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showing 10 items of 4345 documents

Longitudinal study of DNA methylation during the first 5 years of life

2016

[Background]: Early life epigenetic programming influences adult health outcomes. Moreover, DNA methylation levels have been found to change more rapidly during the first years of life. Our aim was the identification and characterization of the CpG sites that are modified with time during the first years of life. We hypothesize that these DNA methylation changes would lead to the detection of genes that might be epigenetically modulated by environmental factors during early childhood and which, if disturbed, might contribute to susceptibility to diseases later in life. [Methods]: The study of the DNA methylation pattern of 485577 CpG sites was performed on 30 blood samples from 15 subjects,…

0301 basic medicineLongitudinal studyADNCentromereBiologyGenomeGeneral Biochemistry Genetics and Molecular BiologyRepressive histone markBioconductor03 medical and health sciencesImmune systemCluster AnalysisHumansLongitudinal StudiesGeneGeneticsMedicine(all)Biochemistry Genetics and Molecular Biology(all)ResearchHistone markAdult health outcomeInfant NewbornInfantGeneral MedicineMethylationDNA MethylationTelomere030104 developmental biologyGene OntologyCpG siteChild PreschoolDNA methylationGene ontologySurrogate variable analysisCpG IslandsJournal of Translational Medicine
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Biallelic variants in LARS2 and KARS cause deafness and (ovario)leukodystrophy

2019

Supplemental Digital Content is available in the text.

0301 basic medicineLysine-tRNA LigaseMalePathologyMagnetic Resonance SpectroscopyMedizinmembrane proteins030204 cardiovascular system & hematologyMitochondrionDeafnessmedicine.disease_causeCompound heterozygosityCorrectionsLeukoencephalopathyMyelin0302 clinical medicineCytosolLeukoencephalopathies030212 general & internal medicineOvarian DiseasesTransfer RNA AminoacylationChildZebrafishMUTATIONExome sequencing10012MutationBrainMetabolic Disorders Radboud Institute for Molecular Life Sciences [Radboudumc 6]General MedicineMiddle AgedDisorders of movement Donders Center for Medical Neuroscience [Radboudumc 3]Magnetic Resonance ImagingMitochondriaProtein Transportendoplasmic reticulummedicine.anatomical_structureChild PreschoolTransfer RNAComputingMethodologies_DOCUMENTANDTEXTPROCESSING/dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_being[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]Biological AssayFemaleWRBRare cancers Radboud Institute for Health Sciences [Radboudumc 9]Adultcardiomyopathiesmedicine.medical_specialtyMitochondrial diseaseAminoacylationMuscle disorderBiologyArticleMEDIATES INSERTIONAmino Acyl-tRNA Synthetases03 medical and health sciencesSDG 3 - Good Health and Well-beingmedicineAnimalsPoint MutationHumansAmino Acid SequenceAlleleAllelesCOMPLEXGenetic heterogeneitybusiness.industryArsenite Transporting ATPasesLeukodystrophyGenetic Variation10090Original ArticlesZebrafish Proteinsbiology.organism_classificationDILATED CARDIOMYOPATHYmedicine.diseasezebrafishGENEMolecular biologyDisease Models Animal030104 developmental biologyMembrane protein[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics10084Neurology (clinical)Transfer RNA AminoacylationMEMBRANEbusinessSequence Alignment030217 neurology & neurosurgeryexomeNeurology
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Associations between parental food choice motives, health-promoting feeding practices, and infants' fruit and vegetable intakes: the Food4toddlers st…

2020

Background: Parents influence their infants' diets and are the providers of healthy foods such as fruit and vegetables. Parental motives can influence infant's diets directly or through parental feeding practices. Objective: This study aimed to assess the associations between parental food choice motives and infants' fruit and vegetable intakes and to examine whether parental feeding practices mediated these associations. Design: A total of 298 parents participated in the Norwegian Food4toddlers study. Before the child's first birthday (mean age = 10.9 months), the parents completed an online baseline questionnaire. Five parental food choice motives were assessed: health, convenience, senso…

0301 basic medicineMEDIATIONMediation (statistics)healthy food intakelcsh:TX341-641CHILDREN030209 endocrinology & metabolismNorwegianVALIDATIONCONSTRUCTS03 medical and health sciences0302 clinical medicineEnvironmental healthFood choiceMedicine and Health SciencesQUALITYMedicinemediationPRACTICES QUESTIONNAIRE030109 nutrition & dieteticsNutrition and DieteticsNutrition Interventionsbusiness.industryPublic Health Environmental and Occupational HealthCONSUMPTIONMean ageinfantlanguage.human_languagePeer reviewDIETARY PATTERNSPRESCHOOLERSlanguageOriginal ArticleParental feedingBootstrap confidence intervalbusinessdietlcsh:Nutrition. Foods and food supplyBEHAVIORFood ScienceFoodnutrition research
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De novo mutations in the X-linked TFE3 gene cause intellectual disability with pigmentary mosaicism and storage disorder-like features

2020

IntroductionPigmentary mosaicism (PM) manifests by pigmentation anomalies along Blaschko’s lines and represents a clue toward the molecular diagnosis of syndromic intellectual disability (ID). Together with new insights on the role for lysosomal signalling in embryonic stem cell differentiation, mutations in the X-linked transcription factor 3 (TFE3) have recently been reported in five patients. Functional analysis suggested these mutations to result in ectopic nuclear gain of functions.Materials and methodsSubsequent data sharing allowed the clustering of de novo TFE3 variants identified by exome sequencing on DNA extracted from leucocytes in patients referred for syndromic ID with or with…

0301 basic medicineMESH: Basic Helix-Loop-Helix Leucine Zipper Transcription Factors[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/NeurobiologyIntellectual disabilityTFE3Biology[SDV.GEN.GH] Life Sciences [q-bio]/Genetics/Human geneticsMESH: Intellectual Disability03 medical and health sciencesExon0302 clinical medicineMESH: Whole Exome SequencingMESH: ChildIntellectual disabilityGeneticsmedicineMissense mutationGeneGenetics (clinical)Exome sequencingPigmentary mosaicismMESH: Pathology MolecularGeneticsMESH: AdolescentMESH: HumansAlternative splicingLysosomal metabolismMESH: Child Preschool[SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/NeurobiologyMESH: Adultmedicine.diseasePhenotypeMESH: InfantMESH: MaleTFE3Storage disorder030104 developmental biologyMESH: Genes X-Linked[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human geneticsMESH: Young AdultMESH: EpilepsyMESH: MosaicismMESH: Pigmentation DisordersMESH: Female030217 neurology & neurosurgery
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A rare disease and education : Neurofibromatosis type 1 decreases educational attainment

2021

Rare heritable syndromes may affect educational attainment. Here, we study education in neurofibromatosis 1 (NF1) that is associated with multifaceted medical, social and cognitive consequences. Educational attainment in the Finnish population‐based cohort of 1408 individuals with verified NF1 was compared with matched controls using Cox proportional hazards model with delayed entry and competing risk for death. Moreover, models accounting for the effects of cancer at age 15–30 years, parental NF1 and developmental disorders were constructed. Overall, the attainment of secondary education was reduced in individuals with NF1 compared to controls (hazard ratio 0.83, 95%CI 0.74–0.92). History …

0301 basic medicineMale030105 genetics & heredityNeoplasmsneurofibromatosis 1ChildGenetics (clinical)FinlandLearning DisabilitiesHazard ratioCognitionVocational educationChild Preschooleducational attainmentCohortEducational StatusFemaleOriginal ArticleAdultopintomenestyscongenital hereditary and neonatal diseases and abnormalitiesNeurofibromatosis 1Adolescentrare diseaseneurofibromatoosiAffect (psychology)multiorgan syndromeschool performance03 medical and health sciencesRare DiseaseskoulutustasoGeneticsmedicineHumansEducation Graduateharvinaiset tauditNeurofibromatosisneoplasmsProportional Hazards ModelsVocational Educationperinnölliset tauditProportional hazards modelbusiness.industrySiblingsOriginal Articlesmedicine.diseaseEducational attainmenteye diseasesnervous system diseases030104 developmental biologybusinessDemographyFollow-Up Studies
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DNA methylomes reveal biological networks involved in human eye development, functions and associated disorders

2017

This work provides a comprehensive CpG methylation landscape of the different layers of the human eye that unveils the gene networks associated with their biological functions and how these are disrupted in common visual disorders. Herein, we firstly determined the role of CpG methylation in the regulation of ocular tissue-specification and described hypermethylation of retinal transcription factors (i.e., PAX6, RAX, SIX6) in a tissue-dependent manner. Second, we have characterized the DNA methylome of visual disorders linked to internal and external environmental factors. Main conclusions allow certifying that crucial pathways related to Wnt-MAPK signaling pathways or neuroinflammation are…

0301 basic medicineMaleADNlcsh:MedicineUllRetinal NeovascularizationEyeEpigenesis Genetic0302 clinical medicinelcsh:ScienceChildCàncerCancerRegulation of gene expressionMultidisciplinaryRetinoblastomaMelanomaMethylationDNA NeoplasmOphthalmopathiesNeoplasm ProteinsGene Expression Regulation NeoplasticOftalmologiaChild PreschoolDNA methylationFemaleMetilacióOftalmopatiesAdultMAP Kinase Signaling SystemBiologyMethylationArticle03 medical and health sciencesETS1medicineHumansEye ProteinsTranscription factorDiabetic RetinopathyEye Neoplasmslcsh:RDNADNA Methylationmedicine.diseaseeye diseasesOphthalmology030104 developmental biologyImmunology030221 ophthalmology & optometryCancer researchlcsh:QPAX6
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Further delineation of the clinical spectrum of de novo TRIM8 truncating mutations.

2018

IF 2.264; International audience; De novo mutations of the TRIM8 gene, which codes for a tripartite motif protein, have been identified using whole exome sequencing (WES) in two patients with epileptic encephalopathy (EE), but these reports were not sufficient to conclude that TRIM8 was a novel gene responsible for EE. Here we report four additional patients presenting with EE and de novo truncating mutations of TRIM8 detected by WES, and give further details of the patient previously reported by the Epi4K consortium. Epilepsy of variable severity was diagnosed in children aged 2 months to 3.5 years of age. All patients had developmental delay of variable severity with no or very limited la…

0301 basic medicineMaleAdolescentNerve Tissue ProteinsBioinformaticswhole exome sequencing03 medical and health sciencesEpilepsyTripartite MotifGeneticsmedicineHumansTRIM8Amino Acid SequenceChildGeneGenetics (clinical)De novo mutationsExome sequencingbusiness.industrynephrotic syndromeEpileptic encephalopathyInfant NewbornInfantmedicine.diseasePhenotype3. Good health030104 developmental biologyepileptic encephalopathy[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human geneticsChild PreschoolMutationFemalebusinessCarrier ProteinsNephrotic syndromeAmerican journal of medical genetics. Part A
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Impact of parental over- and underweight on the health of offspring.

2019

Parental excess weight and especially pregestational maternal obesity and excessive weight gain during pregnancy have been related to an increased risk of metabolic (obesity, type 2 diabetes, cardiovascular disease, metabolic syndrome) and nonmetabolic (cancer, osteoporosis, asthma, neurologic alterations) diseases in the offspring, probably mediated by epigenetic mechanisms of fetal programming. Maternal underweight is less common in developed societies, but the discrepancy between a poor nutritional environment in utero and a normal or excessive postnatal food supply with rapid growth catch-up appears to be the main candidate mechanism of the development of chronic diseases during the off…

0301 basic medicineMaleAdolescentOffspringHealth StatusMaternal HealthPhysiologyChild BehaviorDiseaseType 2 diabetesOverweightRisk Assessment03 medical and health sciences0302 clinical medicineChild DevelopmentChild of Impaired ParentsThinnessPregnancyRisk FactorsmedicineHumansChildPregnancy030219 obstetrics & reproductive medicinebusiness.industryAge FactorsChild HealthInfant NewbornPregnancy OutcomeObstetrics and GynecologyInfantAdolescent DevelopmentOverweightmedicine.diseaseObesityGestational Weight Gain030104 developmental biologyReproductive MedicineAdolescent BehaviorChild PreschoolFemaleDisease Susceptibilitymedicine.symptomUnderweightMetabolic syndromebusinessFertility and sterility
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Shared DNA methylation signatures in childhood allergy: The MeDALL study

2021

Contains fulltext : 232514.pdf (Publisher’s version ) (Open Access) BACKGROUND: Differential DNA methylation associated with allergy might provide novel insights into the shared or unique etiology of asthma, rhinitis, and eczema. OBJECTIVE: We sought to identify DNA methylation profiles associated with childhood allergy. METHODS: Within the European Mechanisms of the Development of Allergy (MeDALL) consortium, we performed an epigenome-wide association study of whole blood DNA methylation by using a cross-sectional design. Allergy was defined as having symptoms from at least 1 allergic disease (asthma, rhinitis, or eczema) and positive serum-specific IgE to common aeroallergens. The discove…

0301 basic medicineMaleAllergyMESH: Asthmalnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4]EczemaImmunoglobulin EEpigenesis GeneticCohort Studies0302 clinical medicineMESH: DNA MethylationMESH: ChildImmunology and AllergyMedicineMESH: Epigenesis GeneticChildMESH: CpG IslandsMESH: Cohort StudiesDNA methylationbiologyMESH: Immunoglobulin EEpigeneticMethylation3. Good healthCpG site030220 oncology & carcinogenesisChild PreschoolDNA methylationMESH: Rhinitis AllergicFemaleEpigeneticsIgEAdolescentMESH: HypersensitivityImmunologyeducationSingle-nucleotide polymorphismArticle03 medical and health sciencesMESH: Cross-Sectional StudieschildrenHypersensitivityHumansEpigeneticsAsthmaMESH: AdolescentMESH: Humansbusiness.industryMESH: TranscriptomeMESH: Child PreschoolImmunoglobulin Emedicine.diseaseallergyRhinitis AllergicAsthmaMESH: Male030104 developmental biologyCross-Sectional StudiesMESH: Eczema3121 General medicine internal medicine and other clinical medicineImmunologybiology.proteinCpG IslandsbusinessTranscriptomeMESH: Female[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
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Lipid Antigen Presentation by CD1b and CD1d in Lysosomal Storage Disease Patients

2019

The lysosome has a key role in the presentation of lipid antigens by CD1 molecules. While defects in lipid antigen presentation and in invariant Natural Killer T (iNKT) cell response were detected in several mouse models of lysosomal storage diseases (LSD), the impact of lysosomal engorgement in human lipid antigen presentation is poorly characterized. Here, we analyzed the capacity of monocyte-derived dendritic cells (Mo-DCs) from Fabry, Gaucher, Niemann Pick type C and Mucopolysaccharidosis type VI disease patients to present exogenous antigens to lipid-specific T cells. The CD1b- and CD1d-restricted presentation of lipid antigens by Mo-DCs revealed an ability of LSD patients to induce CD…

0301 basic medicineMaleAntigens CD1d / metabolismMucopolysaccharidosis type VIMonocytes / metabolismLysosomal Storage Diseases / diagnosisAntigens CD10302 clinical medicineAntigens CD1 / metabolismLysosomal storage diseaseImmunology and AllergyChildOriginal ResearchAged 80 and overAntigen PresentationbiologyKiller Cells Natural / metabolism*lipid antigen presentationAntigen Presentation / immunologyMiddle AgedNatural killer T cellLipidsnatural killer T cellsKiller Cells Naturalmedicine.anatomical_structureCD1DDendritic Cells / metabolismChild Preschool*dendritic cellsFemalelipids (amino acids peptides and proteins)*natural killer T cellsDisease SusceptibilitymonocytesAdultlcsh:Immunologic diseases. AllergyAdolescentT cellImmunologyCD1chemical and pharmacologic phenomenaCD1bLysosomal Storage Diseases / metabolismCD1dImmunophenotyping03 medical and health sciencesYoung AdultAntigenLysosomemedicineDendritic Cells / immunologyHumans*monocytesLymphocyte Countdendritic cellslysosomal storage diseasesLysosomal Storage Diseases / etiologyKiller Cells Natural / immunologyAgedbusiness.industry*CD1dInfantlipid antigen presentationmedicine.diseaseMonocytes / immunology*CD1b*lysosomal storage diseases030104 developmental biologyImmunologybiology.proteinAntigens CD1dbusinesslcsh:RC581-607Lipids / immunologyBiomarkers030215 immunology
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