodeling, impacting both bone resorption and regeneration. Mitophagy also regulates cell apoptosis within periodontal tissues, helping to preserve cellular function and tissue integrity during periodontal disease progression.
    CONCLUSIONS: Mitophagy regulates periodontal disease pathogenesis by modulating inflammation, bone remodeling, and cell death in periodontal tissues. Further research is needed to explore its therapeutic potential in periodontal disease treatment and improve targeted interventions.
    Keywords:  alveolar bone remodeling; apoptosis; inflammation; mitophagy; periodontal disease
    DOI:  https://doi.org/10.1111/odi.15279
  15. Sci Rep. 2025 Feb 10. 15(1): 4918
      Skeletal muscle atrophy is a complication of obesity, partially induced by impaired mitophagy. This study investigates whether Berberine(BBR) protects mice from obese skeletal muscle atrophy and the underlying molecular mechanism. Twenty C57BL/6 mice were fed a high-fat diet until they weighed more than 20% of the average body weight of the control group. The mice were then divided into two groups and gavaged with BBR or vehicle for 8 weeks. 10 mice were used as controls. Fasting blood glucose was measured, an oral glucose tolerance test was performed, and the mice were measured for grip strength and exercise capacity. H&E and Oil Red O staining were used to observe the pathological changes of skeletal muscle. MURF1, FBXO32, BAX, BCL2, P62, LC3 and mitophagy receptor FUNDC1 were observed in mice. BBR was intervened in C2C12 myotubes. The role of FUNDC1 was verified by RNA interference. We found that BBR treatment increased grip strength and improved muscle function. BBR not only reduced weight gain, excessive lipid accumulation and hyperlipidemia, but also ameliorated obesity-induced skeletal muscle atrophy and apoptosis. BBR promoted autophagy and increased FUNDC1 protein expression. The same positive effects were observed after BBR intervening on C2C12 myotubes, whereas FUNDC1 RNA interference attenuated the anti-skeletal muscle atrophy effect of BBR. These results suggest that BBR ameliorated obesity-induced skeletal muscle atrophy in mice by modulating the skeletal muscle mitophagy receptor FUNDC1, which may be a potential therapeutic target for obesity-induced skeletal muscle atrophy.
    Keywords:  Berberine; FUNDC1; Mitophagy; Obesity-induced skeletal muscle atrophy
    DOI:  https://doi.org/10.1038/s41598-025-89297-2
  16. Front Cell Dev Biol. 2024 ;12 1490902
      Mitophagy is a critically important survival mechanism in which toxic, aged, or defective mitochondria are segregated into mitophagosomes, which shuttle the damaged mitochondrial segments to the lysosome and proteasome for destruction. Cancer cells rely on mitophagy under conditions of high oxidative stress or increased energy demand. Oxidative stress can generate a large volume of damaged mitochondria, overwhelming lysosomal removal. Accumulated damaged mitochondria are toxic and their proper removal is crucial for maintaining mitochondrial health. We propose a new cancer cell mechanism for survival that is activated when the demand for segregating and eliminating damaged mitochondria exceeds the capacity of the lysosome or proteasome. Specifically, we show that tumor cells subjected to oxidative stress by carbonyl cyanide-3-chlorophenylhdrazone (CCCP) eliminate damaged mitochondria segments by bypassing the lysosome to export them outside the cell via extracellular vesicles (EVs), a process termed "secretory mitophagy". PINK1, the initiator of mitophagy, remains associated with the damaged mitochondria that exported in EVs. Using several types of cancer cells, we show that tumor cells treated with CCCP can be induced to switch over to secretory mitophagy by treatment with Bafilomycin A1, which blocks the fusion of mitophagosomes with lysosomes. Under these conditions, an increased number of PINK1 + EVs are exported. This is associated with greater cell survival by a given CCCP dose, enhanced mitochondrial ATP production, and reduced mitochondrial oxidative damage (membrane depolarization). Our data supports the hypothesis that secretory mitophagy is a previously unexplored process by which cancer cells adapt to survive therapeutic or hypoxic stress. Ultimately, our findings may inform new prevention strategies targeting pre-malignant lesions and therapeutic approaches designed to sensitize tumor cells to oxidative stress-inducing therapies.
    Keywords:  PINK1; cancer progression; cell survival; extracellular vesicles; mitophagy; oxidative stress
    DOI:  https://doi.org/10.3389/fcell.2024.1490902
  17. Acta Neuropathol Commun. 2025 Feb 13. 13(1): 28
      Dominant optic atrophy (DOA) is the most common inherited optic neuropathy, characterised by the selective loss of retinal ganglion cells (RGCs). Over 60% of DOA cases are caused by pathogenic variants in the OPA1 gene, which encodes a dynamin-related GTPase protein. OPA1 plays a key role in the maintenance of the mitochondrial network, mitochondrial DNA integrity and bioenergetic function. However, our current understanding of how OPA1 dysfunction contributes to vision loss in DOA patients has been limited by access to patient-derived RGCs. Here, we used induced pluripotent stem cell (iPSC)-RGCs to study how OPA1 dysfunction affects cellular homeostasis in human RGCs. iPSCs derived from a DOA+ patient with the OPA1 R445H variant and isogenic CRISPR-Cas9-corrected iPSCs were differentiated to iPSC-RGCs. Defects in mitochondrial networks and increased levels of reactive oxygen species were observed in iPSC-RGCs carrying OPA1 R445H. Ultrastructural analyses also revealed changes in mitochondrial shape and cristae structure, with decreased endoplasmic reticulum (ER): mitochondrial contact length in DOA iPSC-RGCs. Mitochondrial membrane potential was reduced and its maintenance was also impaired following inhibition of the F1Fo-ATP synthase with oligomycin, suggesting that mitochondrial membrane potential is maintained in DOA iPSC-RGCs through reversal of the ATP synthase and ATP hydrolysis. These impairments in mitochondrial structure and function were associated with defects in cytosolic calcium buffering following ER calcium release and store-operated calcium entry, and following stimulation with the excitatory neurotransmitter glutamate. In response to mitochondrial calcium overload, DOA iPSC-RGCs exhibited increased sensitivity to opening of the mitochondrial permeability transition pore. These data reveal novel aspects of DOA pathogenesis in R445H patient-derived RGCs. The findings suggest a mechanism in which primary defects in mitochondrial network dynamics disrupt core mitochondrial functions, including bioenergetics, calcium homeostasis, and opening of the permeability transition pore, which may contribute to vision loss in DOA patients.
    Keywords:  Calcium homeostasis; Dominant optic atrophy; Mitochondrial networks; Neurodegeneration; OPA1; Retinal ganglion cells; iPSCs
    DOI:  https://doi.org/10.1186/s40478-025-01942-z
  18. Kidney Int. 2025 Feb 06. pii: S0085-2538(25)00085-7. [Epub ahead of print]
      Chronic kidney disease-mineral and bone disorder (CKD-MBD) presents with extra-skeletal calcification and renal osteodystrophy (ROD). However, the pathophysiology of ROD remains unclear. Here we examine the hypothesis that stalled mitophagy within osteocytes of CKD-MBD mouse models contributes to bone loss. RNA-seq analysis revealed an altered expression of genes associated with mitophagy and mitochondrial function in tibia of CKD-MBD mice. The expression of mitophagy regulators, p62/SQSTM1, ATG7 and LC3 was inconsistent with functional mitophagy, and in mito-QC reporter mice with ROD, there was a two to three-fold increase in osteocyte mitolysosomes. To determine if uremic toxins were potentially responsible for these observations, treatment of cultured osteoblasts with uremic toxins revealed increased mitolysosome number and mitochondria with distorted morphology. Membrane potential and oxidative phosphorylation were also decreased, and oxygen-free radical production increased. The altered p62 /SQSTM1 and LC3-II expression was consistent with impaired mitophagy machinery, and the effects of uremic toxins were reversible by rapamycin. A causal link between uremic toxins and the development of mitochondrial abnormalities and ROD was established by showing that a mitochondria-targeted antioxidant (MitoQ) and the charcoal adsorbent AST-120 were able to mitigate the uremic toxin-induced mitochondrial changes and improve bone health. Overall, our study shows that impaired clearance of damaged mitochondria may contribute to the ROD phenotype. Targeting uremic toxins, oxygen-free radical production and the mitophagy process may offer novel routes for intervention to preserve bone health in patients with CKD-MBD. This would be timely as our current armamentarium of anti-fracture medications for patients with severe CKD-MBD is limited.
    Keywords:  CKD-MBD; autophagy; bone; mitophagy; renal osteodystrophy; uremic toxins
    DOI:  https://doi.org/10.1016/j.kint.2025.01.022
  19. Cell Death Dis. 2025 Feb 13. 16(1): 94
      Intervertebral disc degeneration (IVDD) is a leading cause of low back pain, with limited effective treatments due to an incomplete understanding of disease mechanisms. In this study, we report that circFUNDC1, a nuclear circular RNA, is markedly downregulated in nucleus pulposus cells (NPCs) from patients with end-stage IVDD. CircFUNDC1 is derived from the gene encoding the FUN14 domain-containing 1 (FUNDC1) protein, which is essential for mitophagy and cell survival. Functional analyses reveal that circFUNDC1 plays a crucial role in maintaining extracellular matrix homeostasis by enhancing the expression of anabolic factors in NPCs. Additionally, we identified the transcriptional regulator cyclin-dependent kinase 9 (CDK9) as a novel binding partner for circFUNDC1. Binding with circFUNDC1 recruits CDK9 via complementary nucleotides to the FUNDC1 promoter to stimulate the production of full-length FUNDC1 mRNAs and proteins, forming a positive feedback loop. Overexpression of circFUNDC1 protects NPCs from oxidative stress by promoting mitophagy, reducing reactive oxygen species levels, and inhibiting cellular senescence. Moreover, circFUNDC1 overexpression delays the onset of IVDD in an ex-vivo culture model. This study is the first to demonstrate that circFUNDC1 is vital for protecting NPCs from oxidative stress, suggesting circFUNDC1 as a potential therapeutic target for IVDD.
    DOI:  https://doi.org/10.1038/s41419-025-07425-2
  20. Eur J Pharmacol. 2025 Feb 11. pii: S0014-2999(25)00117-7. [Epub ahead of print] 177364
       BACKGROUND: Supplemental oxygen is commonly used to treat newborns with respiratory disorders. It has been explored that hyperoxia increases oxidative stress, and have the potential adverse effects on developing organs. Mitochondrial biogenesis plays a crucial role in maintaining mitochondrial homeostasis, and resveratrol (Res) has its unique advantage in promoting mitochondrial biogenesis. However, the molecular mechanisms controlling mitochondrial biogenesis in hyperoxia-induced kidney injury remain unclear. The aim of this study was to evaluate the protective effect and it's mechanisms of Res on hyperoxia-induced kidney injury in neonatal rats.
    METHODS: Sprague-Dawley rats were housed in normoxia or hyperoxia (85% O2) and randomized to receive saline, dimethyl sulfoxide, and Res administered intraperitoneally from postnatal days 1∼14(All medicine is scheduled to be given at six o'clock every afternoon). Split the rats into six groups, and on postnatal days 1, 7 and 14, kidney samples were acquired for HE staining and PAS staining to assess kidney development, terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) to detect apoptosis, and real-time quantitative polymerase chain reaction and immunoblotting to detect the expression levels of SIRT1, PGC-1α, NRF1, NRF2 and TFAM.
    RESULTS: Hyperoxia induced tubular and glomerular injury, increased renal tissue apoptosis, decreased Silent information regulator 2-related enzyme 1(SIRT1), Peroxisome proliferator-activated receptor-γ coactivator-1α(PGC-1α), nuclear respiratory factor 1(Nrf1), Nrf2, mitochondrial transcription factor A (TFAM) protein levels in the kidney, and inhibited TFAM mRNA expression in mitochondria, diminished ND1 copy number and ND4/ND1 ratio. In contrast, Res reduced renal injury and attenuated renal tissue apoptosis in neonatal rats and increased the levels of the corresponding indexes.
    CONCLUSIONS: Res protects neonatal rats from hyperoxia-induced kidney injury by promoting mitochondrial biogenesis, possibly in part through activation of the SIRT1/PGC-1α signaling pathway.
    Keywords:  Acute kidney injury; Hyperoxia; Mitochondrial biogenesis; Resveratrol; SIRT1
    DOI:  https://doi.org/10.1016/j.ejphar.2025.177364
  21. J Cell Mol Med. 2025 Feb;29(3): e70400
      Oral squamous cell carcinomas (OSCCs), like several solid tumours, contain heterogeneous subpopulations of a small subset of cancer cells, termed cancer stem cells (CSCs), that are highly relevant to cancer metastasis and invasive properties. CSCs have also shown a high capacity to survive against various stressful environments, such as hypoxia. However, the molecular underpinnings behind the high potential of CSCs to survive under this stress remain unclear. The current study aimed to investigate the significance of autophagy systems in oral CSC maintenance and survival under stress conditions. Human OSCC cell lines OECM-1 and OECM-1 CSCs were cultured in different hypoxic time periods for proliferation and cytotoxicity analyses. The stemness property of CSCs is evaluated by sphere formation, transwell and wound healing assays protein expression of stemness, and epithelial-to-mesenchymal transition markers. Mitochondrial functions, including mitochondrial ROS generation, mitochondria dynamics, mitophagy, and mitochondrial metabolism (glycolysis and oxidative phosphorylation [OXPHOS]) were examined by western blotting, immunohistochemistry, and XF-seahorse assays, respectively. Under hypoxia, oral CSCs showed a higher proliferation rate with increased invasion/migration/EMT properties than OECM-1 cells. Further, hypoxia-induced BNIP3-driven mitophagy was activated in OECM-1 CSCs than in OECM-1 cells, which also triggered a metabolic shift towards OXPHOS, and BNIP3/-L silencing by siRNA significantly attenuated OECM-1 CSCs stemness features. TCGA data analyses also revealed a higher BNIP3 expression in head and neck squamous carcinoma patients' tumour samples associated with lower patient survival. Collectively, our results revealed a BNIP3/-L-driven autophagy contributes to the OECM-1 CSCs stemness features under hypoxia, suggesting a novel therapeutic strategy involving BNIP3 and autophagy inhibition in oral CSCs.
    Keywords:  BNIP3/‐L; Mitophagy; autophagy; cancer stem cells; oral squamous cell carcinoma; oxidative phosphorylation
    DOI:  https://doi.org/10.1111/jcmm.70400
  22. Pak J Pharm Sci. 2024 Nov-Dec;37(6):37(6): 1455-1465
      During the active phase of ulcerative colitis (UC), mitochondrial autophagy is an important antagonistic mechanism. Our investigation examines the regulatory effect of Dima decoction on UC inflammation via the autophagy pathway. SD rats were divided into 5 groups (n = 10): normal control (NC) model, mesalazine, Dima decoction treatment and Dima decoction combined with YC-1 (inhibitor) group. Results showed that treatment of Dima decoction effectively ameliorated the symptoms of UC. Drug-containing serum from Dima decoction treated rats leads to an significantly increase in IL-4 and IL-10 content in HT-29 cells, while also causing a decrease in IL-1β and IL-6 content. Moreover, protein level and mRNA level of HIF-1α, BNIP3, Beclin-1 were obviously up-regulated. In addition, protein level of LC3B II and the ratio of LC3B II/I were dramatically promoted after Dima decoction serum administration. The protein level of Bax was notably decreased in TH-29 cells after Dima decoction serum supplement, while that of Bcl-xl was remarkably up-regulated. In conclusion, Dima decoction significantly alleviated the symptoms of UC. The regulation could involve modulation of the hypoxia-inducible HIF-1 α/BNIP3/Buclin-1 pathways, leading to effects on mitochondrial autophagy and inflammation. These findings offer new insights into the mechanism of Dima decoction for treating UC.
  23. Cell Commun Signal. 2025 Feb 11. 23(1): 77
       BACKGROUND: Chondrocyte senescence play an essential role in osteoarthritis (OA) progression. Recent studies have shown that snoRNA-derived RNA fragments (sdRNAs) are novel regulators of post-transcriptional gene expression. However, the expression profiles and their role in post-transcriptional gene regulation in chondrocyte senescence and OA progression is unknown. Here, we determined sdRNAs expression profile and explored sdRNA-D43 role in OA and its mechanism.
    METHODS: We used qPCR arrays to determine sdRNAs expression in the chondrocytes of areas undamaged and damaged of the three knee OA samples. SdRNA-D43 expression was determined using quantitative reverse transcription-polymerase chain reaction and in situ hybridization. Then, bioinformatics analysis was conducted on the target genes that might be silenced by sdRNA-D43. Primary chondrocytes of damaged regions of knee OA samples were transfected with a sdRNA-D43 inhibitor or mimic to determine their functions, including in relation to mitophagy and chondrocyte senescence. Argonaute2-RNA immunoprecipitation and luciferase reporter assays were conducted to determine the target gene of sdRNA-D43. In a rat OA model induced by monosodium iodoacetate, adeno-associated virus sh-rat-sdRNA-D43 was injected into the knee joint cavity to assess its in vivo effects.
    RESULTS: sdRNA-D43 expression were upregulated in damaged areas of knee OA cartilage with increased senescent chondrocytes. sdRNA-D43 inhibited mitophagy and promoted chondrocytes senescence during OA progression. Mechanistically, sdRNA-D43 silenced the expression of both NRF1 and WIPI2 by binding to their 3'-UTR in an Argonaute2‑dependent manner, which inhibited PINK1/Parkin-mediated pathway. Additionally, injection of AAV-sh-sdRNA-D43 alleviated the progression of OA in a monosodium iodoacetate-induced rat model.
    CONCLUSION: Our results reveal an important role for a novel sdRNA-D43 in OA progression. sdRNA-D43 improves chondrocyte senescence by negatively regulating PINK1/Parkin-mediated mitophagy pathway via dual-targeting NRF1 and WIPI2, which provide a potential therapeutic strategy for OA treatment.
    Keywords:  Chondrocyte senescence; Mitophagy; Osteoarthritis; sdRNA-D43; snoRNA
    DOI:  https://doi.org/10.1186/s12964-024-01975-2
  24. Autoimmunity. 2025 Dec;58(1): 2465410
      Myasthenia gravis (MG) is a common autoimmune disorder that causes skeletal muscle weakness. Most patients presented with skeletal muscle weakness and endurance decline. Mitophagy refers to removing and interpreting aging or damaged mitochondria in cells. This plays a vital role in maintaining cell homeostasis and normal function. This study explores the role of mitophagy-related genes (GM) in MG. Specifically, we collected the transcriptome data of MG and its control group from the GEO database (Gene Expression Omnibus database). The differentially expressed genes (DEGs) were identified by differential analysis and intersected with GM. Multiple machine learning algorithms were applied to screen and verify the diagnostic genes of intersection genes. In addition, we constructed diagnostic models and nomogram models based on diagnostic genes. The immune landscape of MG was explored by ssGSEA analysis. The correlation between the abundance of immune cell infiltration and diagnostic genes was explored by immune infiltration analysis. Finally, the diagnostic genes were further validated by qPCR experiments.
    Keywords:  Myasthenia gravis; diagnostic model; immunization; machine learning; mitophagy; qPCR
    DOI:  https://doi.org/10.1080/08916934.2025.2465410
  25. Discov Oncol. 2025 Feb 09. 16(1): 142
      Glioblastoma (GBM) is a prevalent and refractory type of brain tumor. Over the past two decades, there have been minimal advancements in GBM therapy. The current standard treatment involves surgical excision followed by radiation and chemotherapy. Compared to other tumors, GBM is more challenging to treat due to the presence of glioma stem-like cells (GSCs) and the blood-brain barrier, resulting in an extremely low survival rate. Mitochondria play a critical role in tumor respiration, metabolism, and multiple signaling pathways involved in tumor formation, progression, and cell apoptosis. Consequently, mitochondria represent promising targets for developing novel anticancer agents, including those targeting oxidative phosphorylation, reactive oxygen species (ROS), mitochondrial transfer, and mitophagy. This review outlines the mitochondrial-related therapeutic targets in GBM, highlighting the potential of mitochondria as a target for GBM treatment.
    Keywords:  Glioblastoma; Mitochondria; Mitochondrial autophagy; Mitochondrial metastasis; Oxidative phosphorylation; ROS
    DOI:  https://doi.org/10.1007/s12672-025-01891-y
  26. Zhongguo Zhen Jiu. 2025 Feb 12. 45(2): 193-9
       OBJECTIVE: To observe the effects of electroacupuncture (EA) at "Shenting" (GV24) and "Baihui" (GV20) on mitochondrial autophagy in hippocampal neurons and silent information regulator sirtuin 1 (Sirt1)/forkhead box O3 (FOXO3)/PTEN-inducible kinase 1 (PINK1)/Parkin pathway in rats with learning-memory impairment after cerebral ischemia reperfusion injury.
    METHODS: A total of 35 male SD rats were randomly divided into a sham operation group (9 rats) and a modeling group (26 rats). In the modeling group, middle cerebral artery occlusion method was used to establish the middle cerebral artery ischemia-reperfusion (MCAO/R) model, and 18 rats of successful modeling were randomly divided into a model group and an EA group, 9 rats in each one. EA was applied at "Shenting" (GV24) and "Baihui" (GV20) in the EA group, 30 min a time, once a day for 14 days. After modeling and on 7th and 14th days of intervention, neurologic deficit score was observed; the learning-memory ability was detected by Morris water maze test; the morphology of neurons in CA1 area of hippocampus was detected by Nissl staining; the mitochondrial morphology was observed by transmission electron microscopy; the protein expression of Beclin-1, microtubule-associated protein 1 light chain 3B (LC3B), P62, Sitrt1, FOXO3, PINK1 and Parkin was detected by Western blot.
    RESULTS: After modeling, the neurologic deficit scores in the model group and the EA group were higher than that in the sham operation group (P<0.001); on 7th and 14th days of intervention, the neurologic deficit scores in the model group were higher than those in the sham operation group (P<0.001), the neurologic deficit scores in the EA group were lower than those in the model group (P<0.05, P<0.01). After modeling, the escape latency in the model group and the EA group was prolonged compared with that in the sham operation group (P<0.001); on 9th-13th days of intervention, the escape latency in the model group was prolonged compared with that in the sham operation group (P<0.001), the escape latency in the EA group was shortened compared with that in the model group (P<0.05, P<0.01, P<0.001). The number of crossing plateau in the model group was less than that in the sham operation group (P<0.001); the number of crossing plateau in the EA group was more than that in the model group (P<0.05). In the model group, in CA1 area of hippocampus, the number of neurons was less, with sparse arrangement, nuclear fixation, deep cytoplasmic staining, and reduction of Nissl substance; the morphology of mitochondrion was swollen, membrane structure was fragmented, and autophagic lysosomes were formed. Compared with the model group, in the EA group, in CA1 area of hippocampus, the number of neurons was increased, the number of cells of abnormal morphology was decreased, and the number of Nissl substance was increased; the morphology of mitochondrion was more intact and the number of autophagic lysosomes was increased. Compared with the sham operation group, in the model group, the protein expression of Beclin-1, FOXO3, PINK1, Parkin and the LC3BⅡ/Ⅰ ratio in hippocampus were increased (P<0.01, P<0.001), while the protein expression of P62 was decreased (P<0.05). Compared with the model group, in the EA group, the protein expression of Beclin-1, Sirt1, FOXO3, PINK1, Parkin and the LC3BⅡ/Ⅰratio in hippocampus were increased (P<0.001, P<0.01), while the protein expression of P62 was decreased (P<0.001).
    CONCLUSION: EA at "Shenting" (GV24) and "Baihui" (GV20) can relieve the symptoms of neurological deficits and improve the learning-memory ability in MCAO/R rats, its mechanism may relate to the modulation of Sirt1/FOXO3/PINK1/Parkin pathway and the enhancement of mitochondrial autophagy.
    Keywords:  Point GV20 (Baihui); Point GV24 (Shenting); cerebral ischemia reperfusion injury; electroacupuncture; learning-memory impairment; mitochondrial autophagy
    DOI:  https://doi.org/10.13703/j.0255-2930.20231012-k0005
  27. Free Radic Biol Med. 2025 Feb 11. pii: S0891-5849(25)00082-6. [Epub ahead of print]
      Tumor hypoxia determines tumor growth, metastasis, drug resistance, and tumor heterogeneity through multiple mechanisms, largely dependent on the extent of hypoxia, further modulated by re-oxygenation events. In order to track the cell fates under hypoxia and re-oxygenation, we have developed a sensor cell for real-time tracking of apoptotic, necrotic, and surviving mitophagy cells under hypoxia and re-oxygenation. The study using this sensor revealed a cell death switch from apoptosis to necrosis by hypoxia-exposed cells under re-oxygenation, where mitophagy plays a key role in acquiring temporally evolving functional phenotypes, including metabolic heterogeneity and mitochondrial redox heterogeneity. RNA transcriptomics also revealed a temporally evolving genomic landscape supporting the complex transcriptional plasticity of cells as a non-genetic adaptive event. Interestingly, cells regained from these distinct stages retained their metastatic potential despite slow growth in animal models. Overall, the study demonstrated that cells acquire distinct functions by tumor hypoxia and re-oxygenation, secondarily acquiring transient functional traits and metabolic heterogeneity governed by cell inherent mitochondrial dynamics. Such cell autonomous temporal alterations in cell states governed by organelle integrity with distinct cell proliferation and apoptosis-necrosis switch may be advantageous for the growing tumor to evolve under complex microenvironmental stress, further contributing to tumorigenesis.
    Keywords:  Apoptosis; Hypoxia; Metabolic Heterogeneity; Mitophagy; Necrosis; Redox
    DOI:  https://doi.org/10.1016/j.freeradbiomed.2025.02.007
  28. J Ethnopharmacol. 2025 Feb 11. pii: S0378-8741(25)00158-8. [Epub ahead of print] 119475
       ETHNOPHARMACOLOGICAL RELEVANCE: Microangiopathy represents a critical pathological characteristic of lupus nephritis (LN), with steroid resistance (SR) frequently observed among patients. Panax notoginseng saponins (PNS) has demonstrated potential in mitigating P-glycoprotein (P-gp)-mediated SR and attenuating inflammatory damage in glomerular endothelial cells (GECs) through exosomal pathways, although the precise mechanisms underlying these effects have yet to be fully elucidated.
    AIM OF THE STUDY: This research examines the impact of PNS on microangiopathy in steroid-resistant lupus nephritis (SR LN) and explores its involvement in the mitochondrial autophagy-NLRP3 inflammasome pathway mediated by exosomes.
    MATERIALS AND METHODS: Steroid-resistant models were developed using methylprednisolone (MPS) in murine peritoneal macrophages (Mø). Exosomes were characterized, and biochemical markers of lupus nephritis (LN) were evaluated. Renal pathological alterations were analyzed using hematoxylin and eosin (H&E), Masson's trichrome, and periodic acid-Schiff (PAS) staining. Mitochondrial autophagy was assessed through transmission electron microscopy. Apoptosis, mitochondrial membrane potential (MMP), P-glycoprotein (P-gp), Rhodamine-123 (Rh-123), and reactive oxygen species (ROS) were measured using flow cytometry. The expression of MDR1, PINK1/Parkin, and NLRP3 at the protein and gene levels was determined via immunoblotting and real-time PCR.
    RESULTS: Exosomes derived from SR Mø increased the expression of MDR1 and P-glycoprotein (P-gp) in GECs, reduced Rhodamine 123 (Rh-123) accumulation, inhibited mitochondrial autophagy, and activated the NLRP3 inflammasome, thereby exacerbating renal inflammation and tissue damage. Conversely, PNS were found to lower the levels of pro-inflammatory cytokines, including interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-α), interleukin-18 (IL-18), and interleukin-1 beta (IL-1β). PNS also decreased P-gp expression and increased Rh-123 accumulation. Furthermore, PNS downregulated cleaved-Caspase1 while upregulating PINK1, Parkin, Beclin-1, and the ratio of LC3II/LC3I. This dual effect of PNS reversed SR and improved renal inflammation damage.
    CONCLUSION: PNS demonstrated an improvement in renal function and a reduction in histopathological damage, suggesting its potential therapeutic applications for LN.
    Keywords:  Panax notoginseng saponins; exosomes; inflammasome; lupus nephritis; microangiopathy; mitochondrial autophagy; steroid resistance
    DOI:  https://doi.org/10.1016/j.jep.2025.119475
  29. Apoptosis. 2025 Feb 13.
      Globally, esophageal cancer stands as a prominent contributor to cancer-related fatalities, distinguished by its poor prognosis. Mitophagy has a significant impact on the process of cancer progression. This study investigated the prognostic significance of mitophagy-related genes (MRGs) in esophageal carcinoma (ESCA) to elucidate molecular subtypes. By analyzing RNA-seq data from The Cancer Genome Atlas (TCGA), 6451 differentially expressed genes (DEGs) were identified. Cox regression analysis narrowed this list to 14 MRGs with potential prognostic implications. ESCA patients were classified into two distinct subtypes (C1 and C2) based on these genes. Furthermore, leveraging the differentially expressed genes between Cluster 1 and Cluster 2, ESCA patients were classified into two novel subtypes (CA and CB). Importantly, patients in C2 and CA subtypes exhibited inferior prognosis compared to those in C1 and CB (p < 0.05). Functional enrichments and immune microenvironments varied significantly among these subtypes, with C1 and CB demonstrating higher immune checkpoint expression levels. Employing machine learning algorithms like LASSO regression, Random Forest and XGBoost, alongside multivariate COX regression analysis, two core genes: HSPD1 and MAP1LC3B were identified. A prognostic model based on these genes was developed and validated in two external cohorts. Additionally, single-cell sequencing analysis provided novel insights into esophageal cancer microenvironment heterogeneity. Through Coremine database screening, Icaritin emerged as a potential therapeutic candidate to potentially improve esophageal cancer prognosis. Molecular docking results indicated favorable binding efficacies of Icaritin with HSPD1 and MAP1LC3B, contributing to the understanding of the underlying molecular mechanisms of esophageal cancer and offering therapeutic avenues.
    Keywords:  Esophageal cancer; Machine learning; Mitophagy; Prognosis; Single-cell sequencing; Subtype
    DOI:  https://doi.org/10.1007/s10495-024-02061-1
  30. Eur Heart J. 2025 Feb 11. pii: ehaf032. [Epub ahead of print]
       BACKGROUND AND AIMS: Myocardial infarction (MI) is an ischaemic cardiovascular disease associated with increased morbidity and mortality. Previous studies have suggested that serine carboxypeptidase 1 (Scpep1) is involved in vascular diseases; however, its role in cardiac diseases remains unclear. This study aims to explore the role of Scpep1 in regulating cardiac homeostasis during MI.
    METHODS: The impact of Scpep1 deficiency or cardiac-specific knock-down and Scpep1 overexpression on heart function was evaluated in mice with MI. Its downstream functional mediators of Scpep1 were elucidated using proteomic analysis and confirmed by employing loss- and gain-of-function strategies.
    RESULTS: Circulating and cardiac Scpep1 levels were up-regulated in mice with MI. Genetic ablation or cardiac-specific knock-down of Scpep1 alleviated MI-induced cardiac dysfunction and damage in mice. In contrast, cardiac-specific Scpep1 overexpression aggravated these adverse effects. Mechanistically, Scpep1 exacerbated MI-induced cardiac dysfunction and damage by impaired mitochondrial bioenergetics via binding to Pex3 to promote its degradation, ultimately contributing to mitochondrial fission and apoptosis. Moreover, the expressional profiles of Scpep1 in plasma samples and heart tissues of patients with MI or ischaemic cardiomyopathy were in line with those observed in the mouse models. In addition, pharmaceutical inhibition of Scpep1 notably improved MI-induced cardiac dysfunction and damage by improving mitochondrial fragmentation and bioenergetics post-MI.
    CONCLUSIONS: Scpep1 deficiency mitigates MI by improving Pex3-mediated mitochondrial fission and subsequent cardiomyocyte apoptosis. Scpep1 constitutes a potential therapeutic target for attenuating MI.
    Keywords:  Cardiomyocyte apoptosis; Mitochondrial bioenergetics; Myocardial infarction; Pex3; Scpep1
    DOI:  https://doi.org/10.1093/eurheartj/ehaf032
  31. Eur J Pharmacol. 2025 Feb 08. pii: S0014-2999(25)00073-1. [Epub ahead of print]992 177320
       BACKGROUND AND AIMS: Chronic consumption of large amounts of alcohol can lead to hepatic lipid accumulation and mitochondrial oxidative stress, resulting in alcoholic liver disease (ALD). Canagliflozin (Cana), an oral antidiabetic drug, regulates blood glucose by inhibiting sodium-glucose cotransporter-2 in renal tubulars, which also improves lipid metabolism and alleviates oxidative stress in hepatocyte. This study aims to determine the therapeutic effects of Cana on alcoholic liver injury and to explore the mechanistic pathways involved.
    METHODS: C57BL/6J male mice at 8 weeks were used to construct a model of alcoholic fatty liver disease using the chronic-plus-binge alcohol feeding model. Primary hepatocytes and AML12 cell lines were used as in vitro models. The effects and mechanisms of Cana on alcoholic liver injury were investigated by using immunofluorescence, ELISA, H&E and Oil Red O staining, RT-PCR, and western blotting analysis.
    RESULTS: Cana treatment reduced hepatic lipid accumulation, decreased glutathione and TNF-α levels, alleviated oxidative stress and inflammation. Mechanistic studies revealed that Cana reduced FAS expression in the liver, decreasing hepatic fatty acid synthesis, and increased PPARα expression, promoting fatty acid oxidation. Additionally, Cana increased mitochondrial content and promoted mitophagy. These effects were mediated by the SIRT1-AMPK-mTORC1 signaling pathway.
    CONCLUSIONS: Cana activates the SIRT1-AMPK-mTORC1 signaling pathway, inhibiting alcohol-induced fatty acid synthesis, promoting fatty acid degradation, thereby alleviating alcoholic liver injury.
    Keywords:  Alcoholic liver disease; Fatty acid oxidation; Lipid synthesis; Mitochondrial biogenesis; Sodium-glucose cotransporter 2 inhibitor; mTORC1 signaling pathway
    DOI:  https://doi.org/10.1016/j.ejphar.2025.177320
  32. Mol Med Rep. 2025 Apr;pii: 93. [Epub ahead of print]31(4):
      Mitophagy plays significant roles in chronic obstructive pulmonary disease (COPD). The present study aimed to screen and validate mitophagy‑related genes in COPD by using bioinformatic analysis and experimental validation. The original data were downloaded from Gene Expression Omnibus datasets and 29 mitophagy‑related genes sets were acquired from the Molecular Signatures Database. The differentially expressed mitophagy‑related genes (DEMRGs) were screened using the Wilcoxon test. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes enrichment analysis were conducted for the identification of DEMRGs. In addition, clustering analysis was used to assess the differential expression characteristics of DEMRGs in patients with COPD. Least absolute shrinkage and selection operator (LASSO) regression analysis was performed to identify signature genes with COPD diagnostic significance; these genes were validated using the test dataset. In addition, the degree of infiltration of 28 immune cells in COPD and control samples was assessed. Finally, cigarette smoke extract (CSE)‑treated bronchial epithelial cells were employed to verify the role of signature genes in regulating mitophagy in vitro using molecular biology approaches. A total of 14 DEMRGs were identified, which were mainly involved in mitophagy‑related processes and pathways. Clustering analysis indicated the expression levels of 14 DEMRGs except for microtubule‑associated protein 1 light chain‑3β, which was significantly different. Moreover, combination with LASSO, receiver operating characteristic curve and the validation dataset resulted in the identification of the mitochondrial transcription termination factor 3 (MTERF3). The infiltrating abundance of the majority of the immune cells was higher in COPD samples than that noted in the control samples; MTERF3 demonstrated the optimal correlation with macrophages, myeloid‑derived suppressor cells, regulatory T cells and activated cluster of differentiation 8 T cells. Further analysis revealed that MTERF3 expression was increased in CSE‑treated 16HBE cells and knockdown of MTERF3 expression promoted mitophagy. These findings provide novel insights into the role of mitophagy in COPD and identify novel targets for COPD diagnosis and treatment.
    Keywords:  MTERF3; bioinformatics analysis; chronic obstructive pulmonary disease; mitophagy; mitophagy‑related genes
    DOI:  https://doi.org/10.3892/mmr.2025.13458
  33. Arch Med Res. 2025 Feb 07. pii: S0188-4409(25)00014-1. [Epub ahead of print]56(4): 103194
       BACKGROUND: Due to their unique properties, extracellular vesicles (EVs) are promising nanocarriers for exogenous drug delivery.
    AIM: We prepared a drug delivery system based on large EVs (LEVs) containing paclitaxel (PTX) (LEVs-PTX) to investigate anticancer effects on lung cancer cells with a focus on autophagy.
    METHODS: LEVs-PTX were isolated from lung cancer cells by ultracentrifugation and characterized using different techniques. Rhodamine B dye (Rh B) was used to label LEVs-PTX for cell tracking. MTT assay was performed to investigate the cellular toxicity of PTX and LEVs-PTX for 24 h and 48 h. The uptake of LEVs-PTX was monitored by immunofluorescence microscopy in breast and lung cancer cells. A colorimetric assay was performed to evaluate apoptosis, while Western blotting assays were used to investigate autophagy proteins. Real-time PCR was used to measure mitophagy genes.
    RESULTS: Characterization techniques showed that LEVs were isolated and loaded with PTX. Rh B labeled LEVs, which was confirmed by a fluorescence spectrophotometer. Immunofluorescence microscopy showed that the lung and breast cancer cells had captured LEVs. Cell viability was decreased in LEVs-PTX cells which coincided with an increase in caspase-3 activity in LEVs-PTX cells. The Beclin-1 protein level and LC3 II/I ratio decreased, while the P62 protein level was increased in LEVs-PTX cells. The mitophagy genes such as Pink-1 and Parkin were upregulated in LEVs-PTX cells.
    CONCLUSION: The data show that LEVs-PTX induced apoptosis, which inhibited the autophagy pathway and increased mitophagy markers, suggesting damage to cell organelles through intracellular delivery of PTX.
    Keywords:  A549 cells; Extracellular vesicles; Lung cancer; Paclitaxel; Rhodamine B
    DOI:  https://doi.org/10.1016/j.arcmed.2025.103194
  34. Mol Neurobiol. 2025 Feb 14.
      The nucleotide-binding oligomerization domain-like receptor pyrin domain-containing 3 (NLRP3) inflammasome, discovered 20 years ago, is crucial in controlling innate immune reactions in Alzheimer's disease (AD). By initiating the release of inflammatory molecules (including caspases, IL-1β, and IL-18), the excessively activated inflammasome complex in microglia leads to chronic inflammation and neuronal death, resulting in the progression of cognitive deficiencies. Even though the involvement of NLRP3 has been implicated in neuroinflammation and widely explored in several studies, there are plenty of controversies regarding its precise roles and activation mechanisms in AD. Another prominent feature of AD is impairment in microglial autophagy, which can be either the cause or the consequence of NLRP3 activation and contributes to the aggregation of misfolded proteins and aberrant chronic inflammatory state seen in the disease course. Studies also demonstrate that intracellular buildup of dysfunctional and damaged mitochondria due to defective mitophagy enhances inflammasome activation, further suggesting that restoration of impaired autophagy and mitophagy can effectively suppress it, thereby reducing inflammation and protecting microglia and neurons. This review is primarily focused on the role of NLRP3 inflammasome in the etiopathology of AD, its interactions with microglial autophagy/mitophagy, and the latest developments in NLRP3 inflammasome-targeted therapeutic interventions being implicated for AD treatment.
    Keywords:  Alzheimer’s disease; Autophagy; Microglia; Mitophagy; NLRP3 inflammasome
    DOI:  https://doi.org/10.1007/s12035-025-04758-z
  35. Mater Today Bio. 2025 Apr;31 101500
      In an effort to address the detrimental effects of myocardial ischemia/reperfusion injury (MI/RI), this study introduces a novel therapeutic strategy that involves a microneedle (MN) patch loaded with exosomes derived from human umbilical cord mesenchymal stem cells (hUCMSCs) and carrying small interfering RNA (siRNA) targeting early growth response-1 (EGR1). By delivering hUCMSCs-derived exosomes containing EGR1 siRNA (hUCMSCs-Exosi-EGR1), the MN patch demonstrated promising cardioprotective effects in both in vitro and in vivo models of MI/RI. The results highlighted the efficacy of Exosi-EGR1 in enhancing cardiomyocyte viability, attenuating oxidative stress levels, and fostering mitophagy regulation. Moreover, the Exosi-EGR1 MN patch exhibited excellent mechanical properties and sustained drug release characteristics when embedded in a Gelatin methacryloyl (GelMA) matrix. Noteworthy outcomes from in vivo experiments included significant improvements in cardiac function, reduced cardiac fibrosis, and decreased apoptosis rates in MI/RI mice, emphasizing the potential therapeutic value of the Exosi-EGR1 MN patch in mitigating MI/RI through modulation of oxidative stress and mitophagy mechanisms.
    Keywords:  EGR1 siRNA; Exosomes; Microneedle patch; Myocardial ischemia/reperfusion injury; Oxidative stress
    DOI:  https://doi.org/10.1016/j.mtbio.2025.101500
  36. Reprod Sci. 2025 Feb 13.
       OBJECTIVE: To investigate the mechanism in the progression of decreased ovarian reserve (DOR).
    METHODS: Three-month-old female SD rats were employed and randomly divided into the model group and the normal group. The model group was intraperitoneally injected with 4-vinylcyclohexene diepoxide (VCD). Thereafter, blood sample from the abdominal aorta was taken, and rats were sacrificed, and ovarian tissues were obtained by laparotomy.
    RESULTS: HE staining results revealed that the model group exhibited significantly reduced ovarian volume, increased follicular atresia, and decreased quantities of growing follicles and corpus luteum, thereby indicating degraded reserve function of ovarian. TEM images revealed that prominent autophagic vacuoles could be observed in the model group, accompanied by the mitochondria shrinkage and generation of the autophagosome. The expression of Pink1, Parkin, BNIP3L and LC3II genes in ovaries of the model group was significantly higher than those of the normal group (P < 0.05). In addition, the protein expression of Pink1, Parkin, BNIP3L and LC3II in ovaries of the model group were higher than those of the normal group (P < 0.05). The expression of Fe2+ and GSH in oocytes of the model group was higher than those of the normal group (P < 0.05). The expression of FTH1 and GPX4 in oocytes of the model group was significantly higher than those of the normal group (P < 0.05).
    CONCLUSIONS: Mitochondrial autophagy and ferroptosis may participate in the progression of decreased ovarian reserve (DOR).
    Keywords:  Decreased ovarian reserve; Ferroptosis; Follicle; Mechanistic study; Mitochondrial autophagy
    DOI:  https://doi.org/10.1007/s43032-025-01811-z
  37. Curr Vasc Pharmacol. 2025 Feb 06.
       BACKGROUND: Cardiac microvessels are significantly reduced in diabetic patients, which is accompanied by a significant increase in the incidence of diabetic cardiac complications and increased mortality. This study aimed to investigate the role and possible mechanism of sirtuin 1 (Sirt1) in microvascular endothelial cell injury in diabetic hearts.
    METHODS: Type 2 diabetes mouse models and cardiac microvascular endothelial cell (CMEC) cell models were established. Cardiac microvessel density (MVD) was detected using Platelet- Endothelial Cell Adhesion Molecule 1 (CD31) immunohistochemistry. Mitochondrial reactive oxygen species (ROS) was detected with MitoSOX and morphology was observed with mitochondrial staining. CMECs angiogenesis was evaluated via scratch and angiogenesis assays. We measured cell viability with a Cell Counting Kit (CCK)-8 assay and cell injury with lactate dehydrogenase (LDH) release assay. We assessed apoptosis using TUNEL staining, Caspase-3 activity, and Western blot.
    RESULTS: The decrease in Sirt1 protein expression was accompanied by a decrease in cardiac microvessel density in type 2 diabetic mice. After 48 h of treating the CMECs with high-glucose and palmitic acid, it was discovered that the expression of Sirt1 and dynamin-related protein 1 (Drp1) Ser637 phosphorylated protein decreased, while the expression of Cleaved Caspase-3 protein increased. Also, the angiogenesis ability of endothelial cells was decreased, while mitochondrial ROS and mitochondrial division were increased, which culminated in aggravated endothelial cell injury and increased endothelial cell apoptosis. Increased Sirt1 protein expression and function at the gene and drug levels alleviated excessive mitochondrial division, reduced apoptosis, and improved the function of CMECs by increasing the phosphorylation of Drp1 Ser637.
    CONCLUSION: Under diabetic conditions, the Sirt1/Drp1 pathway reduces injury to CMECs by inhibiting excessive mitochondrial division.
    Keywords:  Diabetes mellitus; Drp1; Sirt1; cardiac microvascular endothelial cells; mitochondrial preface.
    DOI:  https://doi.org/10.2174/0115701611370387250122050842
  38. Arch Dermatol Res. 2025 Feb 08. 317(1): 368
      Previous research has identified a variety of factors that contribute to the development and maintenance of wounds. Concurrently, electroacupuncture has been demonstrated to facilitate wound healing. However, the effects of transcutaneous electrical acustimulation (TEA) on wound healing, as well as its relationship with key factors such as Wnt3a, TGF-β, Akt, c-Myc, VEGF-A, SP1, nitric oxide (NO), and mitochondrial function, remain largely unexplored. We hypothesize that TEA will activate the signaling factors and enhance mitochondrial functions to promote the repair of skin wounds in mice. An in vivo experimental study was conducted utilizing mouse models with skin wounds. The study comprised three groups: a TEA treatment with wound group, a skin wound model group, and a control group. Wound areas were measured by calculating the product of the length and width of each wound using calipers. Single-cell suspensions were prepared by excising the wound and the immediately surrounding tissue. These suspensions were stained with Trypan blue to assess cell viability, with specific probes to measure the rate of reactive oxygen species (ROS) positivity, and with reagents to quantify NO content. Western blotting (WB) was employed to evaluate protein levels associated with tissue changes, while quantitative polymerase chain reaction (qPCR) was used to assess RNA expression levels. Immunofluorescence staining was performed to visualize protein content and other relevant cellular structures within tissue sections. TEA exhibited anti-inflammatory properties and promoted wound healing in mice. Western blot analysis revealed that TEA enhanced the expression of proteins associated with Wnt3a, TGF-β, Akt, c-Myc, VEGF-A, and SP1 during the wound healing process. Immunofluorescence staining of tissue sections indicated that TEA upregulated the expression of COL1A1, MFN1, GRP75, GRP78, GRP75/ROS, GRP78/ROS, ISCU, and UCP1 while downregulating FIS1. Additionally, qPCR results demonstrated that TEA promoted the expression of IL-10 and miRNA205-5p while inhibiting MMP9 levels. TEA modulates various signaling molecules, influences chaperone proteins related to stress recovery responses, along with mitochondrial dynamics and metabolism.
    Keywords:  Mitochondrial dynamics; Repair; Signaling molecules; Transcutaneous electrical acustimulation; Wound healing
    DOI:  https://doi.org/10.1007/s00403-024-03754-y
  39. Curr Vasc Pharmacol. 2025 Feb 10.
      Abdominal Aortic Aneurysm (AAA) is a life-threatening vascular disease. Despite advancements in understanding the pathogenesis of AAA, significant knowledge gaps persist. Recent evidence increasingly implicates mitochondrial dysfunction as a contributing factor that exacerbates AAA, inducing further expansion of aneurysm, rupture, and subsequent death. This review summarizes the latest research findings and theories associated with AAA pathogenesis, with a particular focus on mitochondrial dysfunction in AAA, including mitochondrial quality control, mitochondrial membrane potential, mitochondrial morphology, oxidation and antioxidation, normal functioning of the respiratory chain, mitochondrial mutations, and the regulation of other mitochondrial signaling pathways. Moreover, we highlight potential medical interventions based on regulating mitochondrial function for AAA treatment.
    Keywords:  Abdominal aortic aneurysm; biogenesis; fission and fusion; inflammation.; mitochondria dysfunction; oxidative stress
    DOI:  https://doi.org/10.2174/0115701611312293241220101556
  40. Toxicol Lett. 2025 Feb 08. pii: S0378-4274(25)00017-7. [Epub ahead of print]405 41-50
      Bisphenols (BPs) are a group of environmental pollutants mainly represented by bisphenol S (BPS) and F (BPF). In ovaries, BPs can accumulate in follicular fluid (FF), changing the follicular microenvironment and simultaneously affecting ovarian granulosa cells (GCs) function. In the present study, we determined the effects of BPS and BPF on oxidative stress and mitochondrial function in human ovarian GCs. Single, short-term treatment with BPs at doses reflecting their concentrations in FF (10 nM) did not affect reactive oxygen species (ROS) levels but induced mitochondrial membrane depolarization. BPF-induced mitophagy decreased the number of active mitochondria and consequently reduced the ATP production rate. The observed changes did not translate into lowered viability of GCs, but long-term treatment with BPF influenced the intrinsic apoptosis pathway by increasing caspase 9 activity without affecting apoptosis. GCs are crucial for ovarian function as they produce primary steroid hormones and regulate oocyte maturation and follicle growth. Mitochondrial dysfunction caused by BPs, manifesting as reduced ATP production in GCs, can directly cause ovarian disorders such as infertility. Therefore, this study highlights the significance of investigating the effects of BPs on reproductive health.
    Keywords:  Apoptosis; Bisphenol S and F; Human ovarian granulosa cells; IGF-1; Mitochondrial function; Mitophagy
    DOI:  https://doi.org/10.1016/j.toxlet.2025.02.002
  41. Cell Biochem Biophys. 2025 Feb 12.
      Hepatocellular carcinoma (HCC) represents a significant global health challenge, characterized by a high incidence rate. Mitochondria have emerged as an important therapeutic target for HCC. Donafenib, a multi-receptor tyrosine kinase inhibitor, has been approved for the treatment of advanced HCC. However, the underlying mechanisms remain to be elucidated. In this study, we aim to investigate the effects of Donafenib on mitochondrial function in HCC cells. Firstly, we show that Donafenib induces mitochondrial oxidative stress in SNU-449 liver cancer cells by increasing mitochondrial ROS while reducing glutathione peroxidase (GPx) activity and the expression of Mn-SOD. We also demonstrate that Donafenib decreases mitochondrial membrane potential (MMP) and induces the opening of the mitochondrial permeability transition pore (mPTP). Furthermore, Donafenib reduces mitochondrial respiratory rate, COX IV activity, and ATP production. Notably, Donafenib induces mitochondrial fragmentation and reduces mitochondrial length by increasing the expression of DRP1, without affecting Mfn1 or Mfn2. Silencing of DRP1 protects against mitochondrial dysfunction induced by Donafenib, indicating that DRP1 plays a key role in mediating Donafenib's effects on mitochondrial function in HCC cells.
    Keywords:  DRP-1; Donafenib; Hepatocellular carcinoma; Mitochondrial dysfunction; Mitochondrial fission
    DOI:  https://doi.org/10.1007/s12013-024-01648-4
  42. Discov Oncol. 2025 Feb 09. 16(1): 140
       BACKGROUND: This study presents a new prognostic model using mitophagy-related genes (MRGs) in glioma, a type of brain tumor, developed through bioinformatics. The model seeks to improve the understanding of glioma prognosis by focusing on mitophagy, a cellular process that eliminates damaged mitochondria and influences tumor behavior and patient outcomes.
    METHODS: The expression profile and clinical information of patients were downloaded from TCGA (The Cancer Genome Atlas) and GEO (Gene Expression Omnibus). By analyzing the correlation between the 14 MRGs and glioma prognosis, we established a novel prognostic model in the TCGA training cohort and validated it in the GSE16011 dataset.
    RESULTS: Using univariate Cox regression, we identified 26 MRGs that were significantly enriched in various mitophagy-related pathways. After filtering variables using least absolute shrinkage and selection operator (Lasso) regression analysis, 14 MRGs were introduced to construct the predictive model. The survival analysis showed overall survival of patients with the high-risk score was considerably poorer than that with the low-risk score in both the training and validating cohorts (p < 0.01). The risk score was found to be an independent prognostic factor for glioma in both univariate and multivariate Cox regression analyses. Interestingly, Geneset enrichment analysis (GSEA) analysis revealed that multiple signaling pathways related to neurotransmission were significantly enriched in the high-risk group. Additionally, a hub miRNA-mRNA network was established, which disclosed the quantity and classification of miRNAs capable of interacting with 14 MRGs. Finally, our analysis revealed a notable association between 14 MRGs and immune functionality in gliomas.
    CONCLUSION: We developed a robust and accurate prognostic model with 14 MRGs. Our findings might provide a reference for the clinical prognosis and management of glioma.
    Keywords:  Bioinformatics; Glioma; Mitophagy; Prognostic model; Risk score
    DOI:  https://doi.org/10.1007/s12672-025-01916-6
  43. Int J Mol Sci. 2025 Jan 30. pii: 1224. [Epub ahead of print]26(3):
      In a recent clinical trial, beetroot juice supplementation for 14 days yielded positive effects on systemic inflammation in adults with long COVID. Here, we explored the relationship between circulating markers of mitochondrial quality and inflammation in adults with long COVID as well as the impact of beetroot administration on those markers. We conducted secondary analyses of a placebo-controlled randomized clinical trial testing beetroot juice supplementation as a remedy against long COVID. Analyses were conducted in 25 participants, 10 assigned to placebo (mean age: 40.2 ± 11.5 years, 60% women) and 15 allocated to beetroot juice (mean age: 38.3 ± 7.7 years, 53.3% women). Extracellular vesicles were purified from serum by ultracentrifugation and assayed for components of the electron transport chain and mitochondrial DNA (mtDNA) by Western blot and droplet digital polymerase chain reaction (ddPCR), respectively. Inflammatory markers and circulating cell-free mtDNA were quantified in serum through a multiplex immunoassay and ddPCR, respectively. Beetroot juice administration for 14 days decreased serum levels of interleukin (IL)-1β, IL-8, and tumor necrosis factor alpha, with no effects on circulating markers of mitochondrial quality control. Significant negative associations were observed between vesicular markers of mitochondrial quality control and the performance on the 6 min walk test and flow-mediated dilation irrespective of group allocation. These findings suggest that an amelioration of mitochondrial quality, possibly mediated by mitochondria-derived vesicle recycling, may be among the mechanisms supporting improvements in physical performance and endothelial function during the resolution of long COVID.
    Keywords:  cell quality; cytokine; extracellular vesicles; functional food; inflammation; mitochondrial DNA; muscle; physical performance
    DOI:  https://doi.org/10.3390/ijms26031224
  44. Free Radic Biol Med. 2025 Feb 06. pii: S0891-5849(25)00078-4. [Epub ahead of print]230 66-78
      Protein S-nitrosylation (SNO), a redox-based posttranslational modification of cysteine thiols, plays a crucial role in various signaling pathways. Peroxiredoxin 2 (PRDX2) is one of the most potent ROS scavenging proteins, providing protection against oxidative stress damage, with its function regulated by SNO. However, the precise role of SNO-PRDX2 in hyperuricemic nephropathy remains poorly understood. In this study, we identified PRDX2 as a highly S-nitrosylated target in hyperuricemic nephropathy using a biotin switch assay. The elevation of SNO-PRDX2 was observed in kidneys of hyperuricemic mice as well as in uric acid (UA)-treated human renal tubular epithelial (HK-2) cells. S-nitrosoglutathione (GSNO), an endogenous nitric oxide carrier, induced SNO modification of PRDX2, promoting mitochondrial dysfunction, oxidative stress, and cell apoptosis in HK-2 cells. Transfection with a plasmid containing a mutated cysteine 172 (Cys172) of PRDX2 yielded a decrease in SNO-PRDX2 levels in both hyperuricemic mice and UA-cultured HK-2 cells. Furthermore, administration of adeno-associated viruses carrying the Cys172-mutated PRDX2 significantly ameliorated renal interstitial fibrosis and reduced mitochondrial dysfunction, oxidative stress, and cell apoptosis in HUA-treated mice. In conclusion, our findings indicate that SNO modification of PRDX2 at Cys172 mediates HUA-induced kidney interstitial fibrosis, suggesting that SNO-PRDX2 may serve as a potential therapeutic target for HUA-induced renal injury.
    Keywords:  Hyperuricemia; Mitochondrial homeostasis; PRDX2; Renal injury; S-nitrosylation
    DOI:  https://doi.org/10.1016/j.freeradbiomed.2025.02.003
  45. Nutrients. 2025 Feb 03. pii: 568. [Epub ahead of print]17(3):
      Ginseng and L-carnitine are natural compounds often used as dietary supplements to enhance athletic performance. However, their combined effects on exercise endurance remain unclear. Objectives: This study aimed to investigate the effects of ginseng extract and L-carnitine supplementation on exercise endurance in a rat model. Methods: Male Wistar rats were divided into 10 groups (n = 5 per group): control, ginseng extract (250 and 500 mg/kg/day), L-carnitine (250 and 500 mg/kg/day), and combined treatment. Half of the groups underwent a 16-day exercise training program of swimming without loading. Exercise endurance was assessed using a tail-suspended forced swimming test. Relative organ weight, glycogen content, blood biochemistry, and gene expression were analyzed. Results: Both ginseng extract and L-carnitine supplementation significantly increased exercise endurance, particularly in the exercise group. Ginseng extract and L-carnitine also increased liver glycogen content and upregulated the expression of AMPKα1 and PGC-1α genes in the liver and muscle. In addition, both supplements reduced oxidative stress by decreasing MDA levels and increasing SOD activity. Conclusions: Ginseng extract and L-carnitine supplementation may enhance exercise endurance by improving energy metabolism, reducing oxidative stress, and upregulating key genes involved in mitochondrial biogenesis.
    Keywords:  AMPK; L-carnitine; PGC-1α; exercise endurance; ginseng extract; glycogen; liver; mitochondrial biogenesis; muscle; oxidative stress
    DOI:  https://doi.org/10.3390/nu17030568
  46. Int J Mol Sci. 2025 Jan 27. pii: 1100. [Epub ahead of print]26(3):
      The coordination of cytoskeletal proteins shapes cell architectures and functions. Age-related changes in cellular mechanical properties have been linked to decreased cellular and tissue dysfunction. Studies have also found a relationship between mitochondrial function and the cytoskeleton. Cytoskeleton inhibitors impact mitochondrial quality and function, including motility and morphology, membrane potential, and respiration. The regulatory properties of the cytoskeleton on mitochondrial functions are involved in the pathogenesis of several diseases. Disassembly of the axon's cytoskeleton and the release of neurofilament fragments have been documented during neurodegeneration. However, these changes can also be related to mitochondrial impairments, spanning from reduced mitochondrial quality to altered bioenergetics. Herein, we discuss recent research highlighting some of the pathophysiological roles of cytoskeleton disassembly in aging, neurodegeneration, and neuromuscular diseases, with a focus on studies that explored the relationship between intermediate filaments and mitochondrial signaling as relevant contributors to cellular health and disease.
    Keywords:  axonal transport; cell architecture; cell quality; cytoskeleton; mitochondrial quality; muscle aging; neurodegeneration; neurofilaments; sarcomere; vimentin
    DOI:  https://doi.org/10.3390/ijms26031100