bims-medebr Biomed News
on Metabolism of the developing brain
Issue of 2025–10–12
twenty papers selected by
Regina F. Fernández, Johns Hopkins University



  1. J Neurochem. 2025 Oct;169(10): e70255
      The brain derives its energy from a combination of several metabolic substrates. The principal energy substrate of the brain is glucose, but the metabolic role of cerebral lactate has been debated for decades. In particular, the hypothesis that astrocyte-derived lactate is needed to fuel neuronal metabolism during activation remains a heated topic. This Editorial highlights a study in the current issue of Journal of Neurochemistry exploring the metabolic relationship between glucose and lactate metabolism in sustaining neuronal network signaling. The study by Söder et al. demonstrates that neurons are only able to sustain energy-demanding synchronized synaptic transmission when glucose is freely available. Blocking lactate transport had no effect on neuronal signaling when glucose was present, highlighting that any potential transfer of lactate is not required during high neuronal workload. In fact, when lactate was supplied as the primary fuel, neurons were unable to sustain synchronized signaling. Using a lactate biosensor, the authors further show that neurons produce and release lactate, both during resting and stimulated conditions. As synchronized synaptic transmission underlies higher brain function, this paper underscores the absolute necessity of neuronal glucose metabolism to maintain brain function.
    Keywords:  astrocyte‐neuron lactate shuttle hypothesis; brain energy metabolism; glucose metabolism; metabolic shuttles; neuronal signaling
    DOI:  https://doi.org/10.1111/jnc.70255
  2. J Neurochem. 2025 Oct;169(10): e70251
      The metabolite lactate (L-lactate) has been hypothesized to represent an important energy source during brain activation. The contribution of lactate in fueling synchronized synaptic transmission during fast neural network oscillations underlying complex cortex function such as visual perception, memory formation, and motor activity is less clear, however. We explored the role of cellular lactate production and lactate transport (uptake and release) via the monocarboxylate transporters 1 and 2 (glial MCT1 and neuronal MCT2) during persistent gamma oscillations (frequency at around 40 Hz) and recurrent rhythmic events called sharp wave-ripples (with "ripples" at around 250 Hz) in cultured rat and acute mouse hippocampal slices (ex vivo) that received energy substrate supply with glucose (D-glucose) only. In addition, we assessed neuronal lactate dynamics during spontaneous activity ("resting state") and during electrical stimulation (10 Hz) in mouse primary neuron-astrocyte cultures (in vitro) receiving glucose only. We combined electrophysiology (local field potential recordings), tissue lactate analysis [ultra-performance liquid chromatography-mass spectrometry (UPLC-MS)], and live-cell fluorescence imaging [Förster resonance energy transfer (FRET) sensor Laconic]. We report that (1) lactate is produced during gamma oscillations when glucose is supplied and oxygen availability is unlimited (high oxygenation) for mitochondrial respiration. (2) The properties of gamma oscillations remain regular in the presence of the MCT1/2 blocker AR-C155858. (3) By contrast, MCT1/2 blockade fully suppresses gamma oscillations when mainly lactate is supplied. (4) The properties of sharp wave-ripples remain regular during MCT1/2 inhibition. (5) Lactate is produced in primary hippocampal neurons during spontaneous activity and electric stimulus-induced excitation, and it accumulates in the neuronal cytosol during MCT1/2 inhibition. In conclusion, lactate is produced in cortical tissue, including neurons fueled by glucose only. Moreover, lactate transport and lactate exchange ("shuttling") via glial MCT1 and neuronal MCT2 are not required to sustain synchronized synaptic transmission during fast neural network oscillations.
    Keywords:  aerobic glycolysis; brain energy metabolism; lactate oxidation; monocarboxylate transporter; neurotransmission; tissue oxygenation
    DOI:  https://doi.org/10.1111/jnc.70251
  3. Exp Neurol. 2025 Oct 06. pii: S0014-4886(25)00358-9. [Epub ahead of print] 115493
      Alzheimer's disease (AD) has been associated with impaired energy metabolism and neuronal mitochondrial dysfunction, which contribute to the development of anxiety-like behaviors and spatial memory deficits. Astrocytes are recognized as a critical source of metabolites for neurons, supporting mitochondrial respiration. Although physical exercise (PE) has shown therapeutic potential in AD models, the molecular mechanisms linking PE to metabolic reprogramming remain elusive. In a 5xFAD mouse model, this study shows that PE increased the expression of solute carrier family 2 member 1 (Slc2a1) and decreased the expression of GFAP in astrocytes. We demonstrate that both PE and the overexpression of astrocytic Slc2a1 alleviated neuronal mitochondria damage and neuron death, shifts astrocytes to an anti-inflammatory phenotype and reduced Aβ accumulation. Conversely, knockdown of Slc2a1 abrogated the protective effects of PE. In vitro, we established an AD glucose-deficiency cell model by incubating cells with 5.5 mM glucose and oligomeric Aβ (oAβ). Our results showed that Slc2a1 overexpression increased lactate secretion in the supernatant of C8-D1A astrocytes. Furthermore, both Slc2a1 overexpression in C8-D1A cells and lactate treatment rescued oAβ-induced mitochondrial oxidative stress and membrane potential alterations in energy-deficient HT22 neurons, thereby enhancing lactate secretion from astrocytes and promoting the lactate shuttle to neuron for energy supply. Collectively, our findings indicate that PE ameliorates anxiety-like behavior and spatial memory deficits, mitigates mitochondrial damage in neurons, and reduces Aβ accumulation in 5xFAD mice through the upregulation of Slc2a1 in astrocytes. Our findings identify Slc2a1 as a pivotal mediator of metabolic support induced by PE and highlights astrocyte-neuron lactate shuttling as a promising therapeutic target for AD.
    Keywords:  Alzheimer's disease; Astrocytes; Energy deficit; Physical exercise; Slc2a1
    DOI:  https://doi.org/10.1016/j.expneurol.2025.115493
  4. J Neurochem. 2025 Oct;169(10): e70252
      Alzheimer's disease (AD), the leading neurodegenerative disorder linked to aging, emerges within a paradoxical metabolic landscape. Despite rising cellular energy demands due to accumulated damage and stress, overall energy expenditure remains stable or declines with age. The brain, acting as the central regulator, responds to hypermetabolic signals from aged tissues by activating energy-conserving mechanisms. In this scenario, astrocytes, strategically located between blood vessels and neurons, play a pivotal role as energy sensors, adapting to systemic stress and modulating brain metabolism. This review explores how astrocytes undergo metabolic reprogramming in the early stages, potentially becoming maladaptive over time, fueling neuroinflammation, oxidative stress, and accelerating AD. By understanding astrocyte energetics, we uncover new avenues for biomarkers and therapies that could transform AD treatment.
    Keywords:  aging; allostasis; glycolysis; hypermetabolism; neurodegeneration
    DOI:  https://doi.org/10.1111/jnc.70252
  5. Front Neuroendocrinol. 2025 Oct 03. pii: S0091-3022(25)00043-3. [Epub ahead of print] 101217
      While mitochondria provide critical energy resources, mitochondrial dysfunction can lead to both metabolic and neurodegenerative disorders. Primary mitochondrial disorders (e.g., Leigh syndrome) are uniformly associated with profound neurodegeneration. Recent studies have also implicated mitochondrial dysfunction as a central feature of progressive neurodegenerative diseases, notably Alzheimer's disease, Parkinson's disease, Amyotrophic Lateral Sclerosis, and Huntington's Disease. In addition to its profound impact on metabolic disease, the glucagon-like peptide-1 receptor agonist, semaglutide, has significant neuroprotective features and may limit the progression of one or more of these disorders. These observations might be explained at least in part by the impact of this drug on mitochondrial function and energy production. Collectively, these observations highlight disrupted energy homeostasis as a critical feature of neurodegenerative disease and suggest novel targets for the development of much-needed new neuropharmaceutical strategies.
    Keywords:  Alzheimer’s disease; Glucagon-like Peptide 1; Mitochondria; Neurodegenerative disease; Oxidative phosphorylation; Parkinson’s disease; Semaglutide
    DOI:  https://doi.org/10.1016/j.yfrne.2025.101217
  6. Brain Commun. 2025 ;7(5): fcaf369
      Biallelic NDUFA9 variants have hitherto been associated with disease in four individuals. Hence, clinicogenetic features of NDUFA9-related disorder remain largely unexplored. To delineate the pheno-genotypic spectrum of NDUFA9-related disorder, we screened genetic databases worldwide and collected phenotypic data on individuals with biallelic NDUFA9 variants, which were functionally investigated when possible. Eight new and four reported cases were identified. Neurodevelopmental delay followed by motor deterioration and seizures were the most common presenting features. Neurodevelopmental disorder was observed in 90% of cases surviving beyond the age of 4 months. Neurological deterioration always started in the first decade. Among ten affected surviving beyond early infancy, major clinical features included dystonia (100%), feeding difficulties/dysphagia/failure to thrive and pyramidal signs (80%), seizures and muscle weakness/atrophy (70%), and moderate-to-severe intellectual disability (60%). All showed basal ganglia MRI signal alterations, with atrophy (50%) and swelling (25%). Four individuals died by the age of 13 years. In addition to four known variants, we identified five new NDUFA9 variants and pinpointed Arg360 (NP_004993.1) as a mutational hotspot. Protein modelling suggested that variants cause NADH:ubiquinone oxidoreductase subunit A9 (NDUFA9) misfolding and/or disruption of binding interfaces. Loss of fully assembled complex I with decreased steady-state NDUFA9 levels and/or complex I activity was documented in fibroblasts from three affected individuals. Our study strengthens the evidence that biallelic NDUFA9 variants cause mitochondrial complex I deficiency presenting with a broad spectrum of progressive neurodevelopmental disorder, often accompanied by prominent dystonia, and a characteristic Leigh syndrome MRI pattern.
    Keywords:  Leigh syndrome; lactic acidosis; mutational hotspot; seizures; spasticity
    DOI:  https://doi.org/10.1093/braincomms/fcaf369
  7. Acta Neuropathol. 2025 Oct 08. 150(1): 39
      Disturbances within the cerebrovascular system substantially contribute to the pathogenesis of age-related cognitive impairment and Alzheimer's disease (AD). Cerebral amyloid angiopathy (CAA) is characterized by the deposition of amyloid-β (Aβ) in the leptomeningeal and cortical arteries and is highly prevalent in AD, affecting over 90% of cases. While the ε4 allele of apolipoprotein E (APOE) represents the strongest genetic risk factor for AD, it is also associated with cerebrovascular dysregulations. APOE plays a crucial role in brain lipid transport, particularly in the trafficking of cholesterol and phospholipids. Lipid metabolism is increasingly recognized as a critical factor in AD pathogenesis. However, the precise mechanism by which APOE influences cerebrovascular lipid signatures in AD brains remains unclear. In this study, we conducted non-targeted lipidomics on cerebral vessels isolated from the middle temporal cortex of 89 postmortem human AD brains, representing varying degrees of CAA and different APOE genotypes: APOE ε2/ε3 (N = 9), APOE ε2/ε4 (N = 14), APOE ε3/ε3 (N = 21), APOE ε3/ε4 (N = 23), and APOE ε4/ε4 (N = 22). Lipidomics detected 10 major lipid classes with phosphatidylcholine (PC) and phosphatidylethanolamine (PE) being the most abundant lipid species. While we observed a positive association between age and total acyl-carnitine (CAR) levels (p = 0.0008), the levels of specific CAR subclasses were influenced by the APOE ε4 allele. Notably, APOE ε4 was associated with increased PE (p = 0.049) and decreased sphingomyelin (SM) levels (p = 0.028) in the cerebrovasculature. Furthermore, cerebrovascular Aβ40 and Aβ42 levels showed associations with sphingolipid levels including SM (p = 0.0079) and ceramide (CER) (p = 0.024). Weighted correlation network analysis revealed correlations between total tau and phosphorylated tau and lipid clusters enriched for PE plasmalogen and lysoglycerophospholipids. Taken together, our results suggest that cerebrovascular lipidomic profiles offer novel insights into the pathogenic mechanisms of AD, with specific lipid alterations potentially serving as biomarkers or therapeutic targets for AD.
    Keywords:  Alzheimer’s disease; Amyloid β; Apolipoprotein E; Cerebral amyloid angiopathy; Human induced pluripotent stem cell; Lipidomics; Tau; Vascular mural cells
    DOI:  https://doi.org/10.1007/s00401-025-02949-5
  8. J Physiol. 2025 Oct 09.
      The mitochondrial Ca2+ uniporter (MCU) links energy metabolism to cell excitability and signalling throughout the lifespan. However, whether neural metabolism responds to MCU impairments in a sex-specific manner has remained unknown, especially in models with partial MCU downregulation. Using hippocampal slices from adult heterozygous Mcu knock-out (hKO) mice, we observed sexually dimorphic changes in NAD(P)H autofluorescence dynamics following neuronal stimulation. In male mice, these signals were preserved despite decreased mitochondrial Ca2+ uptake, likely due to increased MDH2 levels and potentially other enzymes from the tricarboxylic acid cycle, the malate aspartate shuttle, and glycolysis. In contrast to males, neural tissue from female hKO mice showed delayed NAD(P)H production and limited NAD+ availability when compared to sex-matched controls, despite intact mitochondrial Ca2+ uptake. In addition, both male and female hKO mice exhibit decreased NADP+ levels and GSH/GSSG ratios (along with increased protein S-glutathionylation), indicating a weakened antioxidant capacity. Strikingly, markers of oxidative damage were also decreased (albeit more prominently in male mice), suggesting attenuated generation of reactive oxygen species. In addition, sex-specific changes in the hippocampal metabolome were manifested in hKO mice, along with a common decrease in spermidine levels. However, spermidine-dependent hypusination of eIF5A remained unaltered, suggesting further compensatory mechanisms at this age. In summary, our findings indicate that brain tissue can adapt to partial MCU deficits by salvaging most mitochondrial NADH production in active states, while compromising redox signalling and the polyamine pathway. The interplay between these molecular phenotypes likely impacts neurological conditions and potentially cognitive impairment with age. KEY POINTS: The inactivation of one Mcu allele (which encodes the mitochondrial Ca2+ uniporter) leads to altered neuronal excitability and attenuated mitochondrial Ca2+ elevations in active neurons from 6- to 12-months-old female and male mice, respectively. Tissue autofluorescence imaging reveals delayed mitochondrial NAD(P)H production in stimulated hippocampal tissue from female but not male heterozygous Mcu knockout mice. Mitochondrial Ca2+ uniporter haploinsufficiency is characterized by a sex-specific decrease in oxidative stress markers in the brain, despite a decline in NADP+ levels and the GSH/GSSG ratio in both male and female mice. Changes in the abundance of enzymes and polar metabolites in brain tissue reveal sexually dimorphic metabolic remodelling in the context of Mcu haploinsufficiency. Life-long downregulation of the mitochondrial Ca2+ uniporter results in decreased hippocampal spermidine levels in adult male and female mice.
    Keywords:  NAD(P)H; brain metabolism; calcium; hippocampus; mitochondria; sexual dimorphism; spermidine
    DOI:  https://doi.org/10.1113/JP287618
  9. Metabolism. 2025 Oct 06. pii: S0026-0495(25)00282-3. [Epub ahead of print] 156413
      4'-Phosphopantetheinyl (4'PP) groups are essential co-factors added to target proteins by phosphopantetheinyl transferase (PPTase) enzymes. Although mitochondrial 4'PP-modified proteins have been described for decades, a mitochondrially-localized PPTase has never been found in mammals. We discovered that the cytoplasmic PPTase aminoadipate semialdehyde dehydrogenase phosphopantetheinyl transferase (AASDHPPT) is required for mitochondrial respiration and oxidative metabolism. Loss of AASDHPPT results in failed 4'PP modification of the mitochondrial acyl carrier protein and blunted activity of the mitochondrial fatty acid synthesis (mtFAS) pathway. We found that in addition to its cytoplasmic localization, AASDHPPT localizes to the mitochondrial matrix via an N-terminal mitochondrial targeting sequence contained within the first 20 amino acids of the protein. Our data show that this novel mitochondrial localization of AASDHPPT is required to support mtFAS activity and oxidative metabolism. We further identify five variants of uncertain significance in AASDHPPT that are likely pathogenic in humans due to loss of mtFAS activity.
    Keywords:  Electron transport chain; Fatty acid synthesis; Metabolism; Mitochondria; Phosphopantetheine; Reductive carboxylation; Respiration
    DOI:  https://doi.org/10.1016/j.metabol.2025.156413
  10. Cell Death Differ. 2025 Oct 04.
      Neural activity drives blood vessel (BV) formation and energy substrate delivery in the developing brain to meet rising metabolic demands; however, the underlying mechanisms remain poorly understood. In this study, we exposed neonatal mice to chronic whisker stimulation (WS), a paradigm known to enhance BV formation in the somatosensory (S1) cortex. Transcriptomic (RNA-seq) and spatial (RNA-scope) analyses revealed that WS upregulated monocarboxylate transporter 2 (MCT2) in cortical neurons and MCT1 in endothelial cells (ECs). These changes coincided with increased cortical lactate levels, elevated astrocytic vascular endothelial growth factor A (VEGFa), and enhanced angiogenesis. Functional experiments demonstrated that neuronal MCT2 is essential for mediating WS-induced angiogenic and metabolic responses. Mechanistically, MCT2 facilitates L-lactate influx into the cortex with or without WS, promoting lactate uptake by neurons and astrocytes. This, in turn, induces MCT2 expression in neurons and activates hypoxia-inducible factor 1α (HIF1α) and VEGFa expression in astrocytes. Together, these findings uncover a previously unrecognized role for neuronal MCT2 in regulating lactate flux, signaling, and vascular remodeling, thereby linking neural activity to metabolic adaptation and vascular development in the neonatal mouse neocortex.
    DOI:  https://doi.org/10.1038/s41418-025-01581-w
  11. Research (Wash D C). 2025 ;8 0912
      Neurodegenerative diseases represent a major health threat, with dysfunction in energy metabolism and imbalance in glucose-lipid homeostasis constituting key pathogenic factors. As the cell's energy hub, mitochondria are closely associated with neurodegenerative diseases, such as Alzheimer's and Parkinson's diseases. However, the precise mechanism by which mitochondrial energy metabolism affects glucose-lipid homeostasis remains unclear. This review summarizes currents insights into the role of mitochondrial function in energy metabolism and glucose-lipid regulation in neurodegenerative diseases. We examined how mitochondrial dynamics, oxidative phosphorylation, calcium homeostasis, and key signaling pathways-AMP-activated protein kinase/mammalian target of rapamycin, peroxisome proliferator-activated receptor gamma coactivator 1-alpha, and Sirtuin 1-contribute to neuronal energy balance and metabolic regulation. We further explored the impact of other organelles on mitochondria and how the dynamic switching of mitochondrial morphology and function disrupts the critical glucose-lipid homeostasis. By focusing on mitochondrial dysfunction, metabolic disorders, and their interactions, we introduce the mitochondria-centered multi-organelle-energy metabolic-glucose-lipid homeostasis (MMH) network as a unifying theoretical framework that positions the progressive loss of metabolic flexibility as the fundamental essence of neurodegenerative disorders. The MMH network furnishes a novel lens through which the shared mechanistic underpinnings of neurodegenerative diseases can be deciphered, and thereby enable earlier diagnosis and precision therapeutics.
    DOI:  https://doi.org/10.34133/research.0912
  12. Proc Natl Acad Sci U S A. 2025 Oct 14. 122(41): e2519902122
      Glycolytic enzymes sense metabolite levels to adapt rapidly to changing energy demands, but measuring the levels of these effectors with spatiotemporal precision in live cells has been challenging. We addressed this question in the context of neuronal depolarization, which activates glycolysis, focusing on the glycolysis inhibitor citrate. We engineered a pair of quantitative fluorescent biosensors for citrate that address several limitations (affinity, pH, Mg2+, and temperature) of existing citrate biosensors. Using two-photon fluorescence lifetime imaging, we found that free citrate in the cytosol of neurons in acute mouse brain slices declines two-to-threefold within seconds of neuronal activation and then returns to baseline over a few minutes. The stimulation-dependent citrate transient depends at least in part on the mitochondrial calcium uniporter. These types of live metabolite measurements are essential for achieving a nuanced understanding of the fast control of glycolysis.
    Keywords:  fluorescence lifetime; genetically encoded fluorescent biosensor; glycolytic regulation; mitochondrial calcium uniporter
    DOI:  https://doi.org/10.1073/pnas.2519902122
  13. J Psychiatr Res. 2025 Oct 04. pii: S0022-3956(25)00578-3. [Epub ahead of print]191 542-546
       OBJECTIVE: Severe and prolonged underweight can lead to a hypometabolic state and hormonal adaptations that reduce body temperature often found in Anorexia Nervosa (AN). However, the effect of these changes on brain temperature remains unclear. We aimed to investigate whether brain temperature remains stable despite lower body temperature in a severe underweight state, testing the hypothesis that the brain prioritizes its own energy needs during periods of food deprivation.
    METHOD: We collected magnetic resonance spectroscopy (MRS) measurements from 30 female patients with acute Anorexia Nervosa (acAN) in a severe underweight state and 30 age-matched healthy female control participants (HC). MRS allows for a non-invasive assessment of brain temperature by calculating the difference between the temperature-independent peak of N-acetylaspartate (NAA) and the temperature-dependent peak of water (H20) in each voxel (ΔH20-NAA).
    RESULTS: Our results showed no group differences in (ΔH20-NAA) between acAN and HC. This is supported by Bayesian hypothesis testing, providing strong evidence for the absence of lower brain temperatures in severely underweight states.
    CONCLUSION: Our results are an indication that in a state of low energy availability, brain metabolism is prioritized.
    Keywords:  Anorexia nervosa; Brain temperature; Magnetic resonance spectroscopy; Metabolism
    DOI:  https://doi.org/10.1016/j.jpsychires.2025.09.055
  14. J Cell Sci. 2025 Oct 09. pii: jcs.263903. [Epub ahead of print]
      Mitochondrial dynamics relies on the function of dynamin family GTPase proteins including mitofusin 1 (MFN1), mitofusin 2 (MFN2), and dynamin-related protein 1 (DRP1). The mitochondrial phosphatase phosphoglycerate mutase 5 (PGAM5) protein can regulate the phosphorylation levels and the function of both MFN2 and DRP1, however, the precise regulation of PGAM5 activity is unknown. We show that PGAM5 oligomerization and localization controls its function. Under depolarization and/or metabolic stress PGAM5 changes its association from dodecamers to dimers. These PGAM5 oligomers have differential affinity towards MFN2 and DRP1. Simultaneously, PGAM5 is cleaved by the inner mitochondrial membrane resident proteases PARL and OMA1 and a fraction of the cleaved PGAM5 translocates to the cytosol. These two events play an important role in regulating mitochondrial dynamics under depolarization and/or metabolic stress. Taken together, our results identify PGAM5 oligomerization and cleavage-induced relocalization as critical regulators of its function.
    Keywords:  DRP1; Glucose-Starvation; MFN2; Mitochondrial morphology; PGAM5
    DOI:  https://doi.org/10.1242/jcs.263903
  15. FEBS Lett. 2025 Oct 10.
      Respiratory complex I (CI) is a multi-subunit membrane protein complex important for the production of ATP via the oxidative phosphorylation pathway. The structure of CI is roughly conserved across species and is composed of subunits that are either embedded in the membrane or are exposed to the aqueous environment that together form an overall L-shaped 'boot'. The conserved core of CI is generally composed of 14 subunits. Across species, various less conserved 'supernumerary' or 'accessory' subunits have been added. Accessory subunits vary in number across species and can include proteins that are unique to specific lineages. Additionally, there are structural variations in the core subunits between clades. In this Review, we compare seven representative CI structures from divergent eukaryotic lineages to identify what aspects of the CI core subunits are susceptible to variation and classify eukaryotic accessory subunits into those conserved from the last eukaryotic common ancestor (LECA) or those that are lineage specific. Impact statement Understanding the biodiversity and evolution of mitochondrial complex I will reveal patterns that may reflect metabolic niche and can be used to constrain quantitative models of molecular evolution.
    Keywords:  OXPHOS; bioenergetics; cellular respiration; complex I; cryoEM structures; evolution; last eukaryotic common ancestor; metabolism; mitochondria
    DOI:  https://doi.org/10.1002/1873-3468.70181
  16. Cell Death Dis. 2025 Oct 06. 16(1): 693
      The highly conserved CHCHD2 and CHCHD10 are small mitochondrial proteins residing in the intermembrane space. Recently, mutations in the genes encoding these proteins have been linked to severe disorders, including Parkinson's disease and amyotrophic lateral sclerosis. In cultured cells, a small fraction of CHCHD2 and CHCHD10 oligomerize to form a high molecular weight complex of unknown function. Here, we generated a whole-body Chchd2 knockout mouse to investigate the in vivo role of CHCHD2 and its protein complex. We show that CHCHD2 is crucial for sustaining full motor capacity, normal striatal dopamine levels, and lipid homeostasis in the brain of adult male mice. We also demonstrate that in mouse tissues, CHCHD2 and CHCHD10 exist exclusively as a high molecular weight complex, whose levels are finely tuned under physiological conditions. In response to mitochondrial dysfunction, the abundance and size of the CHCHD2-CHCHD10 complex increase, a mechanism conserved across different tissues. Although the loss of CHCHD2 does not abolish CHCHD10 oligomerization, it enhances cell vulnerability to mitochondrial stress, suggesting that CHCHD2 is protective against mitochondrial damage. Our findings uncover the role of CHCHD2 in preserving tissue homeostasis and provide important insights into the involvement of the CHCHD2-CHCHD10 complex in human diseases.
    DOI:  https://doi.org/10.1038/s41419-025-08030-z
  17. Neurosci Lett. 2025 Oct 06. pii: S0304-3940(25)00299-X. [Epub ahead of print]868 138410
      The crucial influence of mitochondria in ischemic stroke pathophysiology presents many unexplored yet promising avenues for therapeutic strategies and clinical outcomes. Post-stroke mitochondrial dysfunction contributes to aggravated levels of calcium overload and apoptosis. This dysfunction is signified by disruption of the mitochondrial lipids such as cardiolipin, along with mitochondrial DNA mutation, leading to an imbalance in mitophagy. Calcium overload-mediated calcineurin overexpression has been reported to exacerbate mitochondrial damage and further contribute to neuronal apoptosis. In our study, we explored the alterations in the mitochondrial function following inhibition of the calcium-mediated calcineurin levels in post-stroke condition. In a rodent model of middle cerebral artery occlusion (MCAo), we observed that the inhibition of the calcium channels in post-stroke condition led to restored neuronal histology and viability following upregulation of the antioxidant levels. At the mitochondrial level, calcium channel inhibition downregulated calcineurin activation and normalized cardiolipin concentration, mitochondrial membrane potential, and respiratory control ratio in post-stroke condition. This inhibition also balanced the mitochondrial dynamics proteins and mitophagy towards neuronal recovery following ischemic stress. Moreover, it also normalized the expression of TERT, a key marker of mitochondrial health and aging. These findings highlight the role of calcium-mediated calcineurin in influencing mitochondrial dysfunction and aging in ischemic stroke. Thus, calcium channel inhibition offers a promising therapeutic strategy by preserving mitochondrial integrity and promoting neuroprotection following stroke.
    Keywords:  Calcineurin; Calcium signaling; Cardiolipin; Mitochondrial aging; Stroke
    DOI:  https://doi.org/10.1016/j.neulet.2025.138410
  18. Cell Biol Toxicol. 2025 Oct 07. 41(1): 137
       BACKGROUND: Ischemic stroke (IS) stands as a principal contributor to high rates of sickness and death. The condition's pathological development is complicated, featuring mechanisms like mitochondrial impairment and the activation of microglial cells. A thorough grasp of these intricate processes is vital for creating successful treatment strategies.
    METHODS: We applied Weighted Gene Co-expression Network Analysis (WGCNA) to find gene sets with a strong correlation to IS. Integrated machine learning approachs were used to identify key mitochondrial-related genes (MRGs). From this analysis, SPTLC2 was identified as a pivotal MRG and was subsequently analyzed in detail using single-cell RNA sequencing (scRNA-seq) datasets. We performed functional confirmation using experimental stroke simulations, which included transient middle cerebral artery occlusion (tMCAO) in mice and in vitro oxygen-glucose deprivation/reoxygenation (OGD/R) on primary microglia.
    RESULTS: WGCNA revealed two critical modules (yellow and blue) comprising 5348 genes, which were predominantly enriched in immune response, nerve regeneration, and lipid metabolism. We exhibited the robust and superior performance of MRGs in stroke prediction, which contributed to an optimal combination of ridge regression and random forest fitted on 18 MRGs. Subsequently, elevated expression of the SPTLC2 gene was observed in microglia following stroke. Functional studies and experimental validation demonstrated that SPTLC2 promoted microglial pro-inflammatory phenotype, metabolic reprogramming towards glycolysis, and exacerbated cell-cell communication alterations. SPTLC2-specific knockdown in myeloid cells using an adeno-associated virus (AAV) in our tMCAO model alleviated neurobehavioral deficits, reduced infarct volume, and improved mitochondrial function by elevating oxidative stress and mitigating mitochondrial membrane potential depolarization. Additionally, SPTLC2 was regulated by the transcription factor FLI1, and molecular docking identified potential drugs targeting SPTLC2, including Nystatin A3, Moxidectin, and Lumacaftor.
    CONCLUSION: Our study highlights SPTLC2 as a critical mediator of microglial activation and metabolic reprogramming in ischemic stroke, providing a foundation for developing novel therapeutic strategies targeting SPTLC2 to improve stroke outcomes.
    Keywords:  Machine learning; Microglia; Mitochondria; SPTLC2; Single-cell RNA sequencing; Stroke
    DOI:  https://doi.org/10.1007/s10565-025-10085-9
  19. J Mass Spectrom. 2025 Nov;60(11): e5190
      In this study, we present the first comprehensive application of ion mobility mass spectrometry (IMS MS) combined with collision-induced dissociation (CID MS/MS) to the comparative analysis of the gangliosidome in human hippocampal tissue affected by temporal lobe epilepsy and corresponding healthy controls. Using nanoESI IMS MS, we profiled complex ganglioside mixtures extracted from the human hippocampus affected by temporal lobe epilepsy and the normal hippocampus. A total of 217 ions corresponding to 192 distinct ganglioside species were identified in the epileptic tissue, compared with 156 ions assigned to 137 species in the healthy hippocampus. The majority of these species were polysialylated and exhibited extensive structural diversity in both glycan and ceramide moieties, with important modifications including fucosylation, O-acetylation, GalNAc, and CH3COO- attachments. The gangliosidome associated with epilepsy was found characterized by a higher overall degree of sialylation than previously known, with the exclusive presence of highly sialylated GP, GS, and GO species reported here for the first time in relation to this disease. CID MS/MS experiments enabled the structural elucidation of several biologically relevant species, including O-Ac-GD1b (d18:1/20:2) and GT1b (d18:1/23:0), demonstrating the method's capacity to resolve complex structures and identify specific ganglioside isomers. Significant differences in the expression and modification patterns between pathological and control samples suggest disease-associated remodeling of membrane components. This study not only reveals novel molecular features of epilepsy-related ganglioside alterations but also establishes IMS CID MS/MS as a powerful analytical platform for advancing glycosphingolipidomics and exploring biomolecular signatures in neurological disorders.
    Keywords:  epilepsy; gangliosides; human hippocampus; structural analysis; travelling waves ion mobility mass spectrometry
    DOI:  https://doi.org/10.1002/jms.5190
  20. Alzheimers Dement. 2025 Oct;21(10): e70718
    ONDRI Investigators
       INTRODUCTION: Type 2 diabetes mellitus (T2DM) is a risk factor for dementia and cerebral small vessel disease, but there remains a need to identify targetable molecular pathways involved in the underlying pathophysiology.
    METHODS: In participants with Alzheimer's disease, related dementias, or cerebrovascular diseases, we assessed associations between ratios of unesterified linoleic acid (LA)-derived soluble epoxide hydrolase (sEH) metabolites (diols) and substrates (epoxides), with imaging-derived white matter hyperintensities (WMHs), brain parenchymal fraction (BPF), and cognitive performance. Potential moderation effects by glycemic control (hemoglobin A1c [HbA1c]) were examined.
    RESULTS: With elevated HbA1c, greater LA-derived diol/epoxide ratios were associated with greater WMH volume (β [95% CI] = 0.565 [0.100, 1.030], p = 0.017), lower global BPF (β [95% CI] = -0.476 [-0.903, -0.048], p = 0.029), and poorer memory performance (β [95% CI] = -0.603 [-1.070, -0.136], p = 0.012), such that detrimental associations were observed only in T2DM.
    DISCUSSION: Cytochrome P450-sEH metabolites may indicate a novel metabolic-vascular contribution to dementia in individuals with T2DM.
    CLINICAL TRIALS REGISTRATION INFORMATION: ClinicalTrials.gov Identifier NCT04104373.
    HIGHLIGHTS: LA-derived sEH metabolite (diol) to substrate (epoxide) ratio was lower in individuals with diabetes. The diol/epoxide ratio with high HbA1c contributed to SVD and brain atrophy. The CYP450-sEH pathway may link metabolic and vascular contributions to dementia. sEH may be a potential therapeutic target in individuals with diabetes.
    Keywords:  biomarkers; cerebral small vessel disease; cerebrovascular disease; cognition; diabetes; neurodegenerative disease; neuroimaging; oxylipins
    DOI:  https://doi.org/10.1002/alz.70718