bims-mibica Biomed News
on Mitochondrial bioenergetics in cancer
Issue of 2025–07–13
twenty-two papers selected by
Kelsey Fisher-Wellman, Wake Forest University



  1. Transl Oncol. 2025 Jul 08. pii: S1936-5233(25)00198-6. [Epub ahead of print]59 102467
      We have previously identified sitravatinib as a potent inhibitor of FLT3, capable of overcoming resistance to gilteritinib in the treatment of acute myeloid leukemia (AML). The combination of venetoclax and FLT3 inhibitors gilteritinib and quizartinib has shown promising results in reducing leukemia burden and improving survival in pre-clinical studies and clinical trials of AML with FLT3 mutation. In this study, we aimed to investigate the therapeutic effect of treating AML with sitravatinib combined with venetoclax. Our findings indicated that the combination of sitravatinib and venetoclax significantly decreased cell viability and increased cell apoptosis in AML cell lines harboring FLT3 mutation, more so than either treatment alone. These two agents exerted strong synergistic effects in FLT3-ITD AML cell lines and patient bone marrow cells in vitro. The activation of MAPK/ERK signaling are common causes that weaken the efficacy of FLT3 inhibitors, while the upregulation of anti-apoptotic proteins including BCL-xL and MCL-1 leads to venetoclax resistance. Our data demonstrated that sitravatinib plus venetoclax further suppressed the phosphorylation of AKT and ERK as well as downregulated MCL-1 and BCL-xL, which mechanically explain the synergistic effect. Finally, we tested the potential application of sitravatinib plus venetoclax in vivo using patient-derived xenografts, and found that the combined therapy was significantly more effective in inhibiting leukemia cell expansion, reducing infiltration in the spleen, and prolonging survival time compared to a single administration. Our study demonstrates the potential use of sitravatinib plus venetoclax as an alternative therapeutic strategy to treat AML patients with FLT3-ITD mutation.
    Keywords:  BCL-2 inhibitor; FLT3-ITD mutation; Sitravatinib; Synergistic effect; Venetoclax
    DOI:  https://doi.org/10.1016/j.tranon.2025.102467
  2. bioRxiv. 2025 Jul 02. pii: 2025.06.18.660357. [Epub ahead of print]
      Mitochondria contribute to compartmentalized metabolism in eukaryotic cells, supporting key enzymatic reactions for cell function and energy homeostasis. However, this compartmentalization necessitates regulated metabolite transport across mitochondrial membranes. Although many transport proteins have been identified, several mitochondrial amino acid transporters remain largely uncharacterized. Using CRISPR-Cas9-mediated candidate transporter knockouts coupled with assessment of metabolite transport via a mitochondrial swelling assay, we identify SFXN1, previously characterized for its role in mitochondrial serine transport, as a protein that mediates mitochondrial transport of a range of other polar neutral amino acids including proline, glycine, threonine, taurine, hypotaurine, β-alanine, and γ-aminobutyric acid (GABA). Furthermore, the SFXN1 paralogues SFXN2 and SFXN3 partially complement loss of SFXN1 to enable glycine transport, while SFXN2 and SFXN5 partially complement loss of SFXN1 to enable GABA transport. Altogether, these data suggest that sideroflexins facilitate the transport of polar neutral amino acids across the inner mitochondrial membrane.
    DOI:  https://doi.org/10.1101/2025.06.18.660357
  3. Cell Rep. 2025 Jul 03. pii: S2211-1247(25)00716-8. [Epub ahead of print]44(7): 115945
      Cancer cells exhibit metabolic reprogramming to sustain proliferation, creating metabolic vulnerabilities absent in normal cells. While prior studies identified specific metabolic dependencies, systematic insights remain limited. Here, we build a graph deep learning-based metabolic vulnerability prediction model, "DeepMeta," which can accurately predict the dependent metabolic genes for cancer samples based on transcriptome and metabolic network information. The performance of DeepMeta has been extensively validated with independent datasets. The metabolic vulnerability of "undruggable" cancer-driving alterations has been systematically explored using The Cancer Genome Atlas (TCGA) dataset. Notably, CTNNB1 T41A-activating mutations showed experimentally confirmed vulnerability to purine/pyrimidine metabolism inhibition. TCGA patients with the predicted pyrimidine metabolism dependency show a dramatically improved clinical response to chemotherapeutic drugs that block this pyrimidine metabolism pathway. This study systematically uncovers the metabolic dependency of cancer cells and provides metabolic targets for cancers driven by genetic alterations that are originally undruggable on their own.
    Keywords:  CP: Cancer; CP: Metabolism; CTNNB1; GAT; cancer metabolism; drug target; graph attention network; metabolic dependency; nucleotide metabolism; undruggable
    DOI:  https://doi.org/10.1016/j.celrep.2025.115945
  4. Leukemia. 2025 Jul 09.
      Acute myeloid leukemia with mutations in TP53 (TP53mut AML) is fatal with a median survival of 6 months. RNA sequencing on purified AML patient samples showed that TP53mut AML had higher expression of mevalonate pathway genes. Using novel, isogenic TP53mut AML cell lines and primary samples, we determined that TP53mut AML resistance to AML chemotherapy cytarabine (AraC) correlated with increased mevalonate pathway activity, a lower induction of reactive oxygen species (ROS), and a mitochondrial response with increased mitochondrial mass and oxidative phosphorylation. Pretreatment with the statin class of mevalonate pathway inhibitors reversed these effects and chemosensitized TP53mut AML. The geranylgeranyl pyrophosphate (GGPP) branch of the mevalonate pathway was required for TP53mut AML chemoresistance. In addition to its role in mitochondria biogenesis, we identified a novel function of GGPP in regulating glutathione for management of AraC-induced ROS. However, statins alone were inadequate to fully reverse chemoresistance in vivo and in a retrospective study of 364 TP53mut AML patients who received chemotherapy concurrently with a statin. Finally, we identified clinical settings and strategies to successfully target the mevalonate pathway, particularly to address the unmet need of TP53mut AML.
    DOI:  https://doi.org/10.1038/s41375-025-02668-6
  5. Sci Adv. 2025 Jul 11. 11(28): eadw1883
      Cell competition is a conserved fitness quality control that eliminates cells that are less fit than their neighbors. How winner cells induce the elimination of losers is poorly understood. We tackle this question by studying the onset of embryonic differentiation in mice, where cell competition eliminates 35% of embryonic cells. These loser cells have mitochondrial dysfunction, which we show causes amino acid deprivation and activation of the integrated stress response (ISR), a pathway essential for their survival. We demonstrate that l-proline is a key amino acid sensed by the ISR and that proline represses the ISR and drives their elimination. These results indicate that cell competition acts as a previously unidentified tissue-sparing mechanism, regulated by the availability of extracellular amino acids, that allows for the elimination of dysfunctional cells when amino acids are plentiful but ensures their survival in nutrient-poor environments.
    DOI:  https://doi.org/10.1126/sciadv.adw1883
  6. Haematologica. 2025 Jul 10.
      Metabolic dependencies are emerging as promising therapy targets in cancer, including acute myeloid leukemia (AML). Several metabolic vulnerabilities have been identified in AML cells, including a requirement for balanced sphingolipid metabolism to maintain survival and proliferation. Here we describe a novel function of the RAS superfamily small GTPase ARF6 in maintaining sphingolipid homeostasis in AML. Genetic depletion of ARF6 inhibited the proliferation of AML cell lines and reduced colony formation of primary AML CD34+ cells. Mechanistically, ARF6 promotes conversion of ceramide to sphingomyelin by enhancing sphingomyelin synthase (SGMS1/2) expression, thereby preventing accumulation of cytotoxic ceramide levels. Accordingly, higher expression of ARF6 and its effectors SGMS1/2 in AML patient cells correlates with shorter survival in two independent AML cohorts, with ARF6 exhibiting an adverse prognostic effect independent of European Leukemia Net genetic risk. Small molecule inhibitors of ARF6 suppressed colony formation by primary AML CD34+ cells, but not cord blood CD34+ cells and showed activity in xenograft models. The dependency of AML cells on ARF6 to regulate sphingolipid homeostasis may present a therapeutic opportunity.
    DOI:  https://doi.org/10.3324/haematol.2024.286228
  7. J Cell Sci. 2025 Jul 01. pii: jcs263701. [Epub ahead of print]138(13):
      Most mitochondrial proteins are imported through the actions of the presequence translocase of the inner membrane, the TIM23 complex, which requires energy in the form of the electrochemical potential of the inner membrane and ATP. Conversions of energy in mitochondria are disturbed in mitochondrial disorders that affect oxidative phosphorylation. Despite the widely accepted dependence of protein import into mitochondria on mitochondrial bioenergetics, effects of mitochondrial disorders on biogenesis of the mitochondrial proteome are poorly characterized. Here, we describe molecular tools that can be used to explore mitochondrial protein import in intact cells, the mitoRUSH assay, and a novel method based on labeling of nascent proteins with an amino acid analog and click chemistry. Using these orthogonal approaches, we discovered that defects in the electron transport chain and manipulating the expression of TIMM23, as well as the TIMM17A or TIMM17B paralogs, in human cells are associated with a decrease in protein import into mitochondria. We postulate that in the absence of a functional electron transfer chain, the mechanisms that support electrochemical potential of the inner membrane and ATP production are insufficient to sustain the import of proteins to mitochondria.
    Keywords:  Bioenergetics; Mitochondria; Mitochondrial diseases; Protein import; TIM23; Translocase; mitoRUSH
    DOI:  https://doi.org/10.1242/jcs.263701
  8. Cancer Lett. 2025 Jul 05. pii: S0304-3835(25)00471-9. [Epub ahead of print]630 217903
      Balancing high metabolic activity with redox homeostasis is crucial for cancer progression, particularly in high-grade serous ovarian cancer (HGSOC), which thrives in a lipid-rich environment abundance in free fatty acids, yet the key molecular regulators of this balance remain undefined. Through an in vivo genome-wide CRISPR/Cas9 knockout screen in an orthotopic ovarian cancer (OC) mouse model, we identify ACAD9 as a pivotal driver of OC progression, with its elevated expression correlating with poor patient prognosis. Multi-omics integration analysis and mechanism studies reveal ACAD9's dual role in maintaining OC metabolic homeostasis. ACAD9 preserves electron transport chain integrity and regulates linoleic acid (LA) metabolism to sustain energy production while mitigating oxidative stress. ACAD9 deficiency triggers mitochondrial respiratory collapse, inducing metabolic crisis marked by oxidative phosphorylation failure and reactive oxygen species (ROS) accumulation. Strikingly, under LA-enriched condition, ACAD9 loss redirects LA flux from β-oxidation toward membrane lipid biosynthesis, increasing polyunsaturated fatty acids incorporation. This membrane remodeling synergizes with ROS overload to create a "perfect storm" triggering ferroptosis. Our findings elucidate the dual metabolic guardianship of ACAD9 in OC, demonstrating its critical role in orchestrating mitochondrial respiration and lipid homeostasis to evade ferroptosis, which offer a potential target for the treatment of OC.
    Keywords:  ACAD9; Ferroptosis; Linoleic acid; Mitochondrial complex I; Ovarian cancer; Redox
    DOI:  https://doi.org/10.1016/j.canlet.2025.217903
  9. Sci Adv. 2025 Jul 11. 11(28): eadu5915
      Although effective for immunologically hot tumors, immune checkpoint inhibitors minimally affect tumors that are not T cell inflamed, including breast cancer. An alternate strategy to combat immune cold breast tumors may be to reeducate innate immunity. This study identifies strategies to skew neutrophils to acquire tumoricidal properties. Systemic Toll-like receptor (TLR)-induced inflammation, concomitant with mitochondrial complex I inhibition in breast tumors, increases neutrophil cytotoxicity against breast cancer cells and independently of CD8+ T cell immunity. These therapy-entrained neutrophils enhance secretory granule production, increasing expression of the reduced form of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase machinery and inducing a respiratory burst. Moreover, systemic administration of TLR agonists elevates nuclear factor κB signaling in neutrophils to increase production of secretory granule and NADPH oxidase machinery components, whereas complex I inhibitors are required to potentiate oxidative damage. In summary, we describe a class of neutrophils, educated by the combined action of inflammatory mediators and metabolic inhibitors, having tumoricidal functions.
    DOI:  https://doi.org/10.1126/sciadv.adu5915
  10. Mol Cancer Ther. 2025 Jul 10.
      Targeting PIK3CA mutant colorectal cancers (CRCs) with precision medicine strategies is of great clinical interest. However, resistance to single agent PI3K pathway inhibitors has been observed across multiple clinical trials, necessitating identification of combination therapies that overcome or prevent resistance to precision medicine strategies. Previously, our group identified that inhibition of MTORC1/2 is necessary to induce a response in PIK3CA mutant CRCs. The PI3K/MTORC1/2 inhibitor copanlisib has demonstrated some clinical activity in PIK3CA mutant solid tumors as part of the NCI MATCH trial. Here we evaluate potential combination therapies that could enhance the efficacy of copanlisib and other similar inhibitors in PIK3CA mutant CRCs. Using a novel high-throughput drug screen method in Apc and Pik3ca mutant mouse-derived cancer organoids, we identify navitoclax, a BCL-2 family inhibitor, as a drug that could potentially enhance the response to copanlisib. Across multiple in vitro and in vivo CRC models, navitoclax enhanced PI3K/MTOR inhibition (copanlisib, sapanisertib, and dactolisib) and induced apoptosis. Furthermore, we examine these combination therapies across a panel of patient-derived cancer organoids with a range of mutation profiles. These studies indicate that KRAS mutations could confer resistance. Furthermore, we identify BCL-xL as the major BCL-2 family target important for the response to this combination in this setting. This provides a strong rationale for MTORC1/2 and BCL-2 family inhibition as a potential treatment strategy for PIK3CA mutant CRCs.
    DOI:  https://doi.org/10.1158/1535-7163.MCT-24-1096
  11. Bioessays. 2025 Jul 06. e70038
      Mitochondrial membrane potential is highly dependent on coupled as well as uncoupled respiration. While brown adipose tissue (BAT) mediates non-shivering thermogenesis (NST), a highly adaptive bioenergetic process critical for energy metabolism, the relationship of coupled and uncoupled respiration in thermogenic adipocytes remains complicated. Uncoupling protein 1 (UCP1)-mediated proton leak is the primary driver of NST, but recent studies have shown that oxidative phosphorylation may be an underappreciated contributor to UCP1-dependent NST. Here, we highlight the role of ATP synthase for BAT thermogenesis and discuss the implications of fine-tuning adrenergic signaling in brown adipocytes by the protein inhibitory factor 1 (IF1). We conclude by hypothesizing future directions for mitochondrial research, such as investigating the potential role of IF1 for mitochondrial substrate preference, structural dynamics, as well as its role in cell fate decision and differentiation.
    Keywords:  UCP1; adipocytes; bioenergetics; metabolism; mitochondria; obesity; thermogenesis
    DOI:  https://doi.org/10.1002/bies.70038
  12. Nat Commun. 2025 Jul 10. 16(1): 6391
      Mitochondria contain their own DNA (mtDNA) and a dedicated gene expression machinery. As the mitochondrial dimensions are close to the diffraction limit of classical light microscopy, the spatial distribution of mitochondrial proteins and in particular of mitochondrial mRNAs remains underexplored. Here, we establish single-molecule fluorescence in situ hybridization (smFISH) combined with STED and MINFLUX super-resolution microscopy (nanoscopy) to visualize individual mitochondrial mRNA molecules and associated proteins. STED nanoscopy reveals the spatial relationships between distinct mRNA species and proteins such as the RNA granule marker GRSF1, demonstrating adaptive changes in mRNA distribution and quantity in challenged mammalian cells and patient-derived cell lines. Notably, STED-smFISH shows the release of mRNAs during apoptosis, while MINFLUX reveals the folding of the mRNAs into variable shapes, as well as their spatial proximity to mitochondrial ribosomes. These protocols are transferable to various cell types and open new avenues for understanding mitochondrial gene regulation in health and disease.
    DOI:  https://doi.org/10.1038/s41467-025-61577-5
  13. Cell Immunol. 2025 Jun 28. pii: S0008-8749(25)00086-3. [Epub ahead of print]414 105000
      Upon activation, naïve T cells undergo rapid proliferation and differentiation, giving rise to clonally expanded populations specifically tailored for an effective immune response. To meet the heightened bioenergetic and biosynthetic demands associated with activation, T cells adapt and reprogram both their metabolism and transcriptome. Beyond this, T cells are also able to dynamically adapt to fluctuations in the microenvironmental nutrient levels. While the adaptability of T cells is a well-established hallmark of their functionality, the molecular mechanisms by which metabolic responses underpin this flexibility remain incompletely defined. Acetyl-CoA, with its role as a central metabolite in mitochondrial ATP production, and a substrate for nuclear histone acetylation reactions, emerges as a key player in a metabolic-epigenetic axis. Recent evidence indicates that enzymes responsible for generating acetyl-CoA can translocate to the nucleus, supporting sub-cellular local acetyl-CoA production. Here, we explore the impact of acetyl-CoA metabolism on T cell functionality within different subcellular compartments and highlight the potential for intervention in acetyl-CoA metabolic pathways in T cell-driven autoimmune diseases and cancers.
    Keywords:  Acetyl-CoA; Epigenetic remodelling; Metabolic reprogramming; Nuclear metabolism; T cells
    DOI:  https://doi.org/10.1016/j.cellimm.2025.105000
  14. Biochim Biophys Acta Mol Cell Biol Lipids. 2025 Jul 08. pii: S1388-1981(25)00069-1. [Epub ahead of print] 159661
      Lipid metabolism reprogramming is a well-established hallmark of many cancer types, including colorectal cancer (CRC). Nevertheless, a clear understanding on how fatty acid (FA) metabolism is fine-tuned during CRC development and progression is still missing. Given that CRC is the second leading cause of cancer-related death, addressing these critical aspects may provide the rationale for new therapeutic approaches and early biomarker identification. Fatty acid binding protein 1 (FABP1) is a small protein that binds FA and other lipophilic compounds, acting as a lipid transporter in the intestine. Little is currently known about the function of FABP1 in CRC. Here we show that the knockdown of FABP1 in CRC cells impairs de novo FA and cholesterol synthesis, specifically, via altering the transcriptional regulation of lipid metabolism genes. FABP1 depletion suppresses the expression of FA and cholesterol synthesis-associated genes while promoting that of FA oxidation genes and mitochondrial oxidative pathways. The latter is associated with increased oxygen consumption rate and activation of the energy sensor 5' AMP-activated kinase (AMPK). Taken together, our results show that FABP1 orchestrates the balance between FA synthesis and oxidation, most likely to prevent the cytotoxic effects of circulating unbound free fatty acids. Thus, targeting FABP1 function may represent a potential therapeutic strategy in advanced CRC.
    Keywords:  Colorectal cancer; FABP1; Fatty acid biosynthesis; Fatty acid β-oxidation; Lipogenesis; Oxidative metabolism
    DOI:  https://doi.org/10.1016/j.bbalip.2025.159661
  15. Nature. 2025 Jul 09.
      Decreased brain levels of coenzyme Q10 (CoQ10), an endogenously synthesized lipophilic antioxidant1,2, underpin encephalopathy in primary CoQ10 deficiencies3,4 and are associated with common neurodegenerative diseases and the ageing process5,6. CoQ10 supplementation does not increase CoQ10 pools in the brain or in other tissues. The recent discovery of the mammalian CoQ10 headgroup synthesis pathway, in which 4-hydroxyphenylpyruvate dioxygenase-like protein (HPDL) makes 4-hydroxymandelate (4-HMA) to synthesize the CoQ10 headgroup precursor 4-hydroxybenzoate (4-HB)7, offers an opportunity to pharmacologically restore CoQ10 synthesis and mechanistically treat CoQ10 deficiencies. To test whether 4-HMA or 4-HB supplementation promotes CoQ10 headgroup synthesis in vivo, here we administered 4-HMA and 4-HB to Hpdl-/- mice, which model an ultra-rare, lethal mitochondrial encephalopathy in humans. Both 4-HMA and 4-HB were incorporated into CoQ9 and CoQ10 in the brains of Hpdl-/- mice. Oral treatment of Hpdl-/- pups with 4-HMA or 4-HB enabled 90-100% of Hpdl-/- mice to live to adulthood. Furthermore, 4-HB treatment stabilized and improved the neurological symptoms of a patient with progressive spasticity due to biallelic HPDL variants. Our work shows that 4-HMA and 4-HB can modify the course of mitochondrial encephalopathy driven by HPDL variants and demonstrates that CoQ10 headgroup intermediates can restore CoQ10 synthesis in vivo.
    DOI:  https://doi.org/10.1038/s41586-025-09246-x
  16. Cells. 2025 Jun 21. pii: 951. [Epub ahead of print]14(13):
      Cytochrome c (Cytc) is a multifunctional protein, essential for respiration and intrinsic apoptosis. Post-translational modifications of Cytc have been linked to physiological and pathophysiologic conditions, including cancer. Cytc tyrosine 67 (Y67) is a conserved residue that is important to the structure and function of Cytc. We here report the phosphorylation of Y67 of Cytc purified from bovine heart mapped by mass spectrometry. We characterized the functional effects of Y67 Cytc modification using in vitro and cell culture models. Y67 was mutated to the phosphomimetic glutamate (Y67E) and to phenylalanyl (Y67F) as a control. The phosphomimetic Y67E Cytc inhibited cytochrome c oxidase (COX) activity, redirecting energy metabolism toward glycolysis, and decreased the pro-apoptotic capabilities of Cytc. The phosphomimetic Y67E Cytc showed a significantly impaired rate of superoxide scavenging and a reduced rate of oxidation by hydrogen peroxide, suggesting a lower ability to transfer electrons and scavenge reactive oxygen species (ROS). Phosphomimetic Y67E replacement led to an almost complete loss of cardiolipin peroxidase activity, pointing to a central role of Y67 for this catalytic function of Cytc. In intact cells, phosphomimetic replacement leads to a reduction in cell respiration, mitochondrial membrane potential, and ROS levels. We propose that Y67 phosphorylation is cardioprotective and promotes cell survival.
    Keywords:  apoptosis; cell signaling; cytochrome c; electron transport chain; heart; mitochondrial membrane potential; phosphorylation; reactive oxygen species
    DOI:  https://doi.org/10.3390/cells14130951
  17. Science. 2025 Jul 10. 389(6756): 157-162
      Maintenance of mitochondrial redox homeostasis is of fundamental importance to cellular health. Mitochondria harbor a host of intrinsic antioxidant defenses, but the contribution of extrinsic, nonmitochondrial antioxidant mechanisms is less well understood. We found a direct role for peroxisomes in maintaining mitochondrial redox homeostasis through contact-mediated reactive oxygen species (ROS) transfer. We found that ACBD5 and PTPIP51 form a contact between peroxisomes and mitochondria. The percentage of these contacts increased during mitochondrial oxidative stress and helped to maintain mitochondrial health through the transfer of mitochondrial ROS to the peroxisome lumen. Our findings reveal a multiorganelle layer of mitochondrial antioxidant defense-suggesting a direct mechanism by which peroxisomes contribute to mitochondrial health-and broaden the scope of known membrane contact site functions.
    DOI:  https://doi.org/10.1126/science.adn2804
  18. FASEB Bioadv. 2025 Jul;7(7): e70030
      Cell homeostasis and metabolic control require the efficient function of mitochondria and implementation of quality control pathways following damage. Cells have various discrete pathways of mitochondrial quality control (mitoQC) to maintain the healthy network. PINK1 and Parkin are two key players in mitoQC, most highly associated with the ubiquitin-dependent capture and degradation of whole mitochondria by autophagy. However, these proteins have alternative roles in repair routes directing locally damaged cargo to the lysosome, such as the mitochondrial-derived vesicle (MDV) pathway. We aimed to clarify the role of PINK1 and determine how its loss of function impacts mitochondrial dynamics and quality control. Results indicate PINK1 knockout (KO) has little impact on whole mitochondrial turnover in response to damage in SH-SY5Y cells, whereas both PINK1 and Parkin KO cells have healthy mitochondrial networks with efficient ATP production. However, TOM20 positive outer-membrane and damage-induced PDH-positive inner-membrane MDVs are elevated in PINK1 KO cells. Although, in contrast to Parkin KO, this is not due to a defect in trafficking to a LAMP1-positive compartment and may instead indicate increased damage-induced flux. In comparison, loss of Atg5-dependent mitophagy has no effect on whole mitochondrial turnover and only results in a limited elevation in inner-membrane MDVs in response to damage, indicating autophagy-independent mechanisms of whole mitochondrial turnover and a minor compensatory increase in damage-induced MDVs. Therefore, these data suggest PINK1 and Parkin are dispensable for whole mitochondrial turnover, but following their perturbation have disparate effects on the MDV pathway.
    Keywords:  Parkinson's; lysosome; membrane trafficking; mitochondria; mitochondrial quality control; vesicle transport
    DOI:  https://doi.org/10.1096/fba.2024-00200
  19. Oncogene. 2025 Jul 10.
      Colorectal cancer (CRC) is the second leading cause of cancer deaths worldwide. One key reason is the lack of durable therapies that target KRAS-dependent disease, which represents approximately 40% of CRC cases. Here, we use liquid chromatography/mass spectrometry (LC/MS) analyses on Drosophila CRC tumour models to identify multiple metabolites in the glucuronidation pathway-a toxin clearance pathway that impacts most drugs-as upregulated in trametinib-resistant RAS/APC/P53 ("RAP") tumours compared to trametinib-sensitive RasG12V single mutant tumours. Genetic inhibition of different steps along the glucuronidation pathway strongly reversed RAP resistance to trametinib; conversely, elevating glucuronidation pathway activity was sufficient to direct trametinib resistance in RasG12V animals. Mechanistically, pairing oncogenic RAS with hyperactive WNT activity strongly elevated PI3K/AKT/GLUT signalling, which in turn directed elevated glucose uptake and glucuronidation; our data also implicate the pentose phosphate pathway in this process. We provide evidence that this mechanism of trametinib resistance is conserved in a KRAS/APC/TP53 mouse CRC tumour organoid model. Finally, we identify two clinically accessible approaches to inhibiting drug glucuronidation: (i) blocking an initial HDAC1-mediated deacetylation step of trametinib with the FDA-approved drug vorinostat; (ii) reducing blood glucose by the alpha-glucosidase inhibitor acarbose. Overall, our observations demonstrate a key mechanism by which oncogenic RAS/WNT activity promotes increased drug clearance in CRC and provides a practical path towards abrogating drug resistance in CRC tumours.
    DOI:  https://doi.org/10.1038/s41388-025-03472-3
  20. Mol Metab. 2025 Jul 08. pii: S2212-8778(25)00111-5. [Epub ahead of print] 102204
       OBJECTIVE: Mitochondrial uncouplers are used as chemical tools to study mitochondrial function in vitro and in vivo, and some molecules are in development for the treatment of metabolic diseases. One problem in the field is that any molecule that increases proton transport into the mitochondrial matrix independent of ATP production can be classified as an uncoupler regardless of off-target activities. Therefore, there are dozens of classes of molecules that exhibit a wide spectrum of phenotypes. Herein we directly compared 15 mitochondrial uncouplers side-by-side in a well-defined cell system to better understand their in vitro dose response profiles and the top molecules with suitable pharmacology and safety profiles were compared in db/db mice.
    METHODS: Fifteen mitochondrial uncouplers were characterised in vitro in CHO-K1 cells. The top five candidates were selected for further characterisation in male db/db mice based on their in vitro dose tolerance and / or tolerability. We tested two doses of each mitochondrial uncoupler in mice and benchmarked their efficacy to a lifestyle intervention of 35% calorie restriction as well as to lean db/+ metabolically healthy mice. Eleven groups of mice were fed either; 1) ad libitum - chow (control), 2) chow with 0.15% BAM15 (w/w), 3) chow with 0.2% BAM15 (w/w), 4) chow with 0.1% NEN (w/w), 5) chow with 0.25% NEN (w/w), 6) chow with 0.01% OPC-163493 (w/w), 7) chow with 0.02% OPC-63493 (w/w), 8) chow with 0.015% ES9 (w/w), 9) chow with 0.03% ES9 (w/w), 10) chow with 0.2% NTZ (w/w), and 11) chow with 0.4% NTZ (w/w). Another group of mice was fed chow to receive ∼65% of the average daily food intake of control mice as a model of calorie restriction (CR). Mice were metabolically phenotyped over 4 weeks of treatment with assessment of key readouts including body weight, HbA1c, blood glucose and glucose tolerance tests. At termination, key tissues were collected and plasma was analysed for markers of toxicity.
    RESULTS: Few mitochondrial uncouplers behaved similarly in vitro, with 11 molecules impairing maximal mitochondrial capacity. In vivo, BAM15 dose-dependently improved body weight and metabolic parameters in db/db mice, with 0.2% BAM15 treatment yielding statistically significant improvements in body weight, fat pad weight, glucose tolerance, blood glucose, HbA1c, liver weight and triglyceride content. The next-best treatment was 0.03% ES9 which significantly improved glucose tolerance, blood glucose levels, and HbA1c, but increased body weight, liver size and steatosis relative to db/db controls.
    CONCLUSIONS: Mitochondrial uncouplers BAM15 and ES9 had the greatest dose tolerance range in vitro, while BAM15 had the best overall effects on body weight, glucose control and liver steatosis in db/db mice. This study reveals diverse phenotypes across 15 classes of mitochondrial uncouplers and underscores the need for rigorous evaluation to identify molecules that drive stable mitochondrial respiration without unwanted mitochondrial inhibition or off-target effects. Ultimately, mitochondrial uncouplers should not be generalized and each uncoupler molecule needs to be considered by its own actions in well-defined experimental conditions.
    Keywords:  Mitochondrial uncoupling; diabetes; metabolic disease; obesity
    DOI:  https://doi.org/10.1016/j.molmet.2025.102204
  21. bioRxiv. 2025 Jul 02. pii: 2025.06.30.662349. [Epub ahead of print]
      Polyamines are essential and evolutionarily conserved metabolites present at millimolar concentrations in mammalian cells. Cells tightly regulate polyamine homeostasis through complex feedback mechanisms, yet the precise role necessitating this regulation remains unclear. Here, we show that polyamines function as endogenous buffers of redox-active iron, providing a molecular link between polyamine metabolism and ferroptosis. Using genome-wide CRISPR screens, we identified a synthetic lethal dependency between polyamine depletion and the key ferroptosis suppressor, GPX4. Mechanistically, we show that polyamine deficiency triggers a redistribution of cellular iron, increasing the labile iron pool and upregulating ferritin. To directly visualize this iron buffering in living cells, we developed a genetically encoded fluorescent reporter for redox-active iron. Live-cell analysis revealed a striking inverse correlation between intracellular polyamine levels and redox-active iron at single-cell resolution. These findings reposition polyamines as key regulators of iron homeostasis, with implications for ferroptosis-linked disease states and cellular redox balance.
    DOI:  https://doi.org/10.1101/2025.06.30.662349
  22. bioRxiv. 2025 Jul 05. pii: 2025.07.01.662602. [Epub ahead of print]
      Colorectal cancer (CRC) is the second leading cause of cancer-related mortality in the United States. CRC tumors exhibit aberrant iron accumulation, which supports tumor cell proliferation through multiple metabolic pathways. However, the oncogenic benefits of elevated iron must be counterbalanced by its potential to catalyze oxidative damage via reactive oxygen species generated from labile, redox-active iron. Ferroptosis is a regulated, non-apoptotic form of cell death characterized by iron-dependent lipid peroxidation. This process is tightly controlled by the selenoenzyme glutathione peroxidase 4 (GPX4), which reduces lipid peroxides and can be pharmacologically inhibited by agents such as RSL3 and JKE1674. A key source of redox-active iron is the labile iron pool (LIP), yet its role in regulating ferroptosis remains incompletely defined. To examine this, we supplemented CRC cells with exogenous iron following pharmacologic induction of ferroptosis. Iron supplementation significantly reduced cell viability, suggesting that expansion of the LIP potentiates ferroptotic cell death. However, whether ferroptosis is accompanied by dynamic changes in the LIP, and if such changes are mechanistically required for its potentiation, was unknown. To further characterize this response, we profiled the expression of iron regulatory genes under ferroptotic conditions and observed no change in transcriptional response in iron homeostasis genes. When using a fluorescent probe for labile iron, we found that the LIP did not measurably increase during ferroptosis induction. These findings suggest that the LIP itself does not expand after the initiation of ferroptosis to become the primary driver of ferroptotic potentiation.
    DOI:  https://doi.org/10.1101/2025.07.01.662602