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showing 10 items of 19159 documents

Genome-wide association meta-analysis highlights light-induced signaling as a driver for refractive error

2018

Skin affections after sulfur mustard (SM) exposure include erythema, blister formation and severe inflammation. An antidote or specific therapy does not exist. Anti-inflammatory compounds as well as substances counteracting SM-induced cell death are under investigation. In this study, we investigated the benzylisoquinoline alkaloide berberine (BER), a metabolite in plants like berberis vulgaris, which is used as herbal pharmaceutical in Asian countries, against SM toxicity using a well-established in vitro approach. Keratinocyte (HaCaT) mono-cultures (MoC) or HaCaT/THP-1 co-cultures (CoC) were challenged with 100, 200 or 300 mM SM for 1 h. Post-exposure, both MoC and CoC were treated with 1…

0301 basic medicineAdultMaleCell typeResearchInstitutes_Networks_Beacons/MICRAIn silicotaittovirheetGenome-wide association studyRetinal Pigment EpitheliumBiologyBlindnessPolymorphism Single NucleotideSensory disorders Donders Center for Medical Neuroscience [Radboudumc 12]ArticleRetinaWhite People03 medical and health sciencesHIGH-GRADE MYOPIA ; RETINAL-PIGMENT EPITHELIUM ; SEROTONIN PATHWAY GENES ; FORM-DEPRIVATION MYOPIA ; COMMON VARIANTS ; OCULAR GROWTH ; RETINITIS-PIGMENTOSA ; GENOTYPE IMPUTATION ; MISSENSE MUTATIONS ; DOPAMINE-RECEPTORSAsian Peoplerefractive errorsRetinitis pigmentosaGeneticsmedicineMyopiaJournal ArticleHumansGenetic Predisposition to Disease610 Medicine & healthRegulation of gene expressionRetinaRetinal pigment epitheliummedicine.diseaseRefractive Errors030104 developmental biologymedicine.anatomical_structureManchester Institute for Collaborative Research on AgeingGene Expression Regulationgenetic factorsEye disorderFemalesense organsgeneettiset tekijätNeuroscienceGenome-Wide Association StudySignal Transduction
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Rescuing Over-activated Microglia Restores Cognitive Performance in Juvenile Animals of the Dp(16) Mouse Model of Down Syndrome.

2020

Microglia are brain-resident immune cells and regulate mechanisms essential for cognitive functions. Down syndrome (DS), the most frequent cause of genetic intellectual disability, is caused by a supernumerary chromosome 21, containing also genes related to the immune system. In the hippocampus of the Dp(16) mouse model of DS and DS individuals, we found activated microglia, as assessed by their morphology; activation markers; and, for DS mice, electrophysiological profile. Accordingly, we found increased pro-inflammatory cytokine levels and altered interferon signaling in Dp(16) hippocampi. DS mice also showed decreased spine density and activity of hippocampal neurons and hippocampus-depe…

0301 basic medicineAdultMaleDown syndromeDendritic spinemedicine.medical_treatmentAminopyridinesMice TransgenicHippocampal formationHippocampus03 medical and health sciencesMice0302 clinical medicineImmune systemCognitionMedicineHippocampus (mythology)AnimalsHumansPyrrolesNeuroinflammationMicrogliabusiness.industryGeneral NeuroscienceAnti-Inflammatory Agents Non-SteroidalAge Factorsmedicine.disease3. Good healthMice Inbred C57BLDisease Models Animal030104 developmental biologymedicine.anatomical_structureCytokinenervous systemFemaleMicrogliaDown SyndromebusinessNeuroscience030217 neurology & neurosurgeryNeuron
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Reducing diagnostic turnaround times of exome sequencing for families requiring timely diagnoses

2017

IF 2.137; International audience; BACKGROUND AND OBJECTIVE:Whole-exome sequencing (WES) has now entered medical practice with powerful applications in the diagnosis of rare Mendelian disorders. Although the usefulness and cost-effectiveness of WES have been widely demonstrated, it is essential to reduce the diagnostic turnaround time to make WES a first-line procedure. Since 2011, the automation of laboratory procedures and advances in sequencing chemistry have made it possible to carry out diagnostic whole genome sequencing from the blood sample to molecular diagnosis of suspected genetic disorders within 50 h. Taking advantage of these advances, the main objective of the study was to impr…

0301 basic medicineAdultMaleExome sequencingmedicine.medical_specialtyTime FactorsAdolescentGenetic counselingBioinformaticsTurnaround timeSensitivity and SpecificityUndiagnosed genetic conditions03 medical and health sciencesGeneticsmedicineHumansExomeGenetic TestingMedical diagnosisIntensive care medicineChildExomeGenetics (clinical)Exome sequencingGenetic testingWhole genome sequencing[SDV.GEN]Life Sciences [q-bio]/Geneticsmedicine.diagnostic_testbusiness.industryInfant NewbornInfantGeneral MedicineSequence Analysis DNADiagnostic turnaround time3. Good healthClinical trial030104 developmental biologyEarly DiagnosisChild PreschoolFemalebusiness[ SDV.GEN ] Life Sciences [q-bio]/Genetics
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M1 Macrophages Activate Notch Signalling in Epithelial Cells: Relevance in Crohn's Disease

2016

Background: The Notch signalling pathway plays an essential role in mucosal regeneration, which constitutes a key goal of Crohn's disease (CD) treatment. Macrophages coordinate tissue repair and several phenotypes have been reported which differ in the expression of surface proteins, cytokines and hypoxia-inducible factors (HIFs). We analysed the role of HIFs in the expression of Notch ligands in macrophages and the relevance of this pathway in mucosal regeneration. Methods: Human monocytes and U937-derived macrophages were polarized towards the M1 and M2 phenotypes and the expression levels of HIF-1α, HIF-2α, Jagged 1 (Jag1) and delta-like 4 (Dll4) were evaluated. The effects of macrophage…

0301 basic medicineAdultMaleJAG1FarmacologiaAdolescentEnterocyteColonNotch signaling pathwayBiologymucosal healing03 medical and health sciencesYoung AdultIntestinal mucosaCrohn DiseasemedicineMacrophageHumansHES1Intestinal MucosaRecte MalaltiesReceptors NotchMacrophagesGastroenterologyEpithelial CellsGeneral MedicineMiddle AgedHypoxia-Inducible Factor 1 alpha SubunitCoculture TechniquesCell biologyCrohn's disease030104 developmental biologymedicine.anatomical_structureAparell digestiu MalaltiesCase-Control StudiesImmunologyLeukocytes MononuclearCytokinesNotch signallingEnterocyte differentiationFemaleOriginal ArticleSignal transductionCaco-2 CellsHT29 CellsBiomarkersSignal Transduction
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Effect of ABC transporter expression and mutational status on survival rates of cancer patients

2020

ATP-binding cassette (ABC) transporters mediate multidrug resistance in cancer. In contrast to DNA single nucleotide polymorphisms in normal tissues, the role of mutations in tumors is unknown. Furthermore, the significance of their expression for prediction of chemoresistance and survival prognosis is still under debate. We investigated 18 tumors by RNA-sequencing. The mutation rate varied from 27,507 to 300885. In ABCB1, three hotspots with novel mutations were in transmembrane domains 3, 8, and 9. We also mined the cBioPortal database with 11,814 patients from 23 different tumor entities. We performed Kaplan-Meier survival analyses to investigate the effect of ABC transporter expression …

0301 basic medicineAdultMaleMutation rateNonsense mutationSingle-nucleotide polymorphismATP-binding cassette transporterRM1-950BiologyMultidrug resistanceP-glycoproteinPolymorphism Single Nucleotide03 medical and health sciences0302 clinical medicineNeoplasmsmedicineMissense mutationHumansSurvival analysisAgedCancerPharmacologyAged 80 and overPrognostic factorSequence Analysis RNACancerABCB5General MedicineMiddle AgedSurvival analysismedicine.diseaseMolecular Docking SimulationSurvival Rate030104 developmental biologyABC transporters030220 oncology & carcinogenesisMutationCancer researchATP-Binding Cassette TransportersFemaleTherapeutics. PharmacologyBiomedicine & Pharmacotherapy
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Perivascular Cells in Diffuse Cutaneous Systemic Sclerosis Overexpress Activated ADAM12 and Are Involved in Myofibroblast Transdifferentiation and De…

2016

Objective.Microvascular damage is pivotal in the pathogenesis of systemic sclerosis (SSc), preceding fibrosis, and whose trigger is not still fully understood. Perivascular progenitor cells, with profibrotic activity and function, are identified by the expression of the isoform 12 of ADAM (ADAM12) and this molecule may be upregulated by transforming growth factor-β (TGF-β). The goal of this work was to evaluate whether pericytes in the skin of patients with diffuse cutaneous SSc (dcSSc) expressed ADAM12, suggesting their potential contribution to the fibrotic process, and whether TGF-β might modulate this molecule.Methods.After ethical approval, mesenchymal stem cells (MSC) and fibroblasts …

0301 basic medicineAdultMalePathologymedicine.medical_specialtyImmunologyADAM12 Protein03 medical and health sciencesYoung AdultRheumatologyFibrosisTransforming Growth Factor betamedicineImmunology and AllergyHumansProgenitor cellMyofibroblastsSkinintegumentary systembusiness.industryMedicine (all)FIBROSIS; PERICYTE; SYSTEMIC SCLEROSIS; Rheumatology; Immunology; Immunology and AllergyMesenchymal stem cellTransdifferentiationMesenchymal Stem CellsMiddle Agedmedicine.diseaseFibrosisActinsUp-RegulationSettore MED/16 - Reumatologia030104 developmental biologymedicine.anatomical_structurePERICYTEFIBROSIS; PERICYTE; SYSTEMIC SCLEROSIS; Immunology and Allergy; Rheumatology; Immunology; Medicine (all)SYSTEMIC SCLEROSISCell TransdifferentiationScleroderma DiffuseFemalePericyteBone marrowbusinessPericytesMyofibroblastTransforming growth factorThe Journal of rheumatology
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Endothelial cell damage is the central part of COVID-19 and a mouse model induced by injection of the S1 subunit of the spike protein☆

2021

Neurologic complications of symptomatic COVID-19 are common. Brain tissues from 13 autopsies of people who died of COVID-19 were examined. Cultured endothelial and neuronal cells were incubated with and wild type mice were injected IV with different spike subunits. In situ analyses were used to detect SARS-CoV-2 proteins and the host response. In 13/13 brains from fatal COVID-19, pseudovirions (spike, envelope, and membrane proteins without viral RNA) were present in the endothelia of microvessels ranging from 0 to 14 positive cells/200× field (mean 4.3). The pseudovirions strongly co-localized with caspase-3, ACE2, IL6, TNFα, and C5b-9. The surrounding neurons demonstrated increased NMDAR2…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyProtein subunitH&E stainCaspase 3Spike proteinThirstPathology and Forensic Medicine03 medical and health sciencesMice0302 clinical medicineS1 subunitmedicineAnimalsHumansAgedAged 80 and overChemistrySARS-CoV-2COVID-19Endothelial CellsGeneral MedicineOriginal ContributionMiddle AgedMolecular biologyEndothelial stem cellDisease Models AnimalProtein Subunits030104 developmental biologyMembrane protein030220 oncology & carcinogenesisMicrovesselsSpike Glycoprotein CoronavirusImmunohistochemistryRNA ViralTumor necrosis factor alphaFemaleAutopsymedicine.symptomAnnals of Diagnostic Pathology
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Management of patients with hereditary angioedema in Germany: comparison with other countries in the Icatibant Outcome Survey

2018

Abstract Background The Icatibant Outcome Survey (IOS; NCT01034969) is a Shire‐sponsored, international, observational study monitoring the safety and effectiveness of icatibant, a bradykinin B2 receptor antagonist approved for the acute treatment of adults with hereditary angioedema with C1 inhibitor deficiency (HAE‐C1‐INH). Objective To report IOS data comparing demographic and icatibant treatment outcomes in patients with HAE‐C1‐INH from Germany to HAE‐C1‐INH patients from 11 other IOS countries. Methods A descriptive, retrospective, comparative analysis of data from 685 IOS patients with HAE‐C1‐INH from seven centres in Germany (n = 93) vs. centres from Austria, Brazil, Czech Republic, …

0301 basic medicineAdultMalePediatricsmedicine.medical_specialtyTime FactorsC1 inhibitor deficiencyTime to treatmentDermatologyBradykininAutoimmune DiseasesTime-to-Treatment03 medical and health scienceschemistry.chemical_compound0302 clinical medicineIcatibantGermanyBradykinin B2 Receptor AntagonistsmedicineHumansIn patientSymptom onsetRetrospective Studiesbusiness.industryAngioedemas HereditaryMiddle Agedmedicine.diseaseSymptom Flare UpHealth Surveys030104 developmental biologyInfectious Diseases030228 respiratory systemchemistryHereditary angioedemaObservational studyOriginal ArticleFemaleOutcome databusinessJournal of the European Academy of Dermatology and Venereology
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Centenarians Overexpress Pluripotency-Related Genes.

2018

Abstract Human mesenchymal cells can become pluripotent by the addition of Yamanaka factors OCT3/4, SOX2, c-MYC, KLF4. We have recently reported that centenarians overexpress BCL-xL, which has been shown to improve pluripotency; thus, we aimed to determine the expression of pluripotency-related genes in centenarians. We recruited 22 young, 32 octogenarian, and 47 centenarian individuals and determined the mRNA expression of Yamanaka factors and other stemness-related cell surface marker genes (VIM, BMP4, NCAM, BMPR2) in peripheral blood mononuclear cells by reverse transcription polymerase chain reaction. We found that centenarians overexpress OCT3/4, SOX2, c-MYC, VIM, BMP4, NCAM, and BMPR2…

0301 basic medicineAdultMalePluripotent Stem CellsAgingCellPeripheral blood mononuclear cellCohort Studies03 medical and health sciencesKruppel-Like Factor 40302 clinical medicineSOX2MedicineHumansGeneCells CulturedAged 80 and overbusiness.industryMesenchymal stem cellAge FactorsMembrane ProteinsReverse transcription polymerase chain reaction030104 developmental biologymedicine.anatomical_structureGene Expression RegulationKLF4Cancer researchLeukocytes MononuclearFemaleGeriatrics and GerontologyCentenarianbusiness030217 neurology & neurosurgeryThe journals of gerontology. Series A, Biological sciences and medical sciences
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Functional microbiome deficits associated with ageing: chronological age-threshold

2019

Composition of the gut microbiota changes during ageing, but questions remain about whether age is also associated with deficits in microbiome function and whether these changes occur sharply or progressively. The ability to define these deficits in populations of different ages may help determine a chronological age threshold at which deficits occur and subsequently identify innovative dietary strategies for active and healthy ageing. Here, active gut microbiota and associated metabolic functions were evaluated using shotgun proteomics in three well‐defined age groups consisting of 30 healthy volunteers, namely, ten infants, ten adults and ten elderly individuals. Samples from each volunte…

0301 basic medicineAdultMaleProteomicsAgingmedia_common.quotation_subjectPhysiologyGut flora03 medical and health sciences0302 clinical medicineGut bacteriaHumansMetabolomicsMicrobiomeVolunteermedia_commonAgedbiologyMicrobiotaLongevityAge FactorsTryptophanCell BiologyChronological agebiology.organism_classificationHealthy VolunteersAgeing030104 developmental biologyEditorialAgeingIndoleChild PreschoolFemaleMicrobiome030217 neurology & neurosurgeryCohort study
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