bims-mibica Biomed News
on Mitochondrial bioenergetics in cancer
Issue of 2026–02–22
nineteen papers selected by
Kelsey Fisher-Wellman, Wake Forest University



  1. Nat Metab. 2026 Feb 17.
      Dihydroorotate dehydrogenase is a rate-limiting enzyme of de novo pyrimidine synthesis. In most eukaryotes, this enzyme is bound to the inner mitochondrial membrane, where it couples orotate synthesis to ubiquinone reduction. As ubiquinone must be regenerated by respiratory complex III, pyrimidine biosynthesis and cellular respiration are tightly coupled. Consequently, inhibition of respiration suppresses DNA synthesis and cell proliferation. Here we show that expression of the Saccharomyces cerevisiae URA1 gene (ScURA) in mammalian cells uncouples pyrimidine biosynthesis from mitochondrial electron transport. ScURA forms a homodimer in the cytosol that uses fumarate as an electron acceptor instead of ubiquinone, enabling respiration-independent pyrimidine biosynthesis. Cells expressing ScURA are resistant to drugs that inhibit complex III and the mitochondrial ribosome. Additionally, ScURA enables growth of mitochondrial-DNA-lacking ρ0 cells in uridine-deficient medium and ameliorates the phenotype of cellular models of mitochondrial diseases. Overall, this genetic tool uncovers the contribution of pyrimidine biosynthesis to the phenotypes arising from electron transport chain defects.
    DOI:  https://doi.org/10.1038/s42255-026-01454-7
  2. Cell Death Differ. 2026 Feb 19.
      It took decades from the discovery of BCL-2, initially identified in chromosomal translocations associated with lymphoid malignancies, to understand how BCL-2 and its family members regulate apoptosis, launching a transformative journey in cancer biology often called "the road to ruin". Developing powerful BCL-2 inhibitors for clinical use required decades. Yet, this remains as one of the most successful achievements in a field that started ~40 years ago, as recounted by its pioneers. BCL-2 was later found to inhibit apoptosis by preventing mitochondrial outer membrane permeabilization (MOMP), a breakthrough that clarified its role in cancer pathogenesis. Such effects of BCL-2 on MOMP prevent cytochrome c release and caspase activation, while its family members-anti-apoptotic proteins (e.g. BCL-2, BCL-XL) and pro-apoptotic proteins (e.g. BAX, BAK, BH3-only proteins)-orchestrate a delicate balance in cell death regulation. MicroRNAs like miR-15/16, often deleted in chronic lymphocytic leukaemia (CLL), modulate BCL-2 expression, driving oncogenesis. Mechanistically, BAX/BAK oligomerization forms mitochondrial pores, with sublethal MOMP triggering inflammation via cGAS-STING and NF-κB pathways. Alternative MOMP inducers (e.g. BOK) and mitochondrial dynamics further refine apoptotic control. Clinically, the BCL-2 inhibitor venetoclax has revolutionized CLL and acute myeloid leukemia (AML) treatment, showing efficacy in TP53-mutant CLL and elderly AML patients when combined with CD20 antibodies or hypomethylating agents. However, resistance, driven by BCL-2 mutations (e.g. Gly101Val) or MCL-1 upregulation, poses challenges. Limited success in solid tumors underscores the complexity of BCL-2 family dependencies. Future directions include novel inhibitors targeting MCL-1 or BCL-XL, BH3 profiling for precision therapy, and combinations with immune or DNA repair modulators. Non-apoptotic roles of BCL-2 in metabolism also warrant exploration. This review highlights the clinical success of BCL-2 inhibitors, addresses resistance mechanisms, and explores future directions, including sublethal MOMP, inflammatory outcomes, and novel inhibitors. Celebrating the collaborative, interdisciplinary efforts that transformed fundamental discoveries into life-saving therapies, this account underscores both the triumphs and the "potholes" encountered on the path to understanding apoptosis, while identifying open questions for ongoing research.
    DOI:  https://doi.org/10.1038/s41418-025-01607-3
  3. Mol Cell. 2026 Feb 13. pii: S1097-2765(26)00032-8. [Epub ahead of print]
      Inter-organellar communication is critical for cellular metabolism. One of the most abundant inter-organellar interactions occurs at the endoplasmic reticulum and mitochondria contact sites (ERMCSs). However, an understanding of the mechanisms governing ERMCS regulation and their roles in cellular metabolism is limited by a lack of tools that permit temporal induction and reversal. Through screening approaches, we identified fedratinib, an FDA-approved drug that dramatically increases ERMCS abundance by inhibiting the epigenetic modifier BRD4. Fedratinib rapidly and reversibly modulates mitochondrial and ER morphology, induces a distinct ER-mitochondria envelopment structure, and alters metabolic homeostasis. Moreover, ERMCS modulation depends on mitochondrial electron transport chain complex III function. Comparison of fedratinib activity to other reported inducers of ERMCSs revealed common mechanisms of induction and function, providing clarity to a growing body of experimental observations. In total, our results uncovered a novel epigenetic signaling pathway and an endogenous metabolic regulator that connects ERMCSs and cellular metabolism.
    Keywords:  bromodomain protein; endoplasmic reticulum-mitochondria contact sites; high-throughput screening; mitochondrial electron transport chain
    DOI:  https://doi.org/10.1016/j.molcel.2026.01.012
  4. Exp Hematol. 2026 Feb 13. pii: S0301-472X(26)00034-2. [Epub ahead of print] 105401
      Acute myeloid leukemia (AML) is one of the hematological malignancies with a poor outcome. AML has a unique metabolic status, and identifying its metabolic vulnerabilities is warranted. Recent genome-wide screenings suggest that heme synthesis might be such a vulnerability. Heme is required not only for hemoglobin synthesis but also for the proper function of hemoproteins. Cytochromes are such hemoproteins and are necessary for mitochondrial respiration. Therefore, heme synthesis inhibition can diminish AML by altering mitochondrial status and function. However, still little is known about the importance of heme synthesis in leukemia cells. To reveal the roles of heme synthesis in leukemia, we treated human leukemia cell lines with heme synthesis inhibitors, succinylacetone (SA) or N-methyl Protoporphyrin IX (NMPP). Heme synthesis inhibition induced cell growth inhibition and cell death in a concentration-dependent manner. Therefore, heme synthesis is required for leukemia cell proliferation and survival. Increased pro-apoptotic factors (cleaved caspase 3 and cleaved PARP) and decreased anti-apoptotic factor (XIAP) were observed following heme synthesis inhibition. Cytochrome c and Smac were released into the cytoplasm by heme synthesis inhibition, suggesting that heme synthesis inhibition led to mitochondrial outer membrane permeabilization and activation of the intrinsic pathway of apoptosis. Comprehensive transcriptomic analysis revealed that heme synthesis inhibition induced OSGIN1 expression, leading to the release of cytochrome c and Smac from mitochondria into the cytoplasm. Therefore, heme synthesis inhibition induced leukemia apoptosis by activating the intrinsic apoptosis pathway.
    Keywords:  OSGIN1; heme; intrinsic apoptosis pathway; leukemia
    DOI:  https://doi.org/10.1016/j.exphem.2026.105401
  5. Cell Death Discov. 2026 Feb 19.
      Mitochondria integrate senescence and apoptotic fates, yet it is unclear whether their ability to oxidize different fuels for energy production influences their vulnerability to senolytics in therapy-induced senescence (TIS). Using MitoPlates™ technology, we functionally mapped the mitophenotypes of TIS cancer cells by quantifying electron transport chain (ETC) flux from various NADH/FADH2 substrates. We then related these profiles to the responsiveness of TIS cancer cells to BCL-xL-targeting BH3 senolytics, as well as to inflammatory SASP signaling sensed by an NF-κB/miR-146a reporter. Mechanistically distinct senogenic stressors produced markedly different bioenergetic outputs and substrate diversity, establishing mitochondria as an emergent, stress-encoded property of TIS phenomena. Increased mitochondrial bioenergetic flexibility corresponded with senolytic permissiveness within each cell lineage. However, the magnitude of the senolytic response was largely limited by the pre-senescent bioenergetic configuration of the parental mitochondria, and baseline succinate oxidation served as a functional indicator of this inherited threshold. TIS SASPs were restricted by the secretome of the cell-of-origin, but only the miR146a-positive, fatty acid β-oxidation-related inflammatory SASP states were senolytically responsive. Inflachromene, an inhibitor of the chromatin remodelers HMGB1/2, decoupled mitochondrial bioenergetics from senolytic susceptibility, yielding SASP-null/miR146a-negative senescent cancer cells that were completely resistant to ABT-263/navitoclax and A1331852 despite extensive mitochondrial reprogramming. Thus, the senolytic response is governed by a layered circuit in which mitochondrial bioenergetic heritage establishes the senolytic ceiling, TIS-acquired bioenergetic flexibility fine-tunes the amplitude of the senolytic response, and establishing a mitochondria-inflammatory SASP crosstalk is required for BH3-mediated senolysis. These results support using functional readouts that integrate mitochondrial metabolic flexibility and inflammatory SASP to predict and potentially enhance senolytic efficacy in TIS cancer cells.
    DOI:  https://doi.org/10.1038/s41420-026-02967-6
  6. Neoplasia. 2026 Feb 14. pii: S1476-5586(26)00016-3. [Epub ahead of print]73 101287
       BACKGROUND: Metabolic reprogramming is a hallmark of colorectal cancer (CRC), yet the molecular regulators that orchestrate this process remain incompletely understood. Although many long non-coding RNAs (lncRNAs) possess protein-coding potential, their translational products and metabolic functions have been largely overlooked. Here, we identify MUCP1, a microprotein encoded by the lncRNA MUC20-OT1, as a critical regulator of mitochondrial metabolism and epigenetic remodeling in CRC.
    METHODS: Multi-omics data were integrated to identify MUC20-OT1 as a candidate lncRNA encoding a functional microprotein. Fusion reporter plasmids, mass spectrometry, and immunoblotting were used to validate MUCP1 translation and mitochondrial localization. Functional assays, metabolomic profiling, 13C5-glutamine isotope tracing, subcellular succinate quantification, CUT&Tag, and xenograft models were performed to investigate the role of MUCP1 in facilitating mitochondrial succinate export and maintaining glutamine metabolism homeostasis.
    RESULTS: The microprotein MUCP1, encoded by the lncRNA MUC20-OT1, serves as an auxiliary regulator of SLC25A10-mediated mitochondrial succinate transport. MUCP1 is upregulated during CRC progression and localizes in the mitochondrial outer membrane, where it facilitates the balance of mitochondrial succinate metabolism. Elevated extramitochondrial succinate subsequently enhances H3K4me3 histone modifications, promoting the transcription of enzymes involved in glutamine metabolism and sustaining the high metabolic demands of CRC cells.
    CONCLUSIONS: This study identifies MUCP1 as a novel lncRNA-encoded microprotein that maintains metabolic homeostasis in CRC by coupling mitochondrial succinate transport to histone methylation. MUCP1 might be a promising metabolic vulnerability and therapeutic target in CRC.
    Keywords:  Glutamine metabolism; H3K4me3; MUCP1; SLC25A10; Succinate
    DOI:  https://doi.org/10.1016/j.neo.2026.101287
  7. Biochem Pharmacol. 2026 Feb 13. pii: S0006-2952(26)00146-2. [Epub ahead of print]247 117815
      Myeloid leukemia associated with Down syndrome (ML-DS), as classified by WHO 2016, includes acute myeloid leukemia (AML) and myelodysplasia in children with DS. While ML-DS patients show high sensitivity to cytarabine (Ara-C)-based chemotherapy with better overall survival than non-DS AML patients, relapsed/refractory cases have dismal outcomes. This underscores the need to understand Ara-C-resistance mechanisms and develop effective therapies. The chromosome 21 gene, cystathionine-β-synthase (CBS), is significantly overexpressed in ML-DS cells. Overexpression of CBS leads to increased hydrogen sulfide (H2S) production, which reduces complex IV activity and oxidative phosphorylation (OXPHOS). OXPHOS has been shown to play an important role in Ara-C resistance in non-DS AML. Thus, in this study, we investigated the role of CBS as a regulator of OXPHOS and Ara-C response. We found that Ara-C-resistant ML-DS cells have lower CBS activity. Overexpression of CBS in an Ara-C-resistant ML-DS cell line resulted in increased H2S and Ara-C sensitivity and decreased both complex IV activity and OXPHOS. Knockdown of CBS in an Ara-C-sensitive ML-DS cell line increased OXPHOS and Ara-C resistance. However, complex IV activity decreased and H2S production was unchanged, indicating that CBS regulates OXPHOS through both a H2S-dependent and -independent mechanism. We further demonstrate that targeting both OXPHOS, using ONC213, and apoptosis, using venetoclax, results in synergistic induction of cell death in Ara-C-resistant ML-DS cells. This study identifies CBS as a regulator of OXPHOS and Ara-C response, while the combination of ONC213 and venetoclax offers a promising therapeutic approach for relapsed/refractory ML-DS, addressing key vulnerabilities to improve patient outcomes.
    Keywords:  Ara-C-resistant; CBS; Cystathionine-β-synthase; Hydrogen sulfide; ML-DS; OXPHOS
    DOI:  https://doi.org/10.1016/j.bcp.2026.117815
  8. BioMed (Basel). 2026 Mar;pii: 7. [Epub ahead of print]6(1):
      Glutamine metabolism has emerged as one of the most critical bioenergetic and biosynthetic programs sustaining leukemic cell growth, survival, stemness and therapeutic resistance. In both acute and chronic leukemias, including acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL), malignant cells display a strong dependency on extracellular glutamine to support mitochondrial respiration, anabolic biosynthesis and redox homeostasis. This dependency is reinforced by oncogenic signaling networks, post-transcriptional metabolic regulation and microenvironmental adaptation within the bone marrow niche. Therapeutic strategies targeting glutamine utilization, including glutaminase inhibition, transporter blockade and enzymatic glutamine depletion, have demonstrated robust antileukemic activity in preclinical models, and early clinical efforts have begun to explore glutamine-directed interventions in myeloid neoplasms. However, metabolic plasticity, microenvironment-derived nutrient buffering and systemic toxicity remain significant limitations to clinical translation. This review provides a detailed synthesis of the biochemical framework of glutamine metabolism in leukemia, the molecular mechanisms enforcing glutamine addiction, the downstream functional consequences on proliferation, redox balance and leukemic stem cell biology, the current landscape of therapeutic strategies and emerging directions aimed at overcoming resistance and improving clinical efficacy.
    Keywords:  FLT3-ITD; IGF2BP2; SLC1A5; acute myeloid leukemia (AML); cancer metabolism; glutaminase inhibition; glutamine addiction; glutamine metabolism; leukemia; leukemic stem cells; metabolic targeting; redox balance; therapeutic resistance
    DOI:  https://doi.org/10.3390/biomed6010007
  9. Nat Commun. 2026 Feb 17.
      Cell fate determination is closely linked to metabolic state, yet how metabolic remodeling influences human pluripotent stem cells differentiation into three germ layers remains incompletely understood. Here, we reveal that definitive endoderm differentiation from human pluripotent stem cells requires a TGFβ-driven metabolic switch characterized by reduced lactate production and enhanced TCA cycle activity and oxidative phosphorylation, mediated by PDHB. Disruption of glucose utilization or pyruvate entry into the TCA cycle markedly impairs endoderm differentiation, whereas inhibition of lactate production enhances differentiation efficiency. Mechanistically, blockade of glucose metabolism or the TCA cycle reduces intracellular ATP levels, compromising the activity of BAF complex, an ATP-dependent chromatin remodeling complex centered on BRG1. This complex promotes chromatin accessibility and activates endodermal gene programs during differentiation. Together, these findings highlight metabolic reprogramming as a key regulator of human endoderm fate through ATP-dependent control of chromatin remodeling.
    DOI:  https://doi.org/10.1038/s41467-026-69510-0
  10. Leukemia. 2026 Feb 17.
      Acute myeloid leukemia (AML) is an aggressive myeloid malignancy with a poor prognosis. Venetoclax (Ven), a BCL2 inhibitor, has shown promising results but often leads to relapse due to mitochondrial dysregulation, particularly due to upregulation of the anti-apoptotic protein MCL1. Overexpression of the transcription factor STAT3 has been linked to poor survival in AML patients. Overexpression of STAT3 in a transgenic murine model induces a myeloid malignancy with a short latency period and inflammatory upregulation. The current study identifies STAT3 upregulation as a key mechanism of Ven resistance. A clinically relevant STAT3 degrader effectively reduces both total and phosphorylated STAT3, corrects mitochondrial structural and functional dysregulation, and induces apoptosis in Ven-resistant AML cell lines. KT-333 significantly decreases STAT3 and MCL1 protein levels and improves survival in Ven-resistant (Ven-Res) AML murine models. In summary, STAT3 hyperactivation is leukemogenic, is further potentiated in Ven-resistance and can be clinically targeted with a novel and specific STAT3 degrader. Pictorial representation depicting upregulation of STAT3 and MCL1 in venetoclax resistant myeloid malignancies such as MDS and AML causing mitochondrial structural abnormalities and dysfunction. By using specific STAT3 degrader, STAT3 inhibition, and thereby indirect downregulation of MCL1 can be a promising therapeutic intervention to target drug resistant clones in MDS and AML.
    DOI:  https://doi.org/10.1038/s41375-026-02883-9
  11. Nat Commun. 2026 Feb 18.
      Chronic lymphocytic leukemia (CLL) can arise from lymphoid clonal hematopoiesis of indeterminate potential (L-CHIP), but many individuals who develop CLL lack detectable L-CHIP prior to diagnosis. To identify additional predictors of CLL risk, we analyze mitochondrial heteroplasmy in 419,154 individuals from the UK Biobank (UKB). Heteroplasmy is associated with a 1.5-fold increased risk of developing CLL, and this risk rises to 4-fold when accounting for deleterious heteroplasmic variants. These findings are confirmed in an independent cohort, the All of Us Research Program (AoU). Notably, the associations remain significant even in the absence of L-CHIP, highlighting heteroplasmy's potential utility as an independent biomarker. Moreover, heteroplasmy is enriched in individuals with high-risk L-CHIP genotypes and large clonal burden, suggesting a potential biological role in malignant transformation. Here, we show that mitochondrial heteroplasmy, especially functionally deleterious variants, identifies individuals at increased risk of CLL who would otherwise go undetected by L-CHIP-based assessments.
    DOI:  https://doi.org/10.1038/s41467-026-69861-8
  12. ACS Chem Biol. 2026 Feb 18.
      The Bcl-2 family of proteins governs mitochondrial outer membrane (MOM) permeabilization, a critical step in apoptosis that is dysfunctional in many cancers. Although cellular studies have long implicated direct interactions between the pore-forming apoptotic Bax protein and its opponent, the antiapoptotic Bcl-2 protein in apoptosis regulation, the underlying basic principles behind this control remained unresolved. To provide in-depth insight, we carried out a systematic biophysical study in which we utilized neutron reflectometry (NR) and ATR-FTIR to elucidate the molecular communication between those proteins in and around the mitochondrial membrane environment. The spatial and temporal changes across model MOM surfaces were resolved during the interaction of Bax with Bcl-2. The NR-derived membrane surface Bax distributions suggested that Bcl-2 mediated Bax sequestration through both Bcl-2/Bax heterodimerization and Bax/Bax oligomerization. Kinetic analysis revealed a two-step process: rapid formation of Bcl-2/Bax heterodimers, followed by slower Bax oligomerization on these complexes. Importantly, this sequestration mechanism was also observed in the presence of cardiolipin, a lipid known to promote the formation of an apoptotic pore by Bax in the absence of Bcl-2. These findings suggest a fundamental mechanism by which cancer cells may evade apoptosis by exploiting Bcl-2's ability to neutralize Bax through structural entrapment, even if excess Bax is present, either in response to treatment or natural death signals.
    DOI:  https://doi.org/10.1021/acschembio.5c00913
  13. Nat Rev Cancer. 2026 Feb 20.
      It is well established that malignant cells alter their metabolism to support proliferation, but the nutrients required to meet the anabolic demands of different cancers located at various anatomical sites throughout the body remain largely unknown. Moreover, the extent to which nutrients are supplied by neighbouring stromal cells or distant tissues, possibly due to metabolic reprogramming, is poorly understood. Metabolomics provides a unique biochemical approach to address these gaps in our knowledge, but cancer studies require careful consideration because it is challenging to identify appropriately matched control samples for comparison. Here, we detail a collection of metabolomics workflows designed to interrogate cancer across three discrete scales. First, we describe experiments to define the nutrient demands of cancer cells themselves. Second, we focus on identifying metabolic relationships between neighbouring cells in the tumour microenvironment. Finally, we highlight strategies to explore the metabolic crosstalk between cancer cells and distant tissues in the tumour macroenvironment. The approaches outlined span cells in culture, animal models and human specimens from patients with cancer. Special emphasis is dedicated to the application of emerging technologies and computational pipelines in the field of mass spectrometry that enable global profiling of metabolites and lipids.
    DOI:  https://doi.org/10.1038/s41568-026-00908-0
  14. Autophagy Rep. 2026 ;5(1): 2627062
      In PINK1 (PTEN induced kinase 1)/PRKN (Parkin)-mediated mitophagy, the rupture of the outer mitochondrial membrane (OMM) emerges as a crucial event required for efficient mitochondrial clearance. Mechanistically, OMM rupture exposes inner mitochondrial membrane (IMM) mitophagy receptors, facilitating subsequent autophagic removal. Despite the important role of OMM rupture in mitophagy, the underlying mechanism remains elusive and technically difficult to monitor. In a recent study, we developed a novel fluorescent biosensor to directly visualize OMM rupture. This technique enables temporal and spatial characterization of OMM rupture and provides a powerful platform to dissect the underlying mechanism. Using this tool, we revealed that VCP (valosin containing protein) and its recruitment factors are required for OMM rupture, suggesting that VCP-dependent remodeling of the OMM proteome primes the rupture of OMM during mitophagy. Abbreviations: ARIH1, Ariadne RBR E3 ubiquitin protein Ligase 1; AMFR, autocrine motility factor receptor; ANKRD13A, ankyrin repeat domain-containing protein 13 A; FUNDC1, FUN14 domain containing 1; OA, oligomycin and antimycin; CID, chemical-induced dimerization; IMM, nner mitochondrial membrane; LC3, microtubule-associated protein 1 light chain 3; MUL1, mitochondrial E3 ubiquitin protein ligase 1; NIX, BCL2 interacting protein 3 like; OMM, outer mitochondrial membrane; UBXN1, ubiquitin regulatory X domain-containing protein 1; UBXN6, ubiquitin regulatory X domain-containing protein 6; VCP, valosin-containing protein; WIPI2, WD repeat domain phosphoinositide interacting protein 2.
    Keywords:  Biosensor; Mitochondrial outer membrane rupture; Mitochondrial quality control; PINK1/Parkin-mediated mitophagy; VCP
    DOI:  https://doi.org/10.1080/27694127.2026.2627062
  15. Endocr Relat Cancer. 2026 Feb 19. pii: ERC-25-0397. [Epub ahead of print]
      The hereditary pheochromocytoma and paraganglioma (hPPGL) syndrome, caused by germline succinate dehydrogenase (SDHx) gene mutation, predisposes individuals to pheochromocytomas (Pheo), paragangliomas (PGL), renal cell carcinoma (RCC), and gastrointestinal stromal tumors (GIST). Notably, tumors with succinate dehydrogenase subunit B (SDHB) deficiency demonstrate increased metastatic risk and current systemic treatments remain only palliative. Hence, discovering novel therapeutic avenues to improve SDHB-cancer prognosis is an urgent need. Here we leveraged human SDHB-deficient UOK269 RCC cells (SDHB-KO) and isogenic SDHB-reconstituted control cells (SDHB-WT) to discover SDH-dependent mitochondria-directed cytotoxic agents. Given the reduced ATP-generating capacity of SDHB-KO cells, we hypothesized they would be uniquely sensitive to futile cycle induction with mitochondrial ionophores. Indeed, ionophores exhibited preferential cytotoxicity toward SDHB-KO cells. However, the mitochondria-directed chemotherapeutic compound Ym155 demonstrated more potent and dramatic preferential cytotoxicity towards SDHB-KO cells. Importantly, SDH-dependent cytotoxicity of Ym155 was validated in multiple cell models, including primary human pheochromocytoma cells, a mouse pheochromocytoma (MPC) cell line and primary SDHB-deficient mouse kidney cells. Notably, genetic evidence of Ym155 synthetic lethality with SDHB-deficiency was buttressed in additional cell models using two chemical inhibitors of SDH enzyme activity. Mechanistically, SDH-deficiency sensitized cells to Ym155-induced DNA damage. Strikingly, SDH-dependent Ym155 sensitivity was recapitulated by inhibition of the histone demethylase KDM4, a downstream consequence of SDH deficiency. In summary, accumulation of succinate in SDH-deficient tumors inhibited KDM4 activity, impaired DNA repair and yielded enhanced susceptibility to Ym155-induced reactive oxygen species (ROS) generation. The identified intrinsic susceptibilities of SDHB-deficient cancers have the potential to be therapeutically leveraged.
    Keywords:  DNA damage; Mitochondrial redox imbalance; Pheochromocytomas and Paragangliomas (PPGL); Succinate dehydrogenase; Ym155; renal cell carcinoma; synthetic lethality
    DOI:  https://doi.org/10.1530/ERC-25-0397
  16. Cell Rep. 2026 Feb 18. pii: S2211-1247(26)00088-4. [Epub ahead of print]45(3): 117010
      Deregulated lipid metabolism contributes to leukemogenesis and the progression of acute myeloid leukemia (AML). By analyzing large-scale CRISPR-Cas9 screening data, we identified acyl-CoA synthetase long-chain family member 4 (ACSL4) as a selective vulnerability in lysine methyltransferase 2A-rearranged (KMT2Ar) AML. Functional validation using CRISPR interference and short hairpin RNA knockdown confirmed that ACSL4 loss impairs the growth of KMT2Ar but not non-KMT2Ar AML cells. ACSL4 knockdown reduced colony formation in cells derived from patients with KMT2Ar AML and murine MLL-AF9 cells and delayed leukemia onset in vivo in MLL-AF9 mice. A multi-omics approach, including transcriptomics, proteomics, and lipidomics, revealed depletion of polyunsaturated lipid species and compensatory activation of lipid metabolic pathways upon ACSL4 loss. Supplementation with exogenous polyunsaturated fatty acids (PUFAs) rescued the growth defect, linking ACSL4 dependency to defective PUFA utilization. Finally, we generated a KMT2Ar-ACSL4 dependency signature (KRADS12) that correlates with KMT2Ar status and predicts poor survival in patients with AML.
    Keywords:  CP: cancer; CP: metabolism; acute myeloid leukemia; chromosomal rearrangements; lipid metabolism
    DOI:  https://doi.org/10.1016/j.celrep.2026.117010
  17. Adv Sci (Weinh). 2026 Feb 17. e02239
      Ferroptosis is a type of regulated cell death characterized by the accumulation of lipid peroxides that damage cell membranes specifically. Mitochondrial swelling and dysfunction are hallmarks of ferroptosis; however, what causes mitochondrial swelling and the consequences of mitochondrial swelling in ferroptotic signal transduction remain poorly understood. Our study found that mitochondrial permeability transition pore (mPTP) opening is essential for mitochondrial swelling and ferroptosis activation. During ferroptosis, oxidized mitochondrial DNAs (mtDNAs) are released through the mPTP. These oxidized mtDNAs activate the cyclic GMP-AMP synthase (cGAS)-stimulator of interferon genes (STING) pathway, promoting ferroptosis through activating ferrotinophagy. Consistently, inhibition of mtDNA-repair enhances cellular sensitivity to ferroptosis and therefore synergizes with ferroptosis inducer in suppressing tumorigenesis in mouse xenograft tumor models. This study provides a fundamental understanding of how mPTP engages in ferroptosis by releasing mitochondrial DNAs as crucial messengers to activate ferroptotic signaling.
    Keywords:  cGAS‐STING; ferroptosis; mPTP; mitochondria; mtDNA
    DOI:  https://doi.org/10.1002/advs.202502239