bims-medebr Biomed News
on Metabolism of the developing brain
Issue of 2022‒02‒13
38 papers selected by
Regina F. Fernández
Johns Hopkins University


  1. Front Mol Neurosci. 2021 ;14 819440
      Astrocytic glycogen plays an important role in brain energy metabolism. However, the contribution of glycogen metabolism to stress-induced depression remains unclear. Chronic social defeat stress was used to induce depression-like behaviors in mice, assessed with behavioral tests. Glycogen concentration in the medial prefrontal cortex (mPFC) and the expression of key enzymes of the glycogen metabolism were investigated using Western blots, immunofluorescent staining, electron microscopy, and biochemical assays. Stereotaxic surgery and viral-mediated gene transfer were applied to knockdown or overexpress brain-type glycogen phosphorylase (PYGB) in the mPFC. The glycogen content increased in the mPFC after stress. Glycogenolytic dysfunction due to inactivation of PYGB was responsible for glycogen accumulation. Behavioral tests on astrocyte-specific PYGB overexpression mice showed that augmenting astrocytic PYGB reduces susceptibility to depression when compared with stress-susceptible mice. Conversely, PYGB genetic down-regulation in the mPFC was sufficient to induce glycogen accumulation and depression-like behaviors. Furthermore, PYGB overexpression in the mPFC decreases susceptibility to depression, at least partially by rescuing glycogen phosphorylase activity to maintain glycogen metabolism homeostasis during stress. These findings indicate that (1) glycogen accumulation occurs in mice following stress and (2) glycogenolysis reprogramming leads to glycogen accumulation in astrocytes and PYGB contributes to stress-induced depression-like behaviors. Pharmacological tools acting on glycogenolysis might constitute a promising therapy for depression.
    Keywords:  astrocyte; brain type glycogen phosphorylase; depression; glycogen; medial prefrontal cortex
    DOI:  https://doi.org/10.3389/fnmol.2021.819440
  2. Prog Neuropsychopharmacol Biol Psychiatry. 2022 Feb 04. pii: S0278-5846(22)00014-8. [Epub ahead of print] 110522
      Autism spectrum disorder (ASD) is a neurodevelopmental disorder of heterogenous etiology exhibiting a challenge in understanding its exact neuro-pathophysiology. Recently, peroxisome proliferator activated receptor (PPAR)-α activation was found to play a fundamental role in neuroprotection and improving autistic-like-behaviors in experimental animal models of ASD through alleviating neuroinflammation, oxidative-stress, astrocyte reactivity, tauopathy in addition to its favorable role in metabolic regulation, thus attracting attention as a possible target in treatment of ASD. This study aimed to investigate the role of PPAR-α, astrocytic dysfunction and tauopathy in ASD and detect the possible neuroprotective effects of metformin (MET), through PPAR-α activation, and risperidone (RIS) either monotherapy or in combination in alleviating autistic-like-changes at behavioral and neurobiological levels in male Wistar rats. Pregnant female Wistar rats received valproic-acid (VPA) to induce autistic-like-behavioral and neurobiological alterations in their offspring. Chronic intra-peritoneal MET (100 mg/kg/day) and RIS (1 mg/kg/day) either monotherapy or in combination started from postnatal day (PND) 24 till PND61 (38 days). Prenatal VPA exposure simulated the autistic core behaviors associated with neurochemical and histopathological neurodevelopmental degenerative changes. Both MET and RIS either monotherapy or in combination were able to reverse these changes. The effect of MET was comparable to RIS. Moreover, MET was able to alleviate the RIS induced weight gain and improve cognitive functions highlighting its promising adjunctive role in alleviating ASD pathophysiology. Our study highlighted the favorable effects of MET and RIS both in monotherapy and in combination in alleviating the autistic-like-changes and proposed PPAR-α activation along with restoring astrocytes homeostasis as promising targets in novel therapeutic strategies in ASD.
    Keywords:  ASD; Apoptosis; Astrocytes and Tauopathy; Metformin and risperidone; PPAR-α
    DOI:  https://doi.org/10.1016/j.pnpbp.2022.110522
  3. Geroscience. 2022 Feb 11.
      Fatty acids are key components in the structural diversity of lipids and play a strategic role in the functional properties of lipids which determine the integrity of neuronal and glial cell membranes, the generation of lipid signaling mediators, and the chemical reactivity of acyl chains. The present study analyzes using gas chromatography the fatty acid profiles of 13 regions of the human central nervous system in healthy individuals ranging from 40 to 80 years old. The outcomes suggest the existence of general traits in fatty acid composition such as an average chain length of 18 carbon atoms, high monounsaturated fatty acid content, and predominance in polyunsaturated fatty acids of those of series n-6 over series n-3 which are shared by all brain regions regardless of age. Our results also show a general sustained and relatively well-preserved lipid profile throughout the adult lifespan in most studied regions (olive, upper vermis, substantia nigra, thalamus, hippocampus, putamen, caudate, occipital cortex, parietal cortex, entorhinal cortex, and frontal cortex) with minor changes that are region-dependent. In contrast, of particular relevance is the involvement of the inferior temporal cortex and cingulate cortex. It is proposed that during normal human brain aging, the lipid profile is resistant to changes with age in most human brain regions to ensure cell survival and function, but some particular regions involved in specific memory domains are greatly affected.
    Keywords:  Average chain length; Cerebral cortex; Fatty acid profile; Human brain regions; Peroxidizability index; Polyunsaturated fatty acids
    DOI:  https://doi.org/10.1007/s11357-022-00527-1
  4. Aging (Albany NY). 2022 Feb 07. 14(undefined):
      In humans, aging, triggers increased plasma concentrations of triglycerides, cholesterol, low-density lipoproteins and lower capacity of high-density lipoproteins to remove cellular cholesterol. Studies in rodents showed that aging led to cholesterol accumulation in the liver and decrease in the brain with reduced cholesterol synthesis and increased levels of cholesterol 24-hydroxylase, an enzyme responsible for removing cholesterol from the brain. Liver diseases are also related to brain aging, inducing changes in cholesterol metabolism in the brain and liver of rats. It has been suggested that late onset Alzheimer's disease is associated with metabolic syndrome. Non-alcoholic fatty liver is associated with lower total brain volume in the Framingham Heart Study offspring cohort study. Furthermore, disorders of cholesterol homeostasis in the adult brain are associated with neurological diseases such as Niemann-Pick, Alzheimer, Parkinson, Huntington and epilepsy. Apolipoprotein E (apoE) is important in transporting cholesterol from astrocytes to neurons in the etiology of sporadic Alzheimer's disease, an aging-related dementia. Desmosterol and 24S-hydroxycholesterol are reduced in ApoE KO hypercholesterolemic mice. ApoE KO mice have synaptic loss, cognitive dysfunction, and elevated plasma lipid levels that can affect brain function. In contrast to cholesterol itself, there is a continuous uptake of 27- hydroxycholesterol in the brain as it crosses the blood-brain barrier and this flow can be an important link between intra- and extracerebral cholesterol homeostasis. Not surprisingly, changes in cholesterol metabolism occur simultaneously in the liver and nervous tissues and may be considered possible biomarkers of the liver and nervous system aging.
    Keywords:  24-hydroxycholesterol; aging; brain; cholesterol; liver
    DOI:  https://doi.org/10.18632/aging.203880
  5. Neural Regen Res. 2022 Sep;17(9): 1861-1866
      Astrocytes protect neurons by modulating neuronal function and survival. Astrocytes support neurons in several ways. They provide energy through the astrocyte-neuron lactate shuttle, protect neurons from excitotoxicity, and internalize neuronal lipid droplets to degrade fatty acids for neuronal metabolic and synaptic support, as well as by their high capacity for glutamate uptake and the conversion of glutamate to glutamine. A recent reported astrocyte system for protection of dopamine neurons against the neurotoxic products of dopamine, such as aminochrome and other o-quinones, were generated under neuromelanin synthesis by oxidizing dopamine catechol structure. Astrocytes secrete glutathione transferase M2-2 through exosomes that transport this enzyme into dopaminergic neurons to protect these neurons against aminochrome neurotoxicity. The role of this new astrocyte protective mechanism in Parkinson´s disease is discussed.
    Keywords:  Parkinson’s disease; aminochrome; astrocytes; dopamine; dopaminergic neurons; exosomes; glutathione transferase M2-2; neuroprotection
    DOI:  https://doi.org/10.4103/1673-5374.335690
  6. Diabetes. 2022 Feb 08. pii: db210600. [Epub ahead of print]
      The present study aimed to investigate the interactive effect between aging and type 2 diabetes mellitus (T2DM) on brain glucose metabolism, individual metabolic connectivity and network properties. A 2 × 2 factorial-designed study was conducted, 83 T2DM patients (40 elderly and 43 middle-aged) and 69 gender-matched HCs (34 elderly and 35 middle-aged) underwent an 18F fluorodeoxyglucose-positron emission tomography/magnetic resonance (18F-FDG PET/MR) scanning. The Jensen-Shannon Divergence was applied to construct individual metabolic connectivity and network. The topological properties of network were quantified with graph theoretical analysis. The general linear model (GLM) was used to mainly estimate the interaction effect between aging and T2DM on glucose metabolism, metabolic connectivity and network. There was an interaction effect between aging and T2DM on glucose metabolism, metabolic connectivity and regional metabolic network properties (all P < 0.05). The post-hoc analyses showed that compared with the elderly HCs and middle-aged with T2DM groups, elderly T2DM group had decreased glucose metabolism, increased metabolic connectivity and regional metabolic network properties in cognition-related brain regions (all P < 0.05). The age and fasting plasma glucose had negative correlations with glucose metabolism, and positive correlations with metabolic connectivity. The elderly with T2DM had glucose hypometabolism, strengthened functional integration and increased efficiency of information communication mainly located in cognition-related brain regions, above metabolic connectivity patterns changes might be compensatory changes for glucose hypometabolism.
    DOI:  https://doi.org/10.2337/db21-0600
  7. Brain. 2022 Feb 08. pii: awab310. [Epub ahead of print]
      Traumatic brain injury is an important risk factor for development of Alzheimer's disease and dementia. Unfortunately, no effective therapies are currently available for prevention and treatment of the traumatic brain injury-induced Alzheimer's disease-like neurodegenerative disease. This is largely due to our limited understanding of the mechanisms underlying traumatic brain injury-induced neuropathology. Previous studies showed that pharmacological inhibition of monoacylglycerol lipase, a key enzyme degrading the endocannabinoid 2-arachidonoylglycerol, attenuates traumatic brain injury-induced neuropathology. However, the mechanism responsible for the neuroprotective effects produced by inhibition of monoacylglycerol lipase in traumatic brain injury remains unclear. Here we first show that genetic deletion of monoacylglycerol lipase reduces neuropathology and averts synaptic and cognitive declines in mice exposed to repeated mild closed head injury. Surprisingly, these neuroprotective effects result primarily from inhibition of 2-arachidonoylglycerol metabolism in astrocytes, rather than in neurons. Single-cell RNA-sequencing data reveal that astrocytic monoacylglycerol lipase knockout mice display greater resilience to traumatic brain injury-induced changes in expression of genes associated with inflammation or maintenance of brain homeostasis in astrocytes and microglia. The monoacylglycerol lipase inactivation-produced neuroprotection is abrogated by deletion of the cannabinoid receptor-1 or by adeno-associated virus vector-mediated silencing of astrocytic peroxisome proliferator-activated receptor-γ. This is further supported by the fact that overexpression of peroxisome proliferator-activated receptor-γ in astrocytes prevents traumatic brain injury-induced neuropathology and impairments in spatial learning and memory. Our results reveal a previously undefined cell type-specific role of 2-arachidonoylglycerol metabolism and signalling pathways in traumatic brain injury-induced neuropathology, suggesting that enhanced 2-arachidonoylglycerol signalling in astrocytes is responsible for the monoacylglycerol lipase inactivation-produced alleviation of neuropathology and deficits in synaptic and cognitive functions in traumatic brain injury.
    Keywords:  2-arachidonoylglycerol; Alzheimer’s disease; monoacylglycerol lipase; neurodegeneration; peroxisome proliferator-activated receptor-γ
    DOI:  https://doi.org/10.1093/brain/awab310
  8. Alzheimers Dement (N Y). 2022 ;8(1): e12241
      Introduction: Although mouse models of Alzheimer's disease (AD) have increased our understanding of the molecular basis of the disease, none of those models represent late-onset Alzheimer's Disease which accounts for >90% of AD cases, and no therapeutics developed in the mouse (with the possible exceptions of aduhelm/aducanumab and gantenerumab) have succeeded in preventing or reversing the disease. This technology has allowed much progress in understanding the molecular basis of AD. To further enhance our understanding, we used wild-type rabbit (with a nearly identical amino acid sequence for amyloid as in humans) to model LOAD by stressing risk factors including age, hypercholesterolemia, and elevated blood glucose levels (BGLs), upon an ε3-like isoform of apolipoprotein. We report a combined behavioral, imaging, and metabolic study using rabbit as a non-transgenic model to examine effects of AD-related risk factors on cognition, intrinsic functional connectivity, and magnetic resonance-based biomarkers of neuropathology.Methods: Aging rabbits were fed a diet enriched with either 2% cholesterol or 10% fat/30% fructose. Monthly tests of novel object recognition (NOR) and object location memory (OLM) were administered to track cognitive impairment. Trace eyeblink conditioning (EBC) was administered as a final test of cognitive impairment. Magnetic resonance imaging (MRI) was used to obtain resting state connectivity and quantitative parametric data (R2*).
    Results: Experimental diets induced hypercholesterolemia or elevated BGL. Both experimental diets induced statistically significant impairment of OLM (but not NOR) and altered intrinsic functional connectivity. EBC was more impaired by fat/fructose diet than by cholesterol. Whole brain and regional R2* MRI values were elevated in both experimental diet groups relative to rabbits on the control diet.
    Discussion: We propose that mechanisms underlying LOAD can be assessed by stressing risk factors for inducing AD and that dietary manipulations can be used to assess etiological differences in the pathologies and effectiveness of potential therapeutics against LOAD. In addition, non-invasive MRI in awake, non-anesthetized rabbits further increases the translational value of this non-transgenic model to study AD.
    Keywords:  cholesterol; diabetes; eyeblink conditioning; late onset Alzheimer's disease; magnetic resonance imaging; whole‐brain R2*
    DOI:  https://doi.org/10.1002/trc2.12241
  9. Food Funct. 2022 Feb 11.
      Acer truncatum Bunge seed oil (ASO) is rich in ω-9 (53.93%) and ω-6 (30.7%) fatty acids (FAs) and characterized by 3-7% nervonic acid (NA, C24:1ω-9). Evidence suggests that ω-9 FAs such as NA participate in processes of cognitive improvement; however, their mechanism remains ambiguous. In this study, we investigated the effect of ASO on rat memory and the change in lipid profiling and underlying metabolism. After ASO was administrated to rats for one, three and seven days, their capacity for learning and memory significantly increased via the MWM test. Lipid profiling showed alterations in a wide range of metabolic features after ASO was administrated to the rats, in which sphingolipids (SP) in the serum and glycerophospholipids (GP) in the brain were regulated significantly. The changes in the fatty acids in the serum and brain showed the synergetic effects of NA, EA, OA and DHA, where NA, EA and OA exhibited similar change trends. The enrichment analysis based on KEGG indicated that ASO supplementation evoked the pathways of neurotrophin signaling, glycerophospholipid metabolism and sphingolipid metabolism, which are related to memory and cognition improvement. Among the metabolites with different molecular forms, the biomarkers with C24:1ω-9 chains exhibited a positive correlation with others both in the serum SP and brain GP. These results suggest the synergistic effects of ω-9 FAs and that their conversion into each other may result in enhanced cognition in rats ingesting Acer truncatum Bunge seed oil.
    DOI:  https://doi.org/10.1039/d1fo03671h
  10. Mol Cell Neurosci. 2022 Feb 04. pii: S1044-7431(22)00010-0. [Epub ahead of print] 103704
      In the central nervous system (CNS), many neurons develop axonal arbors that are crucial for information processing. Previous studies have demonstrated that premature axons contain motile and stationary mitochondria, and their balance is important for axonal arborization. However, the mechanisms by which neurons determine the positions of stationary mitochondria as well as their turnover remain to be elucidated. We observed that the distribution of stationary mitochondrial spots along the unmyelinated and nonsynaptic axons is not random but rather relatively uniform both in primary cultured neurons and in tissues. Intriguingly, whereas the positions of each mitochondrial spot changed over time, the overall distribution remained uniform. In addition, local inactivation of mitochondria by KillerRed mediated chromophore-assisted light inactivation (CALI) inhibited the translocation of mitochondrial spots in adjacent axonal regions, suggesting that functional mitochondria enhance the motility of other mitochondria in the vicinity. Signals of ATP:ADP sensor, PercevalHR indicated that the ATP:ADP ratio was relatively high around mitochondria, and treating axons with phosphocreatine (PCr), which supplies ATP, reduced the immobile mitochondria induced by the local mitochondrial inactivation. In a mathematical model, we found that the ATP gradient generated by mitochondria, and ATP dependent regulation of mitochondrial motility could establish uniform mitochondrial distribution. These observations suggest that axons in the CNS possess the system that distributes mitochondria uniformly, and intermitochondrial signaling contribute to the regulation. In addition, our results suggest the possibility that ATP might be one of the molecules mediating the signaling.
    Keywords:  ATP; Axonal transport; Cerebellar granule neurons; Mitochondrial distribution; Retinal ganglion cells; Stationary mitochondria
    DOI:  https://doi.org/10.1016/j.mcn.2022.103704
  11. Neuroreport. 2022 Feb 02. 33(3): 109-115
      OBJECTIVES: Epidemiological research has indicated that hyperuricemia may impair cognitive ability; however, the underlying mechanisms remain unclear. The present study thus investigated the possible mechanism underlying hyperuricemia-related cognitive impairment.METHODS: Using hyperuricemic rats and high uric acid (UA) intracerebroventricularly treated mice, the current study elucidated whether and how high UA impaired cognitive ability and hippocampal mitochondrial bioenergetic function.
    RESULTS: Hyperuricemia induced UA uptake by hippocampal mitochondria, which impaired cognitive ability and disrupted the bioenergetic function of hippocampal mitochondria, indicated by reduced ATP production and decreased cytochrome c oxidase (COX) activity. Mechanistically, excess UA might trigger intramitochondrial NF-κB inhibitor α (IκBα)/nuclear factor-κB (NF-κB) pathway to downregulate the subunit III of COX (COXIII).
    CONCLUSION: The results provided new insights into the mechanism underlying hyperuricemia-related cognitive decline.
    DOI:  https://doi.org/10.1097/WNR.0000000000001762
  12. Oxid Med Cell Longev. 2022 ;2022 3255296
      Near-term acute hypoxia in utero can result in significant fetal brain injury, with some brain regions more vulnerable than others. As mitochondrial dysfunction is an underlying feature of the injury cascade following hypoxia, this study is aimed at characterizing mitochondrial function at a region-specific level in the near-term fetal brain after a period of acute hypoxia. We hypothesized that regional differences in mitochondrial function would be evident, and that prophylactic creatine treatment would mitigate mitochondrial dysfunction following hypoxia; thereby reducing fetal brain injury. Pregnant Border-Leicester/Merino ewes with singleton fetuses were surgically instrumented at 118 days of gestation (dGa; term is ~145 dGA). A continuous infusion of either creatine (n = 15; 6 mg/kg/h) or isovolumetric saline (n = 16; 1.5 ml/kg/h) was administered to the fetuses from 121 dGa. After 10 days of infusion, a subset of fetuses (8 saline-, 7 creatine-treated) were subjected to 10 minutes of umbilical cord occlusion (UCO) to induce a mild global fetal hypoxia. At 72 hours after UCO, the fetal brain was collected for high-resolution mitochondrial respirometry and molecular and histological analyses. The results show that the transient UCO-induced acute hypoxia impaired mitochondrial function in the hippocampus and the periventricular white matter and increased the incidence of cell death in the hippocampus. Creatine treatment did not rectify the changes in mitochondrial respiration associated with hypoxia, but there was a negative relationship between cell death and creatine content following treatment. Irrespective of UCO, creatine increased the proportion of cytochrome c bound to the inner mitochondrial membrane, upregulated the mRNA expression of the antiapoptotic gene Bcl2, and of PCG1-α, a driver of mitogenesis, in the hippocampus. We conclude that creatine treatment prior to brief, acute hypoxia does not fundamentally modify mitochondrial respiratory function, but may improve mitochondrial structural integrity and potentially increase mitogenesis and activity of antiapoptotic pathways.
    DOI:  https://doi.org/10.1155/2022/3255296
  13. J Neurosci. 2022 Feb 07. pii: JN-RM-1459-21. [Epub ahead of print]
      Mutations in the AASS (Aminoadipate-Semialdehyde Synthase) gene encoding α-aminoadipic semialdehyde synthase lead to hyperlysinemia-I, a benign metabolic variant without clinical significance, and hyperlysinemia-II with developmental delay and intellectual disability. Although both forms of hyperlysinemia display biochemical phenotypes of questionable clinical significance, an association between neurological disorder and a pronounced biochemical abnormality remains a challenging clinical question. Here we report that Aass mutant male and female mice carrying the R65Q mutation in α-ketoglutarate reductase (LKR) domain have an elevated cerebral lysine level and a normal brain development, whereas the Aass mutant mice carrying the G489E mutation in saccharopine dehydrogenase (SDH) domain exhibit elevations of both cerebral lysine and saccharopine levels and a smaller brain with defective neuronal development. Mechanistically, the accumulated saccharopine, but not lysine, leads to impaired neuronal development by inhibiting the neurotrophic effect of glucose-6-phosphate isomerase (GPI). While extracellular supplementation of GPI restores defective neuronal development caused by G498E mutation in SDH of Aass. Altogether, our findings not only unravel the requirement for saccharopine degradation in neuronal development, but also provide the mechanistic insights for understanding the neurometabolic disorder of hyperlysinemia-II.SIGNIFICANCE STATEMENTThe association between neurological disorder and a pronounced biochemical abnormality in hyperlysinemia remains a challenging clinical question. Here, we report that mice carrying the R65Q mutation in LKR domain of AASS have an elevated cerebral lysine levels and a normal brain development, whereas those carrying the G489E mutation in SDH domain of AASS exhibit an elevation of both cerebral lysine and saccharopine and a small brain with defective neuronal development. Furthermore, saccharopine impairs neuronal development by inhibiting the neurotrophic effect of glucose-6-phosphate isomerase. These findings demonstrate saccharopine degradation is essential for neuronal development.
    DOI:  https://doi.org/10.1523/JNEUROSCI.1459-21.2022
  14. Front Physiol. 2021 ;12 787848
      During pregnancy, maternal plasma fatty acids are critically required for cell growth and development, cell signaling, and the development of critical structural and functional aspects of the feto-placental unit. In addition, the fatty acids modulate the early stages of placental development by regulating angiogenesis in the first-trimester human placenta. Preferential transport of maternal plasma long-chain polyunsaturated fatty acids during the third trimester is critical for optimal fetal brain development. Maternal status such as obesity, diabetes, and dietary intakes may affect the functional changes in lipid metabolic processes in maternal-fetal lipid transport and metabolism. Fatty acids traverse the placental membranes via several plasma membrane fatty acid transport/binding proteins (FAT, FATP, p-FABPpm, and FFARs) and cytoplasmic fatty acid-binding proteins (FABPs). This review discusses the maternal metabolism of fatty acids and their effects on early placentation, placental fatty acid transport and metabolism, and their roles in feto-placental growth and development. The review also highlights how maternal fat metabolism modulates lipid processing, including transportation, esterification, and oxidation of fatty acids.
    Keywords:  FABP; FATP; angiogenesis; arachidonic acid; docosahexaenoic acid; fetal development; fetus; placenta
    DOI:  https://doi.org/10.3389/fphys.2021.787848
  15. iScience. 2022 Feb 18. 25(2): 103771
      Huntington disease (HD) is a neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene. Metabolic changes are associated with HD progression, but underlying mechanisms are not fully known. As the IKKβ/NF-κB pathway is an essential regulator of metabolism, we investigated the involvement of IKKβ, the upstream activator of NF-κB in hypothalamus-specific HD metabolic changes. We expressed amyloidogenic N-terminal fragments of mutant HTT (mHTT) in the hypothalamus of mice with brain-specific ablation of IKKβ (Nestin/IKKβlox/lox) and control mice (IKKβlox/lox). We assessed effects on body weight, metabolic hormones, and hypothalamic neuropathology. Hypothalamic expression of mHTT led to an obese phenotype only in female mice. CNS-specific inactivation of IKKβ prohibited weight gain in females, which was independent of neuroprotection and microglial activation. Our study suggests that mHTT in the hypothalamus causes metabolic imbalance in a sex-specific fashion, and central inhibition of the IKKβ pathway attenuates the obese phenotype.
    Keywords:  Human metabolism; Model organism; Molecular neuroscience
    DOI:  https://doi.org/10.1016/j.isci.2022.103771
  16. Complex Psychiatry. 2021 Aug;7(1-2): 19-33
      The kynurenine pathway (KP) is a major route for L-tryptophan (L-TRP) metabolism, yielding a variety of bioactive compounds including kynurenic acid (KYNA), 3-hydroxykynurenine (3-HK), quinolinic acid (QUIN), and picolinic acid (PIC). These tryptophan catabolites are involved in the pathogenesis of many neuropsychiatric disorders, particularly when the KP becomes dysregulated. Accordingly, the enzymes that regulate the KP such as indoleamine 2,3-dioxygenase (IDO)/tryptophan 2,3-dioxygenase, kynurenine aminotransferases (KATs), and kynurenine 3-monooxygenase (KMO) represent potential drug targets as enzymatic inhibition can favorably rebalance KP metabolite concentrations. In addition, the galantamine-memantine combination, through its modulatory effects at the alpha7 nicotinic acetylcholine receptors and N-methyl-D-aspartate receptors, may counteract the effects of KYNA. The aim of this review is to highlight the effectiveness of IDO-1, KAT II, and KMO inhibitors, as well as the galantamine-memantine combination in the modulation of different KP metabolites. KAT II inhibitors are capable of decreasing the KYNA levels in the rat brain by a maximum of 80%. KMO inhibitors effectively reduce the central nervous system (CNS) levels of 3-HK, while markedly boosting the brain concentration of KYNA. Emerging data suggest that the galantamine-memantine combination also lowers L-TRP, kynurenine, KYNA, and PIC levels in humans. Presently, there are only 2 pathophysiological mechanisms (cholinergic and glutamatergic) that are FDA approved for the treatment of cognitive dysfunction for which purpose the galantamine-memantine combination has been designed for clinical use against Alzheimer's disease. The alpha7 nicotinic-NMDA hypothesis targeted by the galantamine-memantine combination has been implicated in the pathophysiology of various CNS diseases. Similarly, KYNA is well capable of modulating the neuropathophysiology of these disorders. This is known as the KYNA-centric hypothesis, which may be implicated in the management of certain neuropsychiatric conditions. In line with this hypothesis, KYNA may be considered as the "conductor of the orchestra" for the major pathophysiological mechanisms underlying CNS disorders. Therefore, there is great opportunity to further explore and compare the biological effects of these therapeutic modalities in animal models with a special focus on their effects on KP metabolites in the CNS and with the ultimate goal of progressing to clinical trials for many neuropsychiatric diseases.
    Keywords:  Enzyme inhibitors; Galantamine; Kynurenic acid; Kynurenine; Memantine; Neuropsychiatric diseases
    DOI:  https://doi.org/10.1159/000515066
  17. Neurobiol Dis. 2022 Feb 02. pii: S0969-9961(22)00040-7. [Epub ahead of print]165 105649
      BACKGROUND: PLA2G6-Associated Neurodegeneration (PLAN) is a rare neurodegenerative disease with autosomal recessive inheritance, which belongs to the NBIA (Neurodegeneration with Brain Iron Accumulation) group. Although the pathogenesis of the disease remains largely unclear, lipid peroxidation seems to play a central role in the pathogenesis. Currently, there is no cure for the disease.OBJECTIVE: In this work, we examined the presence of lipid peroxidation, iron accumulation and mitochondrial dysfunction in two cellular models of PLAN, patients-derived fibroblasts and induced neurons, and assessed the effects of α-tocopherol (vitamin E) in correcting the pathophysiological alterations in PLAN cell cultures.
    METHODS: Pathophysiological alterations were examined in fibroblasts and induced neurons generated by direct reprograming. Iron and lipofuscin accumulation were assessed using light and electron microscopy, as well as biochemical analysis techniques. Reactive Oxygen species production, lipid peroxidation and mitochondrial dysfunction were measured using specific fluorescent probes analysed by fluorescence microscopy and flow cytometry.
    RESULTS: PLAN fibroblasts and induced neurons clearly showed increased lipid peroxidation, iron accumulation and altered mitochondrial membrane potential. All these pathological features were reverted with vitamin E treatment.
    CONCLUSIONS: PLAN fibroblasts and induced neurons reproduce the main pathological alterations of the disease and provide useful tools for disease modelling. The main pathological alterations were corrected by Vitamin E supplementation in both models, suggesting that blocking lipid peroxidation progression is a critical therapeutic target.
    Keywords:  Iron accumulation; Lipid peroxidation; Lipofuscin; Mitochondria; NBIA; PLA2G6-Associated Neurodegeneration; Vitamin E
    DOI:  https://doi.org/10.1016/j.nbd.2022.105649
  18. Free Radic Biol Med. 2022 Feb 02. pii: S0891-5849(22)00042-9. [Epub ahead of print]181 72-81
      Mitochondria damage and apoptosis were found associated with sevoflurane induced neurotoxicity in developing brains of rodent and neuro cell lines. The detailed upstream mechanism remains unclear. This study explored whether sevoflurane induces neurotoxicity by activating a GSK3β (glycogen synthase kinase 3β)/Drp1 (dynamin-related protein-1)-dependent mitochondrial fission and apoptosis. Our results showed that sevoflurane exposure promoted mitochondria fission in hippocampus of neonatal mice, resulted in a prolonged escape latency from P32 (32-day-postnatal) to P35, and decreased platform crossing times on P36 as compared to the control treatment. Additionally, sevoflurane upregulated GSK3β stability and activation, promoted phosphorylation of Drp1 at Ser616 along with its translocation to mitochondria and resulted in increasing cytochrome c and cleaved casepase-3 in hippocampus of neonatal mice and in human SK-N-SH cells. Simultaneously, sevoflurane promoted the interaction between Drp1 and GSK3β. Furthermore, GSK3β activated phosphorylation of Drp1 at Ser616, induced mitochondrial fission, loss of mitochondrial membrane potential (MMP) and apoptosis in SK-N-SH cells, which was attenuated by TDZD-8, an inhibitor of GSK3β. In conclusion, sevoflurane induced neurotoxicity links to a GSK3β/Drp1 dependent mitochondrial fission and apoptosis.
    Keywords:  Apoptosis; Drp1 protein; Glycogen Synthase Kinase 3 beta; Mitochondria; Mitochondrial fission; Neurotoxicity; Sevoflurane
    DOI:  https://doi.org/10.1016/j.freeradbiomed.2022.01.031
  19. Front Aging Neurosci. 2021 ;13 796067
      Introduction: Integrating brain imaging with large scale omics data may identify novel mechanisms of mild cognitive impairment (MCI) and early Alzheimer's disease (AD). We integrated and analyzed brain magnetic resonance imaging (MRI) with cerebrospinal fluid (CSF) metabolomics to elucidate metabolic mechanisms and create a "metabolic map" of the brain in prodromal AD.Methods: In 145 subjects (85 cognitively normal controls and 60 with MCI), we derived voxel-wise gray matter volume via whole-brain structural MRI and conducted high-resolution untargeted metabolomics on CSF. Using a data-driven approach consisting of partial least squares discriminant analysis, a multiomics network clustering algorithm, and metabolic pathway analysis, we described dysregulated metabolic pathways in CSF mapped to brain regions associated with MCI in our cohort.
    Results: The multiomics network algorithm clustered metabolites with contiguous imaging voxels into seven distinct communities corresponding to the following brain regions: hippocampus/parahippocampal gyrus (three distinct clusters), thalamus, posterior thalamus, parietal cortex, and occipital lobe. Metabolic pathway analysis indicated dysregulated metabolic activity in the urea cycle, and many amino acids (arginine, histidine, lysine, glycine, tryptophan, methionine, valine, glutamate, beta-alanine, and purine) was significantly associated with those regions (P < 0.05).
    Conclusion: By integrating CSF metabolomics data with structural MRI data, we linked specific AD-susceptible brain regions to disrupted metabolic pathways involving nitrogen excretion and amino acid metabolism critical for cognitive function. Our findings and analytical approach may extend drug and biomarker research toward more multiomics approaches.
    Keywords:  Alzheimer’s disease; MRI imaging; amino acids (AA); gray matter atrophy; integrative “omics,”; metabolomics (OMICS); mild cognition impairment; multiomics analysis
    DOI:  https://doi.org/10.3389/fnagi.2021.796067
  20. J Recept Signal Transduct Res. 2022 Feb 08. 1-9
      Bipolar disorder (BD) is a severe mental illness characterized by aberrant mood changes between hypomania and mania or mixed states and depression. Metabolic changes also accompany disease progression and cause significant morbidity. Symptomatic treatment options are available, but asymptomatic patients and poor drug responders are significant problems. Based on the most common pharmacological agent that is used in the treatment, lithium and its main mechanisms of action, oxidative stress, and glycogen synthase kinase-3β (GSK-3β) signaling are extensively investigated. However, knowledge about the effects of compounds that positively affect oxidative stress and GSK-3β signaling, such as glucagon-like peptide-1 (GLP-1) mimetics, liraglutide, is still missing. Therefore, in this study, we aimed to investigate the effects of liraglutide on the ouabain-induced bipolar disease model in rats. After intracerebroventricular single dose ouabain administration, animals were treated with 100, 200, and 400 µg/kg liraglutide (s.c.) and valproic acid (200 mg/kg, i.p.) for 10 d. The locomotion and depressive states of animals were assessed by an open field, forced swimming test, and sucrose preference tests. Serum total antioxidant (TAS) and oxidant states (TOS) and glutathione, malonyl dialdehyde (MDA) levels in the brain tissue were determined. GSK-3β phosphorylation was evaluated by western blotting. Our results demonstrated that liraglutide attenuated ouabain-induced hyperlocomotion and depressive state. Additionally, liraglutide prevented oxidative stress after ouabain administration. Decreased GSK-3β phosphorylation due to the ouabain insult was alleviated by liraglutide treatment. These findings indicate that the manic and depressive-like behaviors are ameliorated by liraglutide, which exerted antioxidant action, possibly improving GSK-3β phosphorylation.
    Keywords:  GSK-3β; Ouabain-induced bipolar disorder model; bipolar disease; liraglutide; oxidative stress
    DOI:  https://doi.org/10.1080/10799893.2022.2032747
  21. Neuroimage. 2022 Feb 08. pii: S1053-8119(22)00113-6. [Epub ahead of print] 118984
      Glutamate is the amino acid with the highest cerebral concentration. It plays a central role in brain metabolism. It is also the principal excitatory neurotransmitter in the brain and is involved in multiple cognitive functions. Alterations of the glutamatergic system may contribute to the pathophysiology of many neurological disorders. For example, changes of glutamate availability are reported in rodents and humans during Alzheimer's and Huntington's diseases, epilepsy as well as during aging. Most studies evaluating cerebral glutamate have used invasive or spectroscopy approaches focusing on specific brain areas. Chemical Exchange Saturation Transfer imaging of glutamate (gluCEST) is a recently developed imaging technique that can be used to study relative changes in glutamate distribution in the entire brain with higher sensitivity and at higher resolution than previous techniques. It thus has strong potential clinical applications to assess glutamate changes in the brain. High field is a key condition to perform gluCEST images with a meaningful signal to noise ratio. Thus, even if some studies started to evaluate gluCEST in humans, most studies focused on rodent models that can be imaged at high magnetic field. In particular, systematic characterization of gluCEST contrast distribution throughout the whole brain has never been performed in humans or non-human primates. Here, we characterized for the first time the distribution of the gluCEST contrast in the whole brain and in large-scale networks of mouse lemur primates at 11.7 Tesla. Because of its small size, this primate can be imaged in high magnetic field systems. It is widely studied as a model of cerebral aging or Alzheimer's disease. We observed high gluCEST contrast in cerebral regions such as the nucleus accumbens, septum, basal forebrain, cortical areas 24 and 25. Age-related alterations of this biomarker were detected in the nucleus accumbens, septum, basal forebrain, globus pallidus, hypophysis, cortical areas 24, 21, 6 and in olfactory bulbs. An age-related gluCEST contrast decrease was also detected in specific neuronal networks, such as fronto-temporal and evaluative limbic networks. These results outline regional differences of gluCEST contrast and strengthen its potential to provide new biomarkers of cerebral function in primates.
    Keywords:  Aging; Cerebral network; GluCEST; Glutamate; mouse lemur
    DOI:  https://doi.org/10.1016/j.neuroimage.2022.118984
  22. Neuroreport. 2022 Mar 02. 33(4): 173-179
      OBJECTIVES: Oxidative stress produces neurotoxicity and has been associated with disorders of the nervous system. We observed the neuroprotective effects of N-acetylcysteine amide (NACA) against kainic acid (KA)-induced oxidative stress in aging organotypic hippocampal slice cultures (OHSCs).MATERIALS AND METHODS: We used 6-8-day-old rats for long-term cultured OHSCs (9 w). Cultured slices were injured by KA (5 µM) treatment for 18 h. OHSCs were treated with NACA dose-dependently in a medium for 24 h after KA treatment. The effects of NACA treatment were observed with propidium iodide (PI) uptake, western blotting, and optical imaging.
    RESULTS: Neuronal cell death, as assessed by PI uptake, was dose-dependently reduced by NACA treatment. Western blot analysis revealed that the 1 mM NACA-treated group exhibited significantly increased expression of superoxide dismutase compared with the KA-only group. In addition, NACA activated nuclear factor erythroid 2-related factor 2-dependent anti-inflammation signaling, which is well known to affect reactive oxygen species. Optical imaging revealed that NACA treatment reduced the latency and increased amplitude of the optical signals, which shows that synaptic activity and strength are associated with neuronal survival.
    CONCLUSION: Therefore, the neurons that survived due to the neuroprotective effects of NACA also showed enhanced functional activity in long-term cultured OHSCs using electrophysiological and biochemical assays.
    DOI:  https://doi.org/10.1097/WNR.0000000000001767
  23. Cell Biochem Biophys. 2022 Feb 11.
      Iron accumulation is an important cause of various brain diseases. As a ferroxidase, ceruloplasmin (Cp) plays a key role in iron homeostasis and its abnormal activity leads to iron accumulation. However, the detailed biological function of Cp in brain iron homeostasis needs to be investigated. In this study, Cp knockout mice were prepared and the changes in iron content and protein expression related to iron metabolism were detected. The results showed that iron accumulation occurred in multiple tissues and organs of Cp knockout mice, but there was no obvious change in brain tissues. However, Cp deficiency affected the expression of many iron metabolism-related proteins in midbrain, such as DMT1+IRE, heavy chain ferritin (H-ferritin) and light chain ferritin (L-ferritin). Cp deficiency also impaired the behavioral ability of mice, including weakened exercise ability and reduced motor coordination. In vitro cell experiment indicated that the sensitivity of Cp knockout neuron and astrocyte to hypoxia was higher than that of wild type, which means Cp deficiency leads to the damage of cell self-protection. All these results confirm that Cp exerts a protective effect on the brain by regulating iron metabolism.
    Keywords:  Brain; Ceruloplasmin; Iron metabolism; Iron metabolism-related protein
    DOI:  https://doi.org/10.1007/s12013-022-01061-9
  24. ACS Chem Neurosci. 2022 Feb 11.
      Excitatory neurotoxicity caused by the accumulation of glutamate in the synaptic cleft is an important cause of Parkinson's disease (PD). Astrocyte glutamate transporter 1 (GLT-1) is the main transporter responsible for transporting glutamate, and investigations toward the regulation of GLT-1 in astrocytes can reveal important insights. Vitamin C (VC) has important protective effects on the brain, but its effect on the regulation of GLT-1 expression is unclear. The purpose of this study was to explore any regulatory effect of VC on GLT-1 expression in astrocytes and to clarify the possible mechanism of such regulation. We found that GLT-1 expression was impaired in 1-methyl-4-phenylpyridinium iodide (MPP+)-treated astrocytes, and the transport capacity for glutamate was significantly reduced. Pretreatment with VC restored the GLT-1 expression in the MPP+-treated astrocytes. Intraperitoneal VC administration in a PD murine model confirmed that GLT-1 expression was restored in midbrain tissue. The VC-dependent rescue of GLT-1 expression in the MPP+-treated astrocytes was shown to be due to inhibition of GLT-1 ubiquitination. Transcriptome sequence analysis revealed a number of differentially expressed genes as a result of VC treatment on MPP+-treated astrocytes, including the downregulation of HECT Domain E3 ubiquitin protein ligase 1 (Hectd1). After knocking down Hectd1, the impaired GLT-1 expression caused by MPP+ was alleviated, while overexpression of Hectd1 significantly reduced the expression of GLT-1. After overexpression of Hectd1, VC could no longer increase GLT-1 expression of MPP+-treated astrocytes, indicating that HECTD1 is essential for VC regulation of GLT-1. Thus, VC reduces the ubiquitination of GLT-1 in astrocytes by inhibiting the expression of HECTD1. Our findings have identified a novel mechanism by which VC regulates the expression of GLT-1 in astrocytes.
    Keywords:  HECTD1; Parkinson’s disease; glutamate transporter-1; ubiquitination; vitamin C
    DOI:  https://doi.org/10.1021/acschemneuro.1c00845
  25. Nat Commun. 2022 Feb 08. 13(1): 753
      Presynaptic glutamate replenishment is fundamental to brain function. In high activity regimes, such as epileptic episodes, this process is thought to rely on the glutamate-glutamine cycle between neurons and astrocytes. However the presence of an astroglial glutamine supply, as well as its functional relevance in vivo in the healthy brain remain controversial, partly due to a lack of tools that can directly examine glutamine transfer. Here, we generated a fluorescent probe that tracks glutamine in live cells, which provides direct visual evidence of an activity-dependent glutamine supply from astroglial networks to presynaptic structures under physiological conditions. This mobilization is mediated by connexin43, an astroglial protein with both gap-junction and hemichannel functions, and is essential for synaptic transmission and object recognition memory. Our findings uncover an indispensable recruitment of astroglial glutamine in physiological synaptic activity and memory via an unconventional pathway, thus providing an astrocyte basis for cognitive processes.
    DOI:  https://doi.org/10.1038/s41467-022-28331-7
  26. Brain. 2022 Feb 03. pii: awac037. [Epub ahead of print]
      Metabolism regulates neuronal activity and modulates the occurrence of epileptic seizures. Here, using two rodent models of absence epilepsy, we show that hypoglycemia increases the occurrence of spike-wave seizures. We then show that selectively disrupting glycolysis in the thalamus, a structure implicated in absence epilepsy, is sufficient to increase spike-wave seizures. We propose that activation of thalamic AMP-activated protein kinase (AMPK), a sensor of cellular energetic stress and potentiator of metabotropic GABAB receptor function, is a significant driver of hypoglycemia-induced spike-wave seizures. We show that AMPK augments postsynaptic GABAB receptor-mediated currents in thalamocortical neurons and strengthens epileptiform network activity evoked in thalamic brain slices. Selective thalamic AMPK activation also increases spike-wave seizures. Finally, systemic administration of metformin, an AMPK agonist and common diabetes treatment, profoundly increased spike-wave seizures. These results advance the decades-old observation that glucose metabolism regulates thalamocortical circuit excitability by demonstrating that AMPK and GABAB receptor cooperativity is sufficient to provoke spike-wave seizures.
    Keywords:  AMPK; GABA; epilepsy; metabolism; thalamocortical
    DOI:  https://doi.org/10.1093/brain/awac037
  27. Neuropharmacology. 2022 Feb 07. pii: S0028-3908(22)00042-9. [Epub ahead of print] 108983
      In experimental models, hypothalamic dysfunction is a key component of the pathophysiology of diet-induced obesity. Early after the introduction of a high-fat diet, neurons, microglia, astrocytes and tanycytes of the mediobasal hypothalamus undergo structural and functional changes that impact caloric intake, energy expenditure and systemic glucose tolerance. Inflammation has emerged as a central component of this response, and as in other inflammatory conditions, there is a time course of events that determine the fate of distinct cells involved in the central regulation of whole-body energy homeostasis. Here, we review the work that identified key mechanisms, cellular players and temporal features of diet-induced hypothalamic abnormalities.
    Keywords:  Astrocyte; Inflammation; Microglia; Proopiomelanocortin; Tanycyte
    DOI:  https://doi.org/10.1016/j.neuropharm.2022.108983
  28. Autophagy. 2022 Feb 07. 1-3
      Failed recognition and clearance of damaged mitochondria contributes to memory loss as well as Aβ and MAPT/Tau pathologies in Alzheimer disease (AD), for which there is an unmet therapeutic need. Restoring mitophagy to eliminate damaged mitochondria could abrogate metabolic dysfunction, neurodegeneration and may subsequently inhibit or slow down cognitive decline in AD models. We have developed a high-throughput machine-learning approach combined with a cross-species screening platform to discover novel mitophagy-inducing compounds from a natural product library and further experimentally validated the potential candidates. Two lead compounds, kaempferol and rhapontigenin, induce neuronal mitophagy and reduce Aβ and MAPT/Tau pathologies in a PINK1-dependent manner in both C. elegans and mouse models of AD. Our combinational approach provides a fast, cost-effective, and highly accurate method for identification of potent mitophagy inducers to maintain brain health.
    Keywords:  Aging; Alzheimer’s disease; autophagy; machine learning; mitophagy
    DOI:  https://doi.org/10.1080/15548627.2022.2031382
  29. Neurochem Int. 2022 Feb 05. pii: S0197-0186(22)00023-7. [Epub ahead of print]154 105298
      Disrupted mitochondrial fission/fusion balance is consistently involved in neurodegenerative diseases, including Alzheimer's disease. PTEN-induced putative kinase 1 (PINK1), a mitochondrial kinase, has been reported to prevent mitochondrial injury, oxidative stress, apoptosis, and inflammation. However, to the best of our knowledge, the contribution of PINK1 to Aβ-induced mitochondrial fission/fusion has not been reported. In the present study, we showed that PINK1 deficiency promoted mitochondrial fission and fusion, aggravated mitochondrial dysfunction, and promoted neuroinflammatory cytokine factor production induced by intracerebroventricular (ICV) injection of Aβ25-35 in rats. In vitro experiments have also showed that Aβ25-35 caused more severe cell injury in PINK1-knockdown PC12 cells. These cells suffered more extensive death when exposed to proinflammatory cytokines. Lastly, we found that PINK1 overexpression significantly inhibited mitochondrial fusion, improved mitochondrial dysfunction, and reduced neuroinflammatory cytokine production induced by Aβ25-35. The current study suggests the involvement of PINK1 in Aβ25-35-mediated mitochondrial dynamics and that PINK1 may be a potential target for therapies aimed at enhancing neuroprotection to ameliorate Aβ25-35-induced insults.
    Keywords:  Alzheimer's disease; Mitochondrial fission and fusion; Neuroinflammation; PINK1; β-Amyloid
    DOI:  https://doi.org/10.1016/j.neuint.2022.105298
  30. J Neuroinflammation. 2022 Feb 05. 19(1): 34
      BACKGROUND: The role of physical exercise in the prevention of Alzheimer's disease (AD) has been widely studied. Microglia play an important role in AD. Triggering receptor expressed in myeloid cells 2 (TREM2) is expressed on microglia and is known to mediate microglial metabolic activity and brain glucose metabolism. However, the relationship between brain glucose metabolism and microglial metabolic activity during running exercise in APP/PS1 mice remains unclear.METHODS: Ten-month-old male APP/PS1 mice and wild-type mice were randomly divided into sedentary groups or running groups (AD_Sed, WT_Sed, AD_Run and WT_Run, n = 20/group). Running mice had free access to a running wheel for 3 months. Behavioral tests, [18]F-FDG-PET and hippocampal RNA-Seq were performed. The expression levels of microglial glucose transporter (GLUT5), TREM2, soluble TREM2 (sTREM2), TYRO protein tyrosine kinase binding protein (TYROBP), secreted phosphoprotein 1 (SPP1), and phosphorylated spleen tyrosine kinase (p-SYK) were estimated by western blot or ELISA. Immunohistochemistry, stereological methods and immunofluorescence were used to investigate the morphology, proliferation and activity of microglia.
    RESULTS: Long-term voluntary running significantly improved cognitive function in APP/PS1 mice. Although there were few differentially expressed genes (DEGs), gene set enrichment analysis (GSEA) showed enriched glycometabolic pathways in APP/PS1 running mice. Running exercise increased FDG uptake in the hippocampus of APP/PS1 mice, as well as the protein expression of GLUT5, TREM2, SPP1 and p-SYK. The level of sTREM2 decreased in the plasma of APP/PS1 running mice. The number of microglia, the length and endpoints of microglial processes, and the ratio of GLUT5+/IBA1+ microglia were increased in the dentate gyrus (DG) of APP/PS1 running mice. Running exercise did not alter the number of 5-bromo-2'-deoxyuridine (BrdU)+/IBA1+ microglia but reduced the immunoactivity of CD68 in the hippocampus of APP/PS1 mice.
    CONCLUSIONS: Running exercise inhibited TREM2 shedding and maintained TREM2 protein levels, which were accompanied by the promotion of brain glucose metabolism, microglial glucose metabolism and morphological plasticity in the hippocampus of AD mice. Microglia might be a structural target responsible for the benefits of running exercise in AD. Promoting microglial glucose metabolism and morphological plasticity modulated by TREM2 might be a novel strategy for AD treatment.
    Keywords:  Alzheimer’s disease; Hippocampus; Microglia; Running exercise; TREM2
    DOI:  https://doi.org/10.1186/s12974-022-02401-5
  31. J Neuroinflammation. 2022 Feb 07. 19(1): 42
      BACKGROUND: Multiple sclerosis (MS) is a chronic disabling disease of the central nervous system (CNS) commonly affecting young adults. There is increasing evidence that environmental factors are important in the development and course of MS. The metabolic syndrome (MetS) which comprises dyslipidemia has been associated with a worse outcome in MS disease. Furthermore, the lipid-lowering drug class of statins has been proposed to improve MS disease course. However, cholesterol is also rate-limiting for myelin biogenesis and promotes remyelination in MS animal models. Thus, the impact of circulating blood cholesterol levels during the disease remains debated and controversial.METHODS: We assessed the role of circulating cholesterol on the murine model of MS, the experimental autoimmune encephalomyelitis (EAE) disease using two different approaches: (1) the mouse model of familial hypercholesterolemia induced by low-density lipoprotein receptor (LDLr) deficiency, and (2) the use of the monoclonal anti-PCSK9 neutralizing antibody alirocumab, which reduces LDLr degradation and consequently lowers blood levels of cholesterol.
    RESULTS: Elevated blood cholesterol levels induced by LDLr deficiency did not worsen clinical symptoms of mice during EAE. In addition, we observed that the anti-PCSK9 antibody alirocumab did not influence EAE disease course, nor modulate the immune response in EAE.
    CONCLUSIONS: These findings suggest that blood cholesterol level has no direct role in neuro-inflammatory diseases and that the previously shown protective effects of statins in MS are not related to circulating cholesterol.
    Keywords:  Alirocumab; Autoimmunity; Cholesterol; EAE; Hypercholesterolemia; LDL receptor; Multiple sclerosis; Neuroinflammation; PCSK9
    DOI:  https://doi.org/10.1186/s12974-022-02409-x
  32. Neuroimage. 2022 Feb 07. pii: S1053-8119(22)00106-9. [Epub ahead of print] 118977
      In the technique presented here, dubbed 'qMRS', we quantify the change in 1H MRS signal following administration of 2H-labeled glucose. As in recent human DMRS studies, we administer [6,6'-2H2]-glucose orally to healthy subjects. Since 2H is not detectable by 1H MRS, the transfer of the 2H label from glucose to a downstream metabolite leads to a reduction in the corresponding 1H MRS resonance of the metabolite, even if the total concentration of both isoforms remains constant. Moreover, introduction of the deuterium label alters the splitting pattern of the proton resonances, making indirect detection of the deuterated forms- as well as the direct detection of the decrease of the unlabeled form- possible even without a 2H coil. Because qMRS requires only standard 1H MRS acquisition methods, it can be performed using commonly implemented single voxel spectroscopy (SVS) and chemical shift imaging (CSI) sequences. In this work, we implement qMRS in semi-LASER based CSI, generating dynamic maps arising from the fitted spectra, and demonstrating the feasibility of using qMRS and qCSI to monitor dynamic metabolism in the human brain using a 7T scanner with no auxiliary hardware.
    Keywords:  MRI; chemical shift imaging; deuterium; glucose; glutamate; metabolism; spectroscopy
    DOI:  https://doi.org/10.1016/j.neuroimage.2022.118977
  33. Brain Commun. 2022 Feb;4(1): fcac002
      Endoplasmic reticulum-based N ɛ-lysine acetylation serves as an important protein quality control system for the secretory pathway. Dysfunctional endoplasmic reticulum-based acetylation, as caused by overexpression of the acetyl coenzyme A transporter AT-1 in the mouse, results in altered glycoprotein flux through the secretory pathway and an autistic-like phenotype. AT-1 works in concert with SLC25A1, the citrate/malate antiporter in the mitochondria, SLC13A5, the plasma membrane sodium/citrate symporter and ATP citrate lyase, the cytosolic enzyme that converts citrate into acetyl coenzyme A. Here, we report that mice with neuron-specific overexpression of SLC13A5 exhibit autistic-like behaviours with a jumping stereotypy. The mice displayed disrupted white matter integrity and altered synaptic structure and function. Analysis of both the proteome and acetyl-proteome revealed unique adaptations in the hippocampus and cortex, highlighting a metabolic response that likely plays an important role in the SLC13A5 neuron transgenic phenotype. Overall, our results support a mechanistic link between aberrant intracellular citrate/acetyl coenzyme A flux and the development of an autistic-like phenotype.
    Keywords:  AT-1; SLC13A5; acetyl-CoA; citrate; lysine acetylation
    DOI:  https://doi.org/10.1093/braincomms/fcac002
  34. Reprod Sci. 2022 Feb 09.
      GnRH-I produced by hypothalamic neurosecretory cells is considered a master regulator of mammalian reproduction. Although GnRH-I transcription is well studied, the effect of ageing on transcriptional regulation of GnRH-I has not yet been explored. Here, we elucidate the effects of ageing on the metabolic environment like lactate level and TNF-α and how these affect GnRH-I transcription. Using pathway analysis of transcriptomic data, we found that lactate is upregulated in ageing astrocytes due to the downregulation of cellular respiration pathways possibly resulting in greater pyruvate concentration for lactate production. This lactate could then be shuttled into neurons where it would affect GnRH-I transcription. We showed that supra-physiological level of lactate in young mouse brain can mimic metabolic disturbances in the old brain and cause downregulation in GnRH-I transcription at a young age. In particular, we found upregulation of GnRH-I repressors in the young brain treated with high levels of lactate similar to old brain. Hence, this confirmed that aged metabolic environment can affect GnRH-I transcription even in the young brain. Further downstream analysis using the TRUST database showed NF-Kb signalling which lies downstream of both lactate and TNF-α as being capable of upregulating GnRH-I repressors. Since NF-Kb signalling has been shown in our study as well as others to be induced by TNF-α during ageing, it is likely that GnRH-I transcriptional regulation is mediated through these pathways. Thus, we formed a model for explaining the downregulation of GnRH-I transcription during ageing through differential expression of its TFs in an aged metabolic environment.
    Keywords:  Ageing; GnRH-I; Lactate; TNF-α; Transcription factors
    DOI:  https://doi.org/10.1007/s43032-021-00814-w
  35. J Mol Neurosci. 2022 Feb 07.
      Hypoglycemia has emerged as a prominent complication in anti-diabetic drug therapy or negative energy balance of animals, which causes brain damage, cognitive impairment, and even death. Brain injury induced by hypoglycemia is closely related to oxidative stress and the production of reactive oxygen species (ROS). The intracellular accumulation of ROS leads to neuronal damage, even death. Ketone body β-hydroxybutyrate (BHBA) not only serves as alternative energy source for glucose in extrahepatic tissues, but is also involved in cellular signaling transduction. Previous studies showed that BHBA reduces apoptosis by inhibiting the excessive production of ROS and activation of caspase-3. However, the effects of BHBA on apoptosis induced by glucose deprivation and its related molecular mechanisms have been seldom reported. In the present study, PC12 cells and primary cortical neurons were used to establish a low glucose injury model. The effects of BHBA on the survival and apoptosis in a glucose deficient condition and related molecular mechanisms were investigated by using flow cytometry, immunofluorescence, and western blotting. PC12 cells were incubated with 1 mM glucose for 24 h as a low glucose cell model, in which ROS accumulation and cell mortality were significantly increased. After 24 h and 48 h treatment with different concentrations of BHBA (0 mM, 0.05 mM, 0.5 mM, 1 mM, 2 mM), ROS production was significantly inhibited. Moreover, cell apoptosis rate was decreased and survival rate was significantly increased in 1 mM and 2 mM BHBA groups. In primary cortical neurons, at 24 h after treatment with 2 mM BHBA, the injured length and branch of neurites were significantly improved. Meanwhile, the intracellular ROS level, the proportion of c-Fos+ cells, apoptosis rate, and nuclear translocation of NF-κB protein after treatment with BHBA were significantly decreased when compared with that in low glucose cells. Importantly, the expression of p38, p-p38, NF-κB, and caspase-3 were significantly decreased, while the expression of p-ERK was significantly increased in both PC12 cells and primary cortical neurons. Our results demonstrate that BHBA decreased the accumulation of intracellular ROS, and further inhibited cell apoptosis by mediating the p38 MAPK signaling pathway and caspase-3 apoptosis cascade during glucose deprivation. In addition, BHBA inhibited apoptosis by activating ERK phosphorylation and alleviated the damage of low glucose to PC12 cells and primary cortical neurons. These results provide new insight into the anti-apoptotic effect of BHBA in a glucose deficient condition and the related signaling cascade.
    Keywords:  Apoptosis; Hypoglycemia; Primary cortical neurons; Reactive oxygen species; β-Hydroxybutyrate
    DOI:  https://doi.org/10.1007/s12031-022-01974-3
  36. Neurosci Bull. 2022 Feb 11.
      Alzheimer's disease (AD) is the most prevalent neurodegenerative disease featuring progressive cognitive impairment. Although the etiology of late-onset AD remains unclear, the close association of AD with apolipoprotein E (APOE), a gene that mainly regulates lipid metabolism, has been firmly established and may shed light on the exploration of AD pathogenesis and therapy. However, various confounding factors interfere with the APOE-related AD risk, raising questions about our comprehension of the clinical findings concerning APOE. In this review, we summarize the most debated factors interacting with the APOE genotype and AD pathogenesis, depict the extent to which these factors relate to APOE-dependent AD risk, and discuss the possible underlying mechanisms.
    Keywords:  Aging; Alzheimer’s disease; Apolipoprotein E; Diet; Ethnicity; Gender; Geographic factor
    DOI:  https://doi.org/10.1007/s12264-021-00814-5