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showing 10 items of 549 documents

Gitelman-Like Syndrome Caused by Pathogenic Variants in mtDNA

2022

Contains fulltext : 248375.pdf (Publisher’s version ) (Closed access) BACKGROUND: Gitelman syndrome is the most frequent hereditary salt-losing tubulopathy characterized by hypokalemic alkalosis and hypomagnesemia. Gitelman syndrome is caused by biallelic pathogenic variants in SLC12A3, encoding the Na(+)-Cl(-) cotransporter (NCC) expressed in the distal convoluted tubule. Pathogenic variants of CLCNKB, HNF1B, FXYD2, or KCNJ10 may result in the same renal phenotype of Gitelman syndrome, as they can lead to reduced NCC activity. For approximately 10 percent of patients with a Gitelman syndrome phenotype, the genotype is unknown. METHODS: We identified mitochondrial DNA (mtDNA) variants in th…

MaleKidneyDISEASEion transportGenotypeSolute Carrier Family 12 Member 3Gitelman-s syndromeCHANNEL GENEChildRNA Transfer IlePHOSPHORYLATIONNCCbiologygenetic renal diseaseblood pressureMetabolic Disorders Radboud Institute for Molecular Life Sciences [Radboudumc 6]General MedicineMiddle Agedchronic kidney failureTUBULENa transportPedigreemitochondriaBARTTER-SYNDROMEPhenotypemedicine.anatomical_structureMitochondrial respiratory chainMAGNESIUMNephrologyChild Preschoolepithelial sodium transportFemaleGitelman SyndromeAdultMitochondrial DNAAdolescentGenotypehuman geneticsKCNJ10DNA MitochondrialModels BiologicalPolymorphism Single NucleotideRNA Transfer PheYoung AdultTubulopathymedicineHumansDistal convoluted tubuleHYPOMAGNESEMIAAgedCLCNKBNeurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7]MITOCHONDRIAL-DNA MUTATIONBase SequenceInfantGitelman syndromemedicine.diseaseMolecular biologySODIUM-CHLORIDE COTRANSPORTERHEK293 CellsRenal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11]Basic ResearchMutationbiology.proteinNucleic Acid Conformationchronic kidney disease
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Extended analysis of a genome-wide association study in primary sclerosing cholangitis detects multiple novel risk loci

2012

Background & Aims: A limited number of genetic risk factors have been reported in primary sclerosing cholangitis (PSC). To discover further genetic susceptibility factors for PSC, we followed up on,a second tier of single nucleotide polymorphisms (SNPs) from a genome-wide association study (GWAS). Methods: We analyzed 45 SNPs in 1221 PSC cases and 3508 controls. The association results from the replication analysis and the original GWAS (715 PSC cases and 2962 controls) were combined in a meta-analysis comprising 1936 PSC cases and 6470 controls. We performed an analysis of bile microbial community composition in 39 PSC patients by 16S rRNA sequencing. Results: Seventeen SNPs representing 1…

MaleLinkage disequilibriumendocrine system diseasesGenome-wide association studyPrimary biliary cirrhosisGenotypeBLOOD-GROUPBileChildPOPULATIONAged 80 and overGeneticseducation.field_of_studyPrimary sclerosing cholangitisdigestive oral and skin physiologyMiddle AgedFucosyltransferasesChild PreschoolDISEASESFemaleNeprilysinReceptors Tumor Necrosis Factor Member 14B-LYMPHOCYTEAdultRiskGenome-wide association studyAdolescentGenotypeSUSCEPTIBILITY LOCIFUT2Cholangitis SclerosingPopulationT-LYMPHOCYTE ATTENUATORSingle-nucleotide polymorphismBiologyPolymorphism Single Nucleotidedigestive systemArticlePrimary sclerosing cholangitisGenetic predispositionmedicineImmunogeneticsHumansGenetic Predisposition to DiseaseeducationMETAANALYSISAgedNON-SECRETOR STATUSHepatologymedicine.diseaseGENEdigestive system diseasesSingle nucleotide polymorphismGenetic LociImmunology
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Blood-brain barrier penetration of the enantiomers of venlafaxine and its metabolites in mice lacking P-glycoprotein

2010

According to in vitro studies the enantiomers of venlafaxine display different degrees of serotonin and noradrenaline reuptake inhibition. Therefore, clarification of the enantiomeric drug distribution between serum and brain is highly warranted. To elucidate if P-glycoprotein (P-gp) in a stereoselective manner transports venlafaxine and its metabolites out of the brain we used abcb1ab double-knockout mice that do not express P-gp. A single dose of racemic venlafaxine (10 mg/kg bw) was intraperitoneally injected to knockout (-/-) and wildtype (+/+) mice. Serum and brain samples were collected 1, 3, 6 and 9 h following drug administration for analysis by LC/MS/MS. One to six hours post-dose,…

MaleMedicin och hälsovetenskapVenlafaxinePharmacologyBlood–brain barrierMedical and Health SciencesMicemedicineAnimalsPharmacology (medical)ATP Binding Cassette Transporter Subfamily B Member 1Biological PsychiatryP-glycoproteinPharmacologyMice KnockoutbiologyChemistryVenlafaxine HydrochlorideBiological TransportStereoisomerismCyclohexanolsIn vitroPsychiatry and Mental healthmedicine.anatomical_structureNeurologyBlood-Brain BarrierKnockout mousebiology.proteinStereoselectivityNeurology (clinical)SerotoninEnantiomerSelective Serotonin Reuptake Inhibitorsmedicine.drug
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Altered brain concentrations of citalopram and escitalopram in P-glycoprotein deficient mice after acute and chronic treatment

2013

Background: According to both in vitro and in vivo data P-glycoprotein (P-gp) may restrict the uptake of several antidepressants into the brain, thus contributing to the poor success rate of current antidepressant therapies. The therapeutic activity of citalopram resides in the Senantiomer, whereas the R-enantiomer is practically devoid of serotonin reuptake potency. To date, no in vivo data are available that address whether the enantiomers of citalopram and its metabolites are substrates of P-gp. Methods: P-gp knockout (abcb1ab (-/-)) and wild-type (abcb1ab (+/+)) mice underwent acute (single-dose) and chronic (two daily doses for 10 days) treatment with citalopram (10 mg/kg) or escitalop…

MaleMedicin och hälsovetenskapescitalopramenantiomersCitaloprammice knockoutP-glycoproteinCitalopramPharmacologyMedical and Health Sciencesbehavioral disciplines and activitiesMiceIn vivomental disordersmedicineAnimalsEscitalopramPotencyPharmacology (medical)ATP Binding Cassette Transporter Subfamily B Member 1Biological PsychiatryP-glycoproteinMice KnockoutPharmacologybiologybusiness.industryBrainPsychiatry and Mental healthNeurologyKnockout mousebiology.proteinAntidepressive Agents Second-GenerationAntidepressantNeurology (clinical)Enantiomerbusinessmedicine.drugEuropean Neuropsychopharmacology
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Neuropsychological consequences of chronic stress: the case of informal caregivers.

2018

Introduction: Caring for a family member with a long-term illness is a significant source of chronic stress that might significantly accelerate the cognitive ageing of informal caregivers. Neverthe...

MaleNeuropsychological Tests03 medical and health sciences0302 clinical medicineCognitionHumansChronic stressAged030214 geriatricsNeuropsychologySocial SupportCognitive behavioural interventionsPsychiatry and Mental healthFamily memberCross-Sectional StudiesMemory Short-TermCaregiversAgeingFemaleCognitive ageingGeriatrics and GerontologyPshychiatric Mental HealthPsychologyCognition DisordersGerontology030217 neurology & neurosurgeryStress PsychologicalClinical psychologyAgingmental health
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Mast cell targeting hampers prostate adenocarcinoma development but promotes the occurrence of highly malignant neuroendocrine cancers

2011

Abstract Mast cells (MC) are c-Kit–expressing cells, best known for their primary involvement in allergic reactions, but recently reappraised as important players in either cancer promotion or inhibition. Here, we assessed the role of MCs in prostate tumor development. In prostate tumors from both tumor-prone transgenic adenocarcinoma of the mouse prostate (TRAMP) mice and human patients, MCs are specifically enriched and degranulated in areas of well-differentiated (WD) adenocarcinoma but not around poorly differentiated (PD) foci that coexist in the same tumors. We derived novel TRAMP tumor cell lines, representative of WD and PD variants, and through pharmacologic stabilization or geneti…

MaleOncologyCancer Researchmedicine.medical_specialtyEpithelial-Mesenchymal TransitionMice TransgenicAdenocarcinomaBiologymedicine.disease_causeCell DegranulationMiceProstate cancerProstateCell Line TumorInternal medicineTumor MicroenvironmentmedicineMast CellAnimalsHumansMast CellsReceptors Tumor Necrosis Factor Member 25Tumor microenvironmentAdenocarcinoma; Animals; Carcinoma Neuroendocrine; Cell Degranulation; Cell Line Tumor; Disease Progression; Epithelial-Mesenchymal Transition; Humans; Male; Mast Cells; Matrix Metalloproteinase 9; Mice; Mice Inbred C57BL; Mice Transgenic; Prostatic Neoplasms; Proto-Oncogene Proteins c-kit; Receptors Tumor Necrosis Factor Member 25; Tumor Microenvironment; Cancer Research; OncologyAnimalProstatic NeoplasmsCancermedicine.diseasehumanitiesCarcinoma NeuroendocrineMice Inbred C57BLProto-Oncogene Proteins c-kitmedicine.anatomical_structureMatrix Metalloproteinase 9OncologyTumor progressionProstatic NeoplasmDisease ProgressionAdenocarcinomaCarcinogenesisHumanTramp
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Regulation of organic anion transporters in a new rat model of acute and chronic cholangitis resembling human primary sclerosing cholangitis

2002

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiology. Although the primary defect affects cholangiocytes, cholestatic injury of hepatocytes may promote further liver damage. Since down-regulation of hepatocellular organic anion transporters is implicated in the molecular pathogenesis of cholestasis, expression of these transporters was determined in a novel rat model, which closely resembles human PSC.Hepatic protein and mRNA expression of basolateral (Ntcp, Oatp1, 2 and 4) and canalicular (Mrp2, Bsep) organic anion transporters were analyzed 1, 4 and 12 weeks after induction of experimental PSC by 2,4,6-trinitrobenzenesulfonic acid (TNBS).Specific down-re…

MalePathologymedicine.medical_specialtyOrganic Cation Transport ProteinsOrganic anion transporter 1Cholangitis SclerosingGene ExpressionOrganic Anion Transporters Sodium-DependentInflammationOrganic Anion Transporters Sodium-Independentdigestive systemPrimary sclerosing cholangitisSolute Carrier Organic Anion Transporter Family Member 1B3CholestasismedicineAnimalsRNA MessengerChronic CholangitisLiver injurySymportersHepatologybiologybusiness.industryMultidrug resistance-associated protein 2Membrane Transport ProteinsTransportermedicine.diseasedigestive system diseasesRatsDisease Models AnimalRats Inbred LewAcute DiseaseChronic Diseasebiology.proteinATP-Binding Cassette TransportersFemalemedicine.symptomCarrier ProteinsbusinessJournal of Hepatology
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DNA-Ploidy, Morphometric-Stereological and P-Glycoprotein Study of Superficial Bladder Carcinomas

1992

We carried out a DNA-ploidy, morphometric-stereologic and P-glycoprotein study on 40 newly diagnosed superficial bladder cancer patients (G1-G2), correlating the results with histological grade and clinical outcome. Variations in the number of patients who present recurrences, progression or remain tumor-free during the whole follow-up period (at least 5 years) were not significant when related to nuclear size, proliferative diploid index, presence of aneuploidy and expression of P-glycoprotein. It is striking how the majority of disease-free subjects showed a proliferative diploid index higher than 10%. Moreover, 3 of them presented an aneuploid cell population. In our study, only histolog…

MalePathologymedicine.medical_specialtyUrologyCellPopulationAneuploidyNewly diagnosedBiomarkers TumormedicineHumansIn patientATP Binding Cassette Transporter Subfamily B Member 1educationDna ploidyP-glycoproteineducation.field_of_studyMembrane GlycoproteinsPloidiesbiologybusiness.industryDNA Neoplasmmedicine.diseaseNeoplasm Proteinsmedicine.anatomical_structureUrinary Bladder Neoplasmsbiology.proteinFemalePloidybusinessFollow-Up StudiesEuropean Urology
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Double Negative (IgG+IgD-CD27-) B Cells are Increased in a Cohort of Moderate-Severe Alzheimer’s Disease Patients and Show a Pro-Inflammatory Traffic…

2014

Alzheimer's disease (AD) is a progressive, irreversible, and debilitating disease for which no effective preventive or disease modifying therapies or treatments have so far been detected. The crucial step in AD pathogenesis is the production of amyloid-42 peptide, which causes chronic inflammation. Activated cells in the central nervous system (CNS) produce pro- inflammatory mediators that lead to the recruitment of myeloid or lymphocytic cells. As a consequence, the communication between the CNS and peripheral blood of AD subjects could influence the lymphocyte distribution and/or the expression of phenotypic markers. In the present paper, we show a significant decrease in total CD19 + B l…

MaleReceptors CCR6Receptors CCR7MyeloidLymphocyteB-Lymphocyte SubsetsC-C chemokine receptor type 7InflammationC-C chemokine receptor type 6Immunoglobulin DCD19Cohort StudiesAlzheimer DiseasemedicineHumansB cellAgedAged 80 and overSettore MED/04 - Patologia GeneralebiologyGeneral NeuroscienceGeneral MedicineImmunoglobulin DFlow CytometryTumor Necrosis Factor Receptor Superfamily Member 7Psychiatry and Mental healthClinical Psychologymedicine.anatomical_structurePhenotypeAlzheimer's Disease Inflammation B CellsImmunoglobulin GImmunologybiology.proteinFemaleSettore MED/26 - NeurologiaGeriatrics and Gerontologymedicine.symptomMental Status Schedule
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Co-option of Neutrophil Fates by Tissue Environments

2020

Classically considered short-lived and purely defensive leukocytes, neutrophils are unique in their fast and moldable response to stimulation. This plastic behavior may underlie variable and even antagonistic functions during inflammation or cancer, yet the full spectrum of neutrophil properties as they enter healthy tissues remains unexplored. Using a new model to track neutrophil fates, we found short but variable lifetimes across multiple tissues. Through analysis of the receptor, transcriptional, and chromatin accessibility landscapes, we identify varying neutrophil states and assign non-canonical functions, including vascular repair and hematopoietic homeostasis. Accordingly, depletion…

MaleReceptors CXCR4Transcription GeneticAngiogenesisNeutrophilsMedizinNeovascularization PhysiologicInflammationBiologyCXCR4General Biochemistry Genetics and Molecular Biology03 medical and health sciences0302 clinical medicineSingle-cell analysismedicineNuclear Receptor Subfamily 4 Group A Member 1AnimalsCell LineageReceptorLung030304 developmental biology0303 health sciencesInnate immune systemChromatinChromatinCell biologyHematopoiesisIntestinesMice Inbred C57BLOrgan SpecificityFemalemedicine.symptomSingle-Cell AnalysisTranscriptome030217 neurology & neurosurgeryHomeostasisCell
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