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showing 10 items of 4837 documents

Constitutive psgl-1 correlates with cd30 and tcr pathways and represents a potential target for immunotherapy in anaplastic large t-cell lymphoma

2021

Simple Summary P-selectin glycoprotein ligand-1 (PSGL-1), coded by the SELPLG gene, is the major ligand of selectins and plays a pivotal role in tethering, rolling and extravasation of immune cells. PSGL-1 involvement in core molecular programs, such as SYK, PLCγ2, PI3Kγ or MAPK pathways, suggests additional functions beyond the modulation of cell trafficking. Recently, several studies identified a novel mechanism responsible for PSGL-1-mediated immune suppression in the tumor microenvironment and proved a novel concept of PSGL-1 as a critical checkpoint molecule for tumor immunotherapy. The immunotherapeutic approach has gained an ever-growing interest in the treatment of several hematolog…

0301 basic medicineCancer ResearchPTCLCD30medicine.medical_treatmentSykLymphoproliferative disordersBiologyALCL; ALK; CD30; Immunotherapy; PSGL-1; PTCL; TCRArticle03 medical and health sciences0302 clinical medicinehemic and lymphatic diseasesmedicineT-cell lymphomaPSGL-1RC254-282integumentary systemT-cell receptorNeoplasms. Tumors. Oncology. Including cancer and carcinogensImmunotherapymedicine.diseaseALCLLymphomaGene expression profiling030104 developmental biologyOncologyALK030220 oncology & carcinogenesisCD30Cancer researchImmunotherapyTCR
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Persistent immune stimulation exacerbates genetically driven myeloproliferative disorders via stromal remodeling

2017

Abstract Systemic immune stimulation has been associated with increased risk of myeloid malignancies, but the pathogenic link is unknown. We demonstrate in animal models that experimental systemic immune activation alters the bone marrow stromal microenvironment, disarranging extracellular matrix (ECM) microarchitecture, with downregulation of secreted protein acidic and rich in cysteine (SPARC) and collagen-I and induction of complement activation. These changes were accompanied by a decrease in Treg frequency and by an increase in activated effector T cells. Under these conditions, hematopoietic precursors harboring nucleophosmin-1 (NPM1) mutation generated myeloid cells unfit for normal …

0301 basic medicineCancer ResearchStromal cellMyeloidMice TransgenicVascular RemodelingBiologyInbred C57BLTransgenicMice03 medical and health sciencesMyelogenousMyeloproliferative DisordersmedicineAnimalsHumansMyeloproliferative DisorderAnimals; Cell Proliferation; Humans; Mice; Mice Inbred C57BL; Mice Inbred CBA; Mice Transgenic; Myeloproliferative Disorders; Stromal Cells; Vascular Remodeling; Oncology; Cancer ResearchCell ProliferationMyeloproliferative DisordersAnimalStromal CellInbred CBANeutrophil extracellular trapsmedicine.diseaseMice Inbred C57BLHaematopoiesisLeukemia030104 developmental biologymedicine.anatomical_structureOncologyImmunologyMice Inbred CBABone marrowStromal CellsNucleophosminHuman
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RBFOX1, encoding a splicing regulator, is a candidate gene for aggressive behavior

2020

The RBFOX1 gene (or A2BP1) encodes a splicing factor important for neuronal development that has been related to autism spectrum disorder and other neurodevelopmental phenotypes. Evidence from complementary sources suggests that this gene contributes to aggressive behavior. Suggestive associations with RBFOX1 have been identified in genome-wide association studies (GWAS) of anger, conduct disorder, and aggressive behavior. Nominal association signals in RBFOX1 were also found in an epigenome-wide association study (EWAS) of aggressive behavior. Also, variants in this gene affect temporal lobe volume, a brain area that is altered in several aggression-related phenotypes. In animals, this gen…

0301 basic medicineCandidate geneNeuroimagingRBFOX1Genome-wide association studyBiologyEpigenesis GeneticA2BP103 medical and health sciencesAll institutes and research themes of the Radboud University Medical Center0302 clinical medicineGeneticsmedicineAnimalsHumansPharmacology (medical)TranscriptomicsRBFOX1Genetic Association StudiesBiological PsychiatryRegulator genePharmacologyGeneticsNeurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7]AggressionGenetic Variationmedicine.diseasePhenotypeAnimal modelsAggressionPsychiatry and Mental health030104 developmental biologyNeurologyAutism spectrum disorderEpigeneticsRBFOX1 GeneRNA Splicing FactorsNeurology (clinical)medicine.symptom030217 neurology & neurosurgeryGenome-Wide Association Study
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SLC20A1 Is Involved in Urinary Tract and Urorectal Development

2020

Previous studies in developing Xenopus and zebrafish reported that the phosphate transporter slc20a1a is expressed in pronephric kidneys. The recent identification of SLC20A1 as a monoallelic candidate gene for cloacal exstrophy further suggests its involvement in the urinary tract and urorectal development. However, little is known of the functional role of SLC20A1 in urinary tract development. Here, we investigated this using morpholino oligonucleotide knockdown of the zebrafish ortholog slc20a1a. This caused kidney cysts and malformations of the cloaca. Moreover, in morphants we demonstrated dysfunctional voiding and hindgut opening defects mimicking imperforate anus in human cloacal exs…

0301 basic medicineCandidate genePathologyMorpholinoPediatricsEmbryonalentwicklungBlasenekstrophieBladder exstrophyZebrabärbling0302 clinical medicinebladder exstrophy-epispadias complex; CAKUT; cloacal malformation; functional genetics; kidney formation; SLC20A1; urinary tract development; zebrafish developmentbladder exstrophy-epispadias complexUrinary tract; Growth and developmentZebrafishlcsh:QH301-705.5ZebrafishNiereOriginal Researchcloacal malformationKidney; EmbryologyPediatrikzebrafish developmentKidney; Growth and developmentReconstructive and regenerative medicine Radboud Institute for Molecular Life Sciences [Radboudumc 10]030220 oncology & carcinogenesisembryonic structuresfunctional geneticsmedicine.symptomSLC20A1medicine.medical_specialtyEpispadiasanimal structuresUrinary systemBiologyKidney cystsCell and Developmental Biology03 medical and health sciencesAll institutes and research themes of the Radboud University Medical Centermedicineddc:610CAKUTNeurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7]Cloaca; Abnormalitieskidney formationCell Biologymedicine.diseaseCloacal exstrophybiology.organism_classificationurinary tract developmentReconstructive and regenerative medicine Radboud Institute for Health Sciences [Radboudumc 10]Bladder exstrophy030104 developmental biologyCloaca (embryology)lcsh:Biology (General)Developmental BiologyFrontiers in Cell and Developmental Biology
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Toxic Tau Oligomers Modulated by Novel Curcumin Derivatives

2019

AbstractThe pathological aggregation and accumulation of tau, a microtubule-associated protein, is a common feature amongst more than 18 different neurodegenerative diseases that are collectively known as tauopathies. Recently, it has been demonstrated that the soluble and hydrophobic tau oligomers are highly toxic in vitro due to their capacity towards seeding tau misfolding, thereby propagating the tau pathology seen across different neurodegenerative diseases. Modulating the aggregation state of tau oligomers through the use of small molecules could be a useful therapeutic strategy to target their toxicity, regardless of other factors involved in their formation. In this study, we screen…

0301 basic medicineCell biologyCurcuminCell SurvivalNeurotoxinsChemical biologyBiophysicsDrug Evaluation Preclinicallcsh:Medicinetau ProteinsProtein aggregationOligomerBiochemistryArticleBiophysical Phenomena03 medical and health scienceschemistry.chemical_compoundMiceProtein Aggregates0302 clinical medicineCell Line Tumormental disordersAnimalsHumanslcsh:ScienceNeuronsMultidisciplinaryCell DeathDrug discoveryDrug discoverySettore BIO/16 - Anatomia Umanalcsh:RSettore CHIM/06 - Chimica OrganicaSmall moleculeChemical biologyIn vitro3. Good healthTau protein Curcumin030104 developmental biologychemistryCell cultureBiophysicsCurcuminAlzheimerlcsh:QProtein Multimerization030217 neurology & neurosurgeryNeuroscience
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Grey Matter Microstructural Integrity Alterations in Blepharospasm Are Partially Reversed by Botulinum Neurotoxin Therapy.

2016

OBJECTIVE Benign Essential Blepharospasm (BEB) and hemifacial spasm (HFS) are the most common hyperkinetic movement disorders of facial muscles. Although similar in clinical presentation different pathophysiological mechanisms are assumed. Botulinum Neurotoxin (BoNT) is a standard evidence-based treatment for both conditions. In this study we aimed to assess grey matter microstructural differences between these two groups of patients and compared them with healthy controls. In patients we furthermore tracked the longitudinal morphometric changes associated with BoNT therapy. We hypothesized microstructural differences between the groups at the time point of maximum symptoms representation a…

0301 basic medicineCentral Nervous SystemMaleMovement disordersBotulinum ToxinsBlepharospasmlcsh:MedicineToxicologyPathology and Laboratory MedicineNervous SystemDiagnostic Radiology0302 clinical medicineMaterials PhysicsMedicine and Health SciencesToxinsLongitudinal StudiesGray Matterlcsh:ScienceMicrostructureMultidisciplinaryMovement DisordersRadiology and ImagingPhysicsMotor CortexBrainNeurodegenerative DiseasesAnatomyMiddle AgedMagnetic Resonance ImagingPathophysiologyBotulinum neurotoxinFacial musclesDystoniamedicine.anatomical_structureTreatment OutcomeNeurologyPhysical SciencesFemalePrimary motor cortexmedicine.symptomAnatomyResearch ArticleAdultmedicine.medical_specialtyImaging TechniquesBlepharospasmToxic AgentsBacterial ToxinsMaterials ScienceBotulinum ToxinGrey matterResearch and Analysis Methods03 medical and health sciencesDiagnostic MedicineOphthalmologymedicineHumansHemifacial SpasmDemographyAgedbusiness.industrylcsh:RBiology and Life SciencesCorrectionmedicine.disease030104 developmental biologyCross-Sectional StudiesFacePeople and Placeslcsh:QbusinessHead030217 neurology & neurosurgeryHemifacial spasmPloS one
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OFIP/KIAA0753 forms a complex with OFD1 and FOR20 at pericentriolar satellites and centrosomes and is mutated in one individual with oral-facial-digi…

2016

Item does not contain fulltext Oral-facial-digital (OFD) syndromes are rare heterogeneous disorders characterized by the association of abnormalities of the face, the oral cavity and the extremities, some due to mutations in proteins of the transition zone of the primary cilia or the closely associated distal end of centrioles. These two structures are essential for the formation of functional cilia, and for signaling events during development. We report here causal compound heterozygous mutations of KIAA0753/OFIP in a patient with an OFD VI syndrome. We show that the KIAA0753/OFIP protein, whose sequence is conserved in ciliated species, associates with centrosome/centriole and pericentrio…

0301 basic medicineCentriolecell-cycle progressionGene Expressionmedicine.disease_causeCiliopathieshuman-disease genemolecular characterizationbbs proteinsGenetics (clinical)Conserved SequenceCentriolesGeneticsMutationCiliumCiliary transition zoneMetabolic Disorders Radboud Institute for Molecular Life Sciences [Radboudumc 6]General MedicineOrofaciodigital Syndromes3. Good healthcentriolar satellitesmultiple sequence alignmentbasal body dockingFemaleMicrotubule-Associated ProteinsProtein BindingHeterozygoteMolecular Sequence DataBiology03 medical and health sciencesIntraflagellar transportCiliogenesis[ SDV.MHEP ] Life Sciences [q-bio]/Human health and pathologyGeneticsmedicineHumansAmino Acid SequenceCiliaMolecular BiologyCentrosomeintraflagellar transportBase SequenceInfant NewbornProteins030104 developmental biologyCentrosomeMutationciliary transition zoneSequence Alignment[SDV.MHEP]Life Sciences [q-bio]/Human health and pathologyciliogenesis
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Chronic stress leads to epigenetic dysregulation in the neuropeptide-Y and cannabinoid CB1 receptor genes in the mouse cingulate cortex.

2017

Persistent stress triggers a variety of mechanisms, which may ultimately lead to the occurrence of anxiety- and depression-related disorders. Epigenetic modifications represent a mechanism by which chronic stress mediates long-term effects. Here, we analyzed brain tissue from mice exposed to chronic unpredictable stress (CUS), which induced impaired emotional and nociceptive behaviors. As endocannabinoid (eCB) and neuropeptide-Y (Npy) systems modulate emotional processes, we hypothesized that CUS may affect these systems through epigenetic mechanisms. We found reduced Npy expression and Npy type 1 receptor (Npy1r) signaling, and decreased expression of the cannabinoid type 1 receptor (CB1) …

0301 basic medicineCingulate cortexMalemedicine.medical_specialtyCannabinoid receptormedicine.medical_treatmentBiologyGyrus CinguliEpigenesis Genetic03 medical and health sciencesCellular and Molecular Neurosciencechemistry.chemical_compoundMice0302 clinical medicineReceptor Cannabinoid CB1Internal medicinemental disordersmedicineAnimalsHumansChronic stressNeuropeptide YPharmacologyHistone deacetylase 2URB597Endocannabinoid systemhumanitiesMice Inbred C57BL030104 developmental biologyEndocrinologychemistryBenzamidesCannabinoidHistone deacetylaseCarbamates030217 neurology & neurosurgeryStress PsychologicalNeuropharmacology
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Uptake of polyphosphate microparticles in vitro (SaOS-2 and HUVEC cells) followed by an increase of the intracellular ATP pool size

2017

Recently two approaches were reported that addressed a vitally important problem in regenerative medicine, i. e. the successful treatment of wounds even under diabetic conditions. Accordingly, these studies with diabetic rabbits [Sarojini et al. PLoS One 2017, 12(4):e0174899] and diabetic mice [Müller et al. Polymers 2017, 9, 300] identified a novel (potential) target for the acceleration of wound healing in diabetes. Both studies propose a raise of the intracellular metabolic energy status via exogenous administration either of ATP, encapsulated into lipid vesicles, or of polyphosphate (polyP) micro-/nanoparticles. Recently this physiological polymer, polyP, was found to release metabolic …

0301 basic medicineConfocal MicroscopyBioenergeticsPhysiologyPolymerslcsh:Medicine02 engineering and technologyTrifluoperazineBiochemistryAdenosine TriphosphateEndocrinologyPolyphosphatesSpectroscopy Fourier Transform InfraredMedicine and Health Scienceslcsh:ScienceStainingMicroscopySecretory PathwayMultidisciplinaryChemistryLight MicroscopyCell Staining021001 nanoscience & nanotechnologyEndocytosisMicrospheres3. Good healthCell biologyChemistryMacromoleculesCell ProcessesPhysical SciencesRabbits0210 nano-technologyIntracellularResearch Articlemedicine.drugEndocrine DisordersMaterials by StructureMaterials ScienceBioenergeticsResearch and Analysis MethodsEndocytosisCell Line03 medical and health sciencesTissue RepairDiabetes Mellitusotorhinolaryngologic diseasesmedicineAnimalsHumansCalcium metabolismWound Healinglcsh:RSpectrometry X-Ray EmissionBiology and Life SciencesCell BiologyPolymer Chemistrydigestive system diseasesIn vitroMetabolism030104 developmental biologySpecimen Preparation and TreatmentCell cultureMetabolic DisordersMicroscopy Electron ScanningCalciumlcsh:QEnergy MetabolismPhysiological ProcessesWound healingConfocal Laser MicroscopyPowder DiffractionPLOS ONE
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Genetic association study of childhood aggression across raters, instruments, and age

2021

AbstractChildhood aggressive behavior (AGG) has a substantial heritability of around 50%. Here we present a genome-wide association meta-analysis (GWAMA) of childhood AGG, in which all phenotype measures across childhood ages from multiple assessors were included. We analyzed phenotype assessments for a total of 328 935 observations from 87 485 children aged between 1.5 and 18 years, while accounting for sample overlap. We also meta-analyzed within subsets of the data, i.e., within rater, instrument and age. SNP-heritability for the overall meta-analysis (AGGoverall) was 3.31% (SE = 0.0038). We found no genome-wide significant SNPs for AGGoverall. The gene-based analysis returned three sign…

0301 basic medicineDISORDER/45/43Genome-wide association study3124 Neurology and psychiatry0302 clinical medicineChildPsychiatry0303 health sciences:trastornos mentales [PSIQUIATRÍA Y PSICOLOGÍA]HERITABILITYMental DisordersCognitionGenomicsExplained variationJustice and Strong InstitutionsAggressionPsychiatry and Mental healthMeta-analysisADOLESCENCEChild Preschool:conducta y mecanismos de la conducta::conducta::síntomas conductuales::agresión [PSIQUIATRÍA Y PSICOLOGÍA]/631/208/212/dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_beingFemaleBiological psychiatrymedicine.symptomLife Sciences & Biomedicine:Investigative Techniques::Genetic Techniques::Genetic Association Studies [ANALYTICAL DIAGNOSTIC AND THERAPEUTIC TECHNIQUES AND EQUIPMENT]BEHAVIORRC321-571Childhood aggressionClinical psychologySDG 16 - PeaceAdolescent:Mental Disorders [PSYCHIATRY AND PSYCHOLOGY]Neurosciences. Biological psychiatry. NeuropsychiatrySingle-nucleotide polymorphismBiology3121 Internal medicineMalalties mentals - Aspectes genèticsGenetic correlationArticle1117 Public Health and Health ServicesCellular and Molecular Neuroscience03 medical and health sciences/631/477/2811SDG 3 - Good Health and Well-beingHuman behaviourmedicineSNPHumansGENOME-WIDE ASSOCIATIONBiological PsychiatryGenetic Association Studies030304 developmental biologyGenetic associationRetrospective Studies:técnicas de investigación::técnicas genéticas::estudios de asociación genética [TÉCNICAS Y EQUIPOS ANALÍTICOS DIAGNÓSTICOS Y TERAPÉUTICOS]Science & TechnologyAggressionSDG 16 - Peace Justice and Strong InstitutionsInfant:Behavior and Behavior Mechanisms::Behavior::Behavioral Symptoms::Aggression [PSYCHIATRY AND PSYCHOLOGY]1103 Clinical SciencesAgressivitat en els infantsHeritability/dk/atira/pure/sustainabledevelopmentgoals/peace_justice_and_strong_institutions030104 developmental biology1701 PsychologyORIGINSResearch Programm of Donders Centre for Neuroscience3111 BiomedicineTRAJECTORIES030217 neurology & neurosurgeryDemographyGenome-Wide Association Study
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