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Myelin changes in Alexander disease

2018

Introduction: Alexander disease (AxD) is a type of leukodystrophy. Its pathological basis, along with myelin loss, is the appearance of Rosenthal bodies, which are cytoplasmic inclusions in astrocytes. Mutations in the gene coding for glial fibrillary acidic protein (GFAP) have been identified as a genetic basis for AxD. However, the mechanism by which these variants produce the disease is not understood. Development: The most widespread hypothesis is that AxD develops when a gain-of-function mutation causes an increase in GFAP. However, this mechanism does not explain myelin loss, given that experimental models in which GFAP expression is normal or mutated do not exhibit myelin disorders. …

0301 basic medicineMutationGlial fibrillary acidic proteinbiologyMechanism (biology)Cytoplasmic inclusionLeukodystrophymedicine.diseasemedicine.disease_causelcsh:RC346-429Alexander diseaseCell biology03 medical and health sciencesMyelin030104 developmental biology0302 clinical medicinemedicine.anatomical_structurenervous systembiology.proteinmedicineEpigeneticslcsh:Neurology. Diseases of the nervous system030217 neurology & neurosurgeryNeurología (English Edition)
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Myeloid leukemia with transdifferentiation plasticity developing from T-cell progenitors

2016

Unfavorable patient survival coincides with lineage plasticity observed in human acute leukemias. These cases are assumed to arise from hematopoietic stem cells, which have stable multipotent differentiation potential. However, here we report that plasticity in leukemia can result from instable lineage identity states inherited from differentiating progenitor cells. Using mice with enhanced c-Myc expression, we show, at the single-cell level, that T-lymphoid progenitors retain broad malignant lineage potential with a high capacity to differentiate into myeloid leukemia. These T-cell-derived myeloid blasts retain expression of a defined set of T-cell transcription factors, creating a lymphoi…

0301 basic medicineMyeloidBone Marrow CellsBiologyGeneral Biochemistry Genetics and Molecular Biology03 medical and health scienceshemic and lymphatic diseasesmedicineCell LineageProgenitor cellMolecular BiologyGeneral Immunology and MicrobiologyGeneral NeuroscienceTransdifferentiationMyeloid leukemiaCell DifferentiationArticlesmedicine.diseaseHematopoietic Stem CellsHaematopoiesisLeukemia030104 developmental biologymedicine.anatomical_structureImmunologyCancer researchLymphoid Progenitor CellsStem cell
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Sng1 associates with Nce102 to regulate the yeast Pkh–Ypk signalling module in response to sphingolipid status

2016

International audience; All cells are delimited by biological membranes, which are consequently a primary target of stress-induced damage. Cold alters membrane functionality by decreasing lipid fluidity and the activity of membrane proteins. In Saccharomyces cerevisiae, evidence links sphingolipid homeostasis and membrane phospholipid asymmetry to the activity of the Ypk1/2 proteins, the yeast orthologous of the mammalian SGK1-3 kinases. Their regulation is mediated by different protein kinases, including the PDK1 orthologous Pkh1/2p, and requires the function of protein effectors, among them Nce102p, a component of the sphingolipid sensor machinery. Nevertheless, the mechanisms and the act…

0301 basic medicineMyriocinOrm2Saccharomyces-cerevisiaeMembrane propertiesFatty Acids MonounsaturatedGlycogen Synthase Kinase 3Bacteriocins[SDV.IDA]Life Sciences [q-bio]/Food engineeringHomeostasisPhosphorylationMicroscopy ConfocalbiologyEffectorPlasma-membraneActin cytoskeleton[ SDV.IDA ] Life Sciences [q-bio]/Food engineeringPhospholipid translocationTransmembrane proteinCell biologyCold TemperatureBiochemistryP-type atpasesSignal transductionCold stressCell-wall integrityProtein BindingSignal TransductionProteins slm1Saccharomyces cerevisiae ProteinsPhospholipid translocationHigh-pressureSaccharomyces cerevisiaeImmunoblottingFluorescence PolarizationSaccharomyces cerevisiaeSignallingModels Biological3-Phosphoinositide-Dependent Protein Kinases03 medical and health sciencesBudding yeastMolecular BiologySphingolipids030102 biochemistry & molecular biologyTryptophan permeasePhospholipid flippingMembrane ProteinsCell Biologybiology.organism_classificationActin cytoskeletonSphingolipidYeast030104 developmental biologyMembrane proteinMutationPeptidesReactive Oxygen Species
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Effect of graphene substrate type on formation of Bi2Se3 nanoplates

2019

AbstractKnowledge of nucleation and further growth of Bi2Se3 nanoplates on different substrates is crucial for obtaining ultrathin nanostructures and films of this material by physical vapour deposition technique. In this work, Bi2Se3 nanoplates were deposited under the same experimental conditions on different types of graphene substrates (as-transferred and post-annealed chemical vapour deposition grown monolayer graphene, monolayer graphene grown on silicon carbide substrate). Dimensions of the nanoplates deposited on graphene substrates were compared with the dimensions of the nanoplates deposited on mechanically exfoliated mica and highly ordered pyrolytic graphite flakes used as refer…

0301 basic medicineNanostructureMaterials scienceNucleationlcsh:MedicineSubstrate (electronics)Chemical vapor depositionTOPOLOGICAL INSULATORGRAIN-BOUNDARIESArticlelaw.invention03 medical and health scienceschemistry.chemical_compoundTHIN-FILMS0302 clinical medicinelawSilicon carbide[CHIM]Chemical SciencesPyrolytic carbonThin filmlcsh:ScienceMultidisciplinaryGraphenelcsh:R030104 developmental biologySINGLEchemistryChemical engineeringGROWTHlcsh:Q030217 neurology & neurosurgeryScientific Reports
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A Systematic Nomenclature for the Drosophila Ventral Nerve Cord.

2020

Drosophila melanogaster is an established model for neuroscience research with relevance in biology and medicine. Until recently, research on the Drosophila brain was hindered by the lack of a complete and uniform nomenclature. Recognizing this, Ito et al. (2014) produced an authoritative nomenclature for the adult insect brain, using Drosophila as the reference. Here, we extend this nomenclature to the adult thoracic and abdominal neuromeres, the ventral nerve cord (VNC), to provide an anatomical description of this major component of the Drosophila nervous system. The VNC is the locus for the reception and integration of sensory information and involved in generating most of the locomotor…

0301 basic medicineNervous systemanatomytectulumanimal structures1.1 Normal biological development and functioningneuropilSensory systemhemilineageArticle03 medical and health sciences0302 clinical medicineTerminology as TopicmedicineNeuropilPsychologyAnimalsCell LineageInvertebrateontologyNomenclatureNeuronsNeurology & NeurosurgerybiologyGeneral NeurosciencefungiNeurosciencesCommissuremotorneuronbiology.organism_classificationNeuromeretractGanglia Invertebrate030104 developmental biologymedicine.anatomical_structureDrosophila melanogasterVentral nerve cordNeurologicalGangliacommissureinsectCognitive SciencesDrosophila melanogasterNerve NetNeuroscience030217 neurology & neurosurgeryneuromereNeuron
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Binge-like ethanol treatment in adolescence impairs autophagy and hinders synaptic maturation: Role of TLR4.

2018

Abstract Adolescence is a developmental period of brain maturation in which remodeling and changes in synaptic plasticity and neural connectivity take place in some brain regions. A different mechanism participates in adolescent brain maturation, including autophagy processes that play a role in synaptic development and plasticity. Alcohol is a neurotoxic compound whose abuse in adolescence causes TLR4 response activation by triggering neuroinflammation, neural damage and behavioral alterations. However, the potential participation of autophagy in long-term neurochemical and cognitive dysfunctions induced by binge ethanol drinking in adolescence is uncertain. We therefore evaluated whether …

0301 basic medicineNeurogenesisImmune receptorBiologyBinge Drinking03 medical and health sciencesMice0302 clinical medicineNeurochemicalAutophagyAnimalsTLR4PI3K/AKT/mTOR pathwayNeuroinflammationMice KnockoutBinge ethanol treatmentEthanolGeneral NeuroscienceAutophagyAge FactorsAdolescenceMice Inbred C57BLToll-Like Receptor 4030104 developmental biologyStructural synaptic plasticitySynaptic plasticitySynapsesExcitatory postsynaptic potentialTLR4FemaleNeuroscience030217 neurology & neurosurgeryNeuroscience letters
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Impact of Cholesterol Metabolism in Immune Cell Function and Atherosclerosis

2020

Cholesterol, the most important sterol in mammals, helps maintain plasma membrane fluidity and is a precursor of bile acids, oxysterols, and steroid hormones. Cholesterol in the body is obtained from the diet or can be de novo synthetized. Cholesterol homeostasis is mainly regulated by the liver, where cholesterol is packed in lipoproteins for transport through a tightly regulated process. Changes in circulating lipoprotein cholesterol levels lead to atherosclerosis development, which is initiated by an accumulation of modified lipoproteins in the subendothelial space; this induces significant changes in immune cell differentiation and function. Beyond lesions, cholesterol levels also play …

0301 basic medicineNeutrophilsLipoproteinsT-LymphocytesT cellInflammationlcsh:TX341-641Review030204 cardiovascular system & hematologyMonocytesMice03 medical and health scienceschemistry.chemical_compound0302 clinical medicineImmune systemimmune cellsmedicineAnimalsHomeostasisHumansCell ProliferationImmunity CellularNutrition and DieteticsChemistryCholesterolMacrophagesMonocytecholesterolLipid MetabolismSterolhematopoiesisCell biology030104 developmental biologymedicine.anatomical_structureLiverinflammationlipids (amino acids peptides and proteins)medicine.symptomatherosclerosismetabolismlcsh:Nutrition. Foods and food supplyIntracellularFood ScienceHormoneNutrients
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Carnosine protects pancreatic beta cells and islets against oxidative stress damage

2018

Abstract Islet transplantation is a valid therapeutic option for type 1 diabetes treatment. However, in this procedure one of the major problems is the oxidative stress produced during pancreatic islet isolation. The aim of our study was to evaluate potential protective effects of L-carnosine and its isomer D-carnosine against oxidative stress. We evaluated the carnosine effect on cell growth, cell death, insulin production, and the main markers of oxidative stress in rat and murine stressed beta cell lines as well as in human pancreatic islets. Both isomers clearly inhibited hydrogen peroxide induced cytotoxicity, with a decrease in intracellular reactive oxygen and nitrogen species, preve…

0301 basic medicineNitrous OxideCarnosineApoptosismedicine.disease_causeBiochemistrychemistry.chemical_compoundMice0302 clinical medicineEndocrinologyInsulin-Secreting CellsInsulin Secretiongeography.geographical_feature_categoryChemistryNitrotyrosineCarnosineDiabetesIsletReactive Nitrogen Speciesmedicine.anatomical_structureBeta cellPancreatic islet transplantationmedicine.medical_specialtyCell SurvivalProtective AgentsCell Line03 medical and health sciencesInternal medicinemedicineAnimalsHumansMolecular BiologyBeta cell lineCell ShapeCell ProliferationSettore MED/04 - Patologia GeneralegeographyPancreatic isletsTranscription Factor RelAHydrogen PeroxideRatsTransplantationOxidative Stress030104 developmental biologyEndocrinologyGlucoseGene Expression RegulationCytoprotectionTyrosinePancreatic islet transplantationReactive Oxygen Species030217 neurology & neurosurgeryOxidative stressBiomarkers
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Exploring the readthrough of nonsense mutations by non-acidic Ataluren analogues selected by ligand-based virtual screening

2016

Abstract Ataluren, also known as PTC124, is a 5-(fluorophenyl)-1,2,4-oxadiazolyl-benzoic acid suggested to suppress nonsense mutations by readthrough of premature stop codons in the mRNA. Potential interaction of PTC124 with mRNA has been recently studied by molecular dynamics simulations highlighting the importance of H-bonding and stacking π−π interactions. A series of non-acidic analogues of PTC124 were selected from a large database via a ligand-based virtual screening approach. Eight of them were synthesized and tested for their readthrough activity using the Fluc reporter harboring the UGA premature stop codon. The most active compound was further tested for suppression of the UGA non…

0301 basic medicineNonsense mutationDrug Evaluation PreclinicalMolecular ConformationCystic Fibrosis Transmembrane Conductance RegulatorMolecular Dynamics SimulationOxadiazolemedicine.disease_causeCftr geneCFTR gene03 medical and health scienceschemistry.chemical_compoundDrug DiscoverymedicineHumansRNA MessengerPharmacologyGeneticsOxadiazolesMessenger RNAVirtual screeningMutationNonsense mutationChemistryDrug Discovery3003 Pharmaceutical ScienceOrganic ChemistryGeneral MedicineLigand (biochemistry)PTCs readthroughMolecular biologyStop codonAtaluren030104 developmental biologyCodon NonsenseCystic fibrosiHeLa CellsEuropean Journal of Medicinal Chemistry
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Nut and legume consumption and human health: an umbrella review of observational studies

2021

Consumption of nuts and legumes has been associated with several health benefits. The aim of this study was to systematically review existing meta-analyses of observational studies on nut and legume intake and non-communicable diseases, and assess the level of evidence. Out of the six meta-analyses focussed on legume and 15 on nut intake, a possible association with decreased risk of colorectal adenoma and coronary heart disease was found for higher legume consumption, and a decreased risk of cardiovascular and cancer mortality, colon cancer, hypertension and ischaemic stroke for higher nut consumption. The association between legume consumption and cardiovascular diseases (CVDs), as well a…

0301 basic medicineNut030209 endocrinology & metabolismColorectal adenomameta-analysiBrain Ischemia03 medical and health sciences0302 clinical medicineMeta-Analysis as TopicRisk Factorscardiovascular diseaseNeoplasmsEnvironmental healthVegetablesmedicineHumansNutscancercoronary heart diseaseumbrella review.NutConsumption (economics)030109 nutrition & dieteticsumbrella reviewbusiness.industryIncidence (epidemiology)Confoundingdigestive oral and skin physiologyCancerfood and beveragesFabaceaelegumemedicine.diseaseDietStrokemeta-analysisObservational Studies as TopicCardiovascular DiseasesMeta-analysisHypertensionObservational studycoronary heart diseasesbusinessFood Science
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