Search results for " DOM"

showing 10 items of 2750 documents

A constitutive BCL2 down-regulation aggravates the phenotype of PKD1-mutant-induced polycystic kidney disease

2017

IF 5.340; International audience; The main identified function of BCL2 protein is to prevent cell death by apoptosis. Mice knock-out for Bcl2 demonstrate growth retardation, severe polycystic kidney disease (PKD), gray hair and lymphopenia, and die prematurely after birth. Here, we report a 40-year-old male referred to for abdominal and thoracic aortic dissection with associated aortic root aneurysm, PKD, lymphocytopenia with a history of T cell lymphoblastic lymphoma, white hair since the age of 20, and learning difficulties. PKD, which was also detected in the father and sister, was related to an inherited PKD1 mutation. The combination of PKD with gray hair and lymphocytopenia was also r…

AdultMale0301 basic medicineTRPP Cation Channelsphenotypebcl2 geneBiologymicro rnaMice03 medical and health sciencesdown-regulationsymptom aggravating factorshemic and lymphatic diseasest-lymphocyteGene expressionGeneticsmedicinePolycystic kidney diseaseAnimalsHumansGenetic Predisposition to Disease[ SDV.GEN.GH ] Life Sciences [q-bio]/Genetics/Human geneticsgenesMolecular BiologyGeneGenetics (clinical)Exome sequencingMice KnockoutPKD1apoptosisExonsGeneral MedicinePolycystic Kidney Autosomal Dominantmedicine.diseasePhenotypePedigreeUp-Regulation3. Good healthMicroRNAs030104 developmental biologyMRNA SequencingProto-Oncogene Proteins c-bcl-2[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human geneticsImmunologyCancer researchLymphocytopeniapolycystic kidney diseasesbcl-2 proteinHuman Molecular Genetics
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Curvature of iris profile in spectral domain optical coherence tomography and dependency to refraction, age and pupil size - the MIPH Eye&Health …

2016

Purpose Optical coherence tomography (OCT) of the anterior segment allows quantitative analysis of the geometry of the iris. We performed spectral domain OCT examinations in healthy emmetropic, hyperopic and myopic subjects to investigate iris curvature and its associations. Methods In a cross-sectional study, out of 4617 eyes (2309 subjects) those with refractive errors of +3 dioptres were identified by objective refraction. The iris was examined using the anterior segment mode of a spectral domain 3D OCT-2000 (Topcon Inc., Japan) in the temporal meridian, and OCT scans were investigated with respect to presence and amount of convex and concave iris configuration. Ninety-three eyes of 50 s…

AdultMaleAdolescentgenetic structuresGonioscopyIrisEmmetropiaSpectral domainRefraction OcularCurvatureYoung Adult03 medical and health sciences0302 clinical medicineOptical coherence tomographyAnterior Eye SegmentGermanyEye healthHumansMedicineIntraocular PressureAgedmedicine.diagnostic_testbusiness.industryIncidenceAge FactorsPupil sizePupilGeneral MedicineMiddle AgedRefractive ErrorsRefractioneye diseasesOphthalmologyCross-Sectional StudiesMeridian (perimetry visual field)030221 ophthalmology & optometryOptometryFemalesense organsbusinessTomography Optical Coherence030217 neurology & neurosurgeryActa Ophthalmologica
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A genetic basis for IFN-gamma production and T-bet expression in humans.

2005

Abstract Th1 and Th2 cytokines secreted by polarized effector T cells play a pivotal role in the development of autoimmune and allergic diseases. However, the genetic basis of cytokine production by T lymphocytes in humans is poorly understood. In this study, we investigated the genetic contribution to cytokine production and regulation of T cell-specific transcription factors in a prospective twin study. We found a substantial genetic contribution to the production of Th1 cytokines such as IFN-γ and TNF-α with heritabilities of 0.85 (95% confidence intervals, 0.74–0.95) and 0.72 (0.50–0.93), respectively, whereas no genetic influence on production of the Th2 signature cytokine IL-4 was obs…

AdultMaleAdolescentmedicine.medical_treatmentImmunologyGATA3 Transcription FactorBiologyBody Mass IndexInterferon-gammaSex FactorsGenetic variationmedicineImmunology and AllergyHumansProspective StudiesGeneTranscription factorCells CulturedAgedGeneticsNFATC Transcription FactorsEffectorTumor Necrosis Factor-alphaAge FactorsNF-kappa BNFATHeritabilityMiddle AgedTwin studyCytokineImmunologyFemaleInterleukin-4T-Box Domain ProteinsTranscription FactorsJournal of immunology (Baltimore, Md. : 1950)
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Time course of human 40 Hz EEG activity accompanying P3 responses in an auditory oddball paradigm

1997

In order to quantify the time course of auditory P3-related gamma activity, root mean square (RMS) values were calculated from band-filtered (30-45 Hz) target and non-target responses in an auditory oddball experiment. Evoked (phase locked) gamma activity was evaluated from the time domain averages, whereas induced (not necessarily phase locked) activity was analyzed on the basis of single trials. Gamma RMS values were integrated across different time windows, namely the prestimulus, N50/P50, N100, pre P3, P3 and post P3 window. The single trial P3 window hereby was defined by a maximum amplitude criterion. In accordance with other studies, we found a pronounced increase of evoked gamma act…

AdultMaleAnalysis of VarianceN100medicine.medical_specialtyTime Factorsmedicine.diagnostic_testGeneral NeuroscienceAuditory oddballElectroencephalographyMiddle AgedElectroencephalographyStimulus (physiology)AudiologyDevelopmental psychologyRoot mean squareElectrophysiologyAcoustic StimulationTime courseEvoked Potentials AuditorymedicineHumansFemaleTime domainPsychologyNeuroscience Letters
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Neutralizing antibodies against SARS-CoV-2 variants of concern elicited by the comirnaty COVID-19 vaccine in nursing home residents.

2022

Immunosenescence may impact the functionality and breadth of vaccine-elicited humoral immune responses. The ability of sera to neutralize the SARS-CoV-2 spike protein (S) from Beta, Gamma, Delta, and Epsilon variants of concern (VOCs) relative to the ancestral Wuhan-Hu-1 strain was compared in Comirnaty COVID-19-vaccinated elderly nursing home residents, either SARS-CoV-2 naïve (n = 22) or experienced (n = 8), or SARS-CoV-2 naïve younger individuals (n = 18) and non-vaccinated individuals who recovered from severe COVID-19 (n = 19). In all groups, except that including SARS-CoV-2-experienced nursing home residents, some participants lacked NtAb against one or more VOCs, mainly the Beta vari…

AdultMaleCOVID-19 VaccinesCoronavirus disease 2019 (COVID-19)Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)Antibodies ViralImmune systemProtein DomainsNeutralization TestsMedicineHumansBeta (finance)AgedRetrospective StudiesAged 80 and overMultidisciplinarybiologybusiness.industrySARS-CoV-2COVID-19ImmunosenescenceMiddle AgedAntibodies NeutralizingFold changeImmunity HumoralNursing HomesTiterImmunologySpike Glycoprotein Coronavirusbiology.proteinFemaleAntibodybusinessScientific reports
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Genome-Wide Association Studies of the PR Interval in African Americans.

2011

The PR interval on the electrocardiogram reflects atrial and atrioventricular nodal conduction time. The PR interval is heritable, provides important information about arrhythmia risk, and has been suggested to differ among human races. Genome-wide association (GWA) studies have identified common genetic determinants of the PR interval in individuals of European and Asian ancestry, but there is a general paucity of GWA studies in individuals of African ancestry. We performed GWA studies in African American individuals from four cohorts (n = 6,247) to identify genetic variants associated with PR interval duration. Genotyping was performed using the Affymetrix 6.0 microarray. Imputation was p…

AdultMaleCancer ResearchMuscle ProteinsSingle-nucleotide polymorphismGenome-wide association studyQH426-470030204 cardiovascular system & hematologyBiologyGenetics and Genomics/Complex TraitsPolymorphism Single NucleotideSodium ChannelsWhite PeopleNAV1.5 Voltage-Gated Sodium ChannelNAV1.8 Voltage-Gated Sodium Channel03 medical and health sciencesElectrocardiography0302 clinical medicineAsian PeopleCardiovascular Disorders/Arrhythmias Electrophysiology and PacingGeneticsSNPHumansCardiac and Cardiovascular SystemsPR intervalInternational HapMap ProjectMyeloid Ecotropic Viral Integration Site 1 ProteinMolecular BiologyGenotypingGenetics (clinical)Ecology Evolution Behavior and Systematics030304 developmental biologyAgedGeneticsHomeodomain Proteins0303 health sciencesArrhythmias CardiacHeart-rate;Atherosclerosis risk; Genetic-analysis; Common variants; Design; Populations; Objectives; Conduction; Disease; TwinsMiddle AgedNeoplasm ProteinsMinor allele frequencyBlack or African AmericanAtrioventricular NodeFemaleT-Box Domain ProteinsImputation (genetics)Research ArticleGenome-Wide Association Study
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Migration of renal tumor cells depends on dephosphorylation of Shc by PTEN.

2011

The tumor suppressor PTEN is a phosphatase using FAK and Shc as direct substrates, and Akt as a key effector via PIP3. PTEN regulates cell migration and may influence metastases. We quantified PTEN in 135 clear cell renal cell carcinomas (ccRCC) by Western blot analysis and found statistically significant lower PTEN expression in patients who died, usually caused by metastases, within 5 years after surgery, compared to those surviving this time period. In athymic mice, PTEN transfected 786-O cells were injected into the tail vein and metastatic load of the lungs was quantified. We observed a strongly reduced metastatic load after PTEN transfection. For analyses of the PTEN activities, trans…

AdultMaleCancer ResearchSrc Homology 2 Domain-Containing Transforming Protein 1PhosphataseTransplantation HeterologousMice NudeBiologyMiceCell MovementTumor Cells CulturedPTENAnimalsHumansNeoplasm MetastasisPhosphorylationProtein kinase BCarcinoma Renal CellAgedAged 80 and overOncogenePTEN PhosphohydrolaseCell migrationCell cycleMiddle AgedPrognosisSurvival AnalysisKidney NeoplasmsGene Expression Regulation NeoplasticOncologyShc Signaling Adaptor ProteinsLipid phosphatase activityCancer researchbiology.proteinPhosphorylationFemaleInternational journal of oncology
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Overestimation of numerical distances in the left side of space

2004

Normal subjects presented with a middle number and two left- and right-sided outer numbers overestimate the numerical distance between the middle number and that positioned at its left side. Repetitive transcranial magnetic stimulation (rTMS) of the right posterior parietal cortex specifically counteracts this bias, suggesting that the mental representation of space defined by numbers is shifted toward the left side depending on a greater activity of the right hemisphere.

AdultMaleCerebralmedicine.medical_treatmentPosterior parietal cortexSpace (mathematics)Functional LateralityNOMagneticsReference ValuesParietal LobemedicineHumansRight hemisphereDominance CerebralDominanceMathematicsDistance PerceptionAnatomyTranscranial magnetic stimulationReference valuesRight posteriorSettore MED/26 - NeurologiaFemaleNeurology (clinical)Reference Values; Magnetics; Humans; Adult; Distance Perception; Dominance Cerebral; Parietal Lobe; Male; Functional Laterality; Female; MathematicsNeuroscienceMathematics
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Clinical and molecular genetic spectrum of autosomal dominant Emery-Dreifuss muscular dystrophy due to mutations of the lamin A/C gene.

2000

Emery-Dreifuss muscular dystrophy (EDMD) is characterized by early contractures of the elbows and Achilles tendons, slowly progressive muscle wasting and weakness, and life-threatening cardiomyopathy with conduction blocks. We recently identified LMNA encoding two nuclear envelope proteins, lamins A and C, to be implicated in the autosomal dominant form of EDMD. Here, we report on the variability of the phenotype and spectrum of LMNA mutations in 53 autosomal dominant EDMD patients (36 members of 6 families and 17 sporadic cases). Twelve of the 53 patients showed cardiac involvement exclusively, although the remaining 41 all showed muscle weakness and contractures. We were able to identify …

AdultMaleContractureAdolescentGenotypeBiopsyNonsense mutationDNA Mutational AnalysisEmerinMutation MissenseLaminopathyBiologyLMNACardiovascular Physiological PhenomenamedicineMissense mutationHumansEmery–Dreifuss muscular dystrophyMuscular dystrophyAge of OnsetChildCreatine KinasePhysical ExaminationMuscle contractureAgedGenes DominantGeneticsMuscle WeaknessMyocardiumNuclear ProteinsHeartMiddle Agedmedicine.diseaseLamin Type ALaminsMuscular Dystrophy Emery-DreifussPedigreeMuscular AtrophyPhenotypeNeurologyDisease ProgressionFemaleNeurology (clinical)Gene DeletionAnnals of neurology
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Reproducibility and Daytime-Dependent Changes of Corneal Epithelial Thickness and Whole Corneal Thickness Measured With Fourier Domain Optical Cohere…

2016

To evaluate the reproducibility of Fourier domain anterior segment optical coherence tomography (RTVue) based on repeated measurements of corneal thickness (CT) and epithelial thickness (ET) and to test daytime-dependent changes of these parameters.Twenty-three eyes from 23 healthy volunteers were included in this prospective study. Three clinical observers performed 3 consecutive measurements each of CT and ET in 3 sessions using RTVue, resulting in 9 measurements per session. Session 1 was performed at 9 AM ± 1 hour, session 2 at 4 PM ± 1 hour on the same day, and session 3 at 9 AM ± 1 hour 2 days later. CT and ET were assessed in 9 areas: corneal vertex, 4 paracentral zones, and 4 periph…

AdultMaleDaytimeMaterials sciencegenetic structuresCornea03 medical and health sciences0302 clinical medicineOpticsOptical coherence tomographyCorneamedicineHumansProspective StudiesFourier domainObserver VariationReproducibilitymedicine.diagnostic_testbusiness.industryEpithelium CornealReproducibility of ResultsMiddle Agedeye diseasesOphthalmologymedicine.anatomical_structure030221 ophthalmology & optometryFemalesense organsObserver variationbusiness030217 neurology & neurosurgeryTomography Optical CoherenceBiomedical engineeringCornea
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