Search results for "Genotype"

showing 10 items of 1725 documents

Molecular diagnosis of PIK3CA-related overgrowth spectrum (PROS) in 162 patients and recommendations for genetic testing.

2017

Postzygotic activating mutations of PIK3CA cause a wide range of mosaic disorders collectively referred to as PIK3CA-related overgrowth spectrum (PROS). We describe the diagnostic yield and characteristics of PIK3CA sequencing in PROS. We performed ultradeep next-generation sequencing (NGS) of PIK3CA in various tissues from 162 patients referred to our clinical laboratory and assessed diagnostic yield by phenotype and tissue tested. We identified disease-causing mutations in 66.7% (108/162) of patients, with mutant allele levels as low as 1%. The diagnostic rate was higher (74%) in syndromic than in isolated cases (35.5%; P = 9.03 × 10−5). We identified 40 different mutations and found stro…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyAdolescentGenotypeClass I Phosphatidylinositol 3-KinasesPrenatal diagnosisBioinformaticsmedicine.disease_causeDNA sequencing03 medical and health sciencesYoung Adult0302 clinical medicinePrenatal DiagnosisGenotypemedicineHumansGenetic Predisposition to DiseaseGenetic TestingAlleleChildGenetics (clinical)AllelesGenetic Association StudiesGrowth DisordersGenetic testingMutationmedicine.diagnostic_testbusiness.industryMosaicismInfant NewbornDisease ManagementHigh-Throughput Nucleotide SequencingInfantSequence Analysis DNAPhenotype030104 developmental biologyPhenotypeAmino Acid SubstitutionChild PreschoolMutationAllelic heterogeneityFemalebusiness030217 neurology & neurosurgeryGenetics in medicine : official journal of the American College of Medical Genetics
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Clinical course of sly syndrome (mucopolysaccharidosis type VII).

2016

WOS: 000377110800007

0301 basic medicineAdultMalePediatricsmedicine.medical_specialtyAdolescentMucopolysaccharidosisSly syndromeHepatosplenomegalyMetabolic disordersMucopolysaccharidosis VIIMedical and Health Sciences03 medical and health sciencesYoung Adult0302 clinical medicineHydrops fetalisSurveys and QuestionnairesmedicineGeneticsHumansMedical history1506Clinical geneticsFamily historyPreschoolChildGenetics (clinical)GlucuronidaseGenetics & Hereditybusiness.industryGenotype-Phenotype CorrelationsMucopolysaccharidosis VIIInfantEnzyme replacement therapyBiological Sciencesmedicine.diseaseLysosomal Storage Diseases030104 developmental biologyPhenotypeClinical genetics Genetics Metabolic disordersChild PreschoolFemalemedicine.symptombusiness030217 neurology & neurosurgeryMPS ; lysosomal storage disease ; β-glucuronidase
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Antibodies against Lewis antigens inhibit the binding of human norovirus GII.4 virus-like particles to saliva but not to intestinal Caco-2 cells.

2016

BACKGROUND: Human noroviruses (NoVs) are the main cause of gastroenteritis worldwide. The most commonly detected NoV strains belong to the genetically diverse GII.4 genotype, with new pandemic variants emerging periodically. Despite extensive efforts, NoV investigation has been hampered by the lack of an effective in vitro cell culture system. However, NoV-derived recombinant virus-like particles (VLPs) resembling empty capsids are good surrogates for analysing NoV antigenicity and virus-ligand interactions. NoV VLPs have been reported to bind to histo-blood group antigens (HBGAs). We have analysed the ability of NoV VLPs derived from GI.1 genotype and from three GII.4 genotype variants, GI…

0301 basic medicineAdultMaleSalivaAntigenicitymedicine.drug_classSwinevirusesVirus AttachmentMonoclonal antibodyImmunofluorescenceVirusMicrobiology03 medical and health sciencesLewis Blood Group Antigensfluids and secretionsAntigenstomatognathic systemGII.4 genotypeHisto-blood group antigens (HBGAs)VirologymedicineAnimalsHumansChildSalivaAutoantibodiesbiologymedicine.diagnostic_testResearchMucinNorovirusvirus diseasesEpithelial CellsMiddle AgedVirology3. Good healthVirus-like particles (VLPs)030104 developmental biologyInfectious DiseasesHuman norovirus (NoV)Host-Pathogen Interactionsbiology.proteinReceptors VirusReceptor bindingFemaleAntibodyCaco-2 Cells
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Relevance of secretor status genotype and microbiota composition in susceptibility to rotavirus and norovirus infections in humans

2017

Host genetic factors, such as histo-blood group antigens (HBGAs), are associated with susceptibility to norovirus (NoV) and rotavirus (RV) infections. Recent advances point to the gut microbiome as a key player necessary for a viral pathogen to cause infection. In vitro NoV attachment to host cells and resulting infections have been linked to interactions with certain bacterial types in the gut microbiota. We investigated the relationship between host genotype, gut microbiota, and viral infections. Saliva and fecal samples from 35 adult volunteers were analysed for secretor status genotype, the gut microbiota composition by 16S rRNA gene sequencing, and salivary IgA titers to NoV and RV. Hi…

0301 basic medicineAdultMaleSalivaGenotype030106 microbiologyResistanceMicrobiologiaGut floraHuman gut microbiomemedicine.disease_causeArticleRotavirus InfectionsMicrobiologyAssociation03 medical and health sciencesFecesfluids and secretionsBlood group antigensFut2 geneRotavirusGenotypemedicineHumansGenetic Predisposition to DiseaseDiseaseMicrobiomePolymorphismSalivaPathogenEcosystemCaliciviridae InfectionsMultidisciplinaryEnteric bacteriabiologyRuminococcusMiddle Agedbiology.organism_classificationFucosyltransferasesVirusGastrointestinal MicrobiomeGastroenteritis030104 developmental biologyImmunologyNorovirusFemale
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Characterisation of a household norovirus outbreak occurred in Valencia (Spain)

2016

Background Human noroviruses (NoVs) are the main cause of non-bacterial gastroenteritis worldwide. Several studies have linked human susceptibility to NoVs with the expression of histo-blood group antigens (HBGAs). In January 2012, a NoV gastroenteritis outbreak affected a household in Valencia, Spain, and the personal susceptibility to NoV was investigated. Methods To reach this aim 8 members of the affected household were recruited for this study and their secretor status, ABO and Lewis antigens were determined. NoV-specific saliva IgA and serum IgG antibody titers were analyzed. Their capacity to block viral binding to saliva receptors was analyzed, using virus-like particles (VLPs) of t…

0301 basic medicineAdultMaleSalivaGenotypeIgGFUT2030106 microbiologyHisto-blood group antigensBiologymedicine.disease_causeDisease Outbreaks03 medical and health sciencesSecretorFecesfluids and secretionsstomatognathic systemABO blood group systemGenotypemedicineHumansChildSalivaCaliciviridae InfectionsBlood typeBinding assayFamily CharacteristicsNorovirusAntibody titerOutbreakVirus-like particlesMiddle AgedVirologyGastroenteritis030104 developmental biologyInfectious DiseasesSusceptibilitySpainImmunologybiology.proteinNorovirusFemaleAntibodyIgAResearch ArticleBMC Infectious Diseases
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KIR2DL3 and the KIR ligand groups HLA-A-Bw4 and HLA-C2 predict the outcome of hepatitis B virus infection.

2017

Killer immunoglobulin-like receptors (KIRs) regulate the activation of Natural Killer cells through their interaction with human leukocyte antigens (HLA). KIR and HLA loci are highly polymorphic and certain HLA-KIR combinations have been found to protect against viral infections. In this study we analyzed whether the KIR/HLA repertoire may influence the course of hepatitis B virus (HBV) infection. Fifty-seven subjects with chronic hepatitis B (CHB), 44 subjects with resolved HBV infection, and 60 healthy uninfected controls (HC) were genotyped for KIR and their HLA ligands. The frequency of the HLA-A-Bw4 ligand group was higher in CHB (58%) than subjects with resolved infection (23%) (crude…

0301 basic medicineAdultMaleSettore MED/09 - Medicina InternaGenotypeSettore MED/17 - Malattie InfettiveKIR LigandHuman leukocyte antigenHLA-C AntigensBiologymedicine.disease_cause03 medical and health sciencesYoung Adult0302 clinical medicineHepatitis B ChronicVirologymedicineHumansGenetic Predisposition to DiseaseAlleleReceptorGeneAgedSettore MED/04 - Patologia GeneraleHepatitis B virusHepatologyHLA-A AntigensMiddle AgedVirologyHLA-AKIRs HLAA HBV030104 developmental biologyInfectious DiseasesTreatment OutcomeHLA-B AntigensReceptors KIR2DL3ImmunologyFemalePredictive variables030215 immunology
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The molecular epidemiology of HIV-1 in the Comunidad Valenciana (Spain): analysis of transmission clusters

2017

AbstractHIV infections are still a very serious concern for public heath worldwide. We have applied molecular evolution methods to study the HIV-1 epidemics in the Comunidad Valenciana (CV, Spain) from a public health surveillance perspective. For this, we analysed 1804 HIV-1 sequences comprising protease and reverse transcriptase (PR/RT) coding regions, sampled between 2004 and 2014. These sequences were subtyped and subjected to phylogenetic analyses in order to detect transmission clusters. In addition, univariate and multinomial comparisons were performed to detect epidemiological differences between HIV-1 subtypes, and risk groups. The HIV epidemic in the CV is dominated by subtype B i…

0301 basic medicineAdultMalemedicine.medical_specialtyAdolescentGenotypeHuman immunodeficiency virus (HIV)lcsh:MedicineHIV InfectionsBiologymedicine.disease_causeBioinformaticsArticleMen who have sex with menEvolution Molecular03 medical and health sciencesYoung AdultRisk groupsPublic health surveillanceRisk FactorsEpidemiologymedicineHumansEpidemiologialcsh:ScienceAgedMolecular EpidemiologyMultidisciplinaryPhylogenetic treeMolecular epidemiologyTransmission (medicine)lcsh:RMiddle Aged030104 developmental biologySpainPopulation SurveillanceHIV-1Femalelcsh:QMalalties de transmissió sexualReassortant VirusesDemographyScientific Reports
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Effect of gene-gene and gene-environment interactions associated with antituberculosis drug-induced hepatotoxicity.

2017

This study evaluated the association between environmental factors and genetic variations in enzymes that metabolize antituberculosis (anti-TB) drugs [arylamine N-acetyltransferase 2, cytochrome P450 2E1 (CYP2E1), glutathione S-transferase theta 1 (GSTT1), and glutathione S-transferase mu 1] with antituberculosis drug-induced hepatotoxicity (ATDH). We also investigated the potential gene-gene and gene-environment interactions as well as their association with ATDH development in a population of hospitalized TB patients from Buenos Aires.We investigated 364 TB patients who received anti-TB drugs. Physicians collected demographic and clinical data to identify environmental risk factors for AT…

0301 basic medicineAdultMalemedicine.medical_specialtyAntitubercular AgentsBiologyPharmacologyPolymorphism Single Nucleotide03 medical and health scienceschemistry.chemical_compoundYoung Adult0302 clinical medicineMolecular geneticsGenotypeGenetic variationGeneticsmedicineHumansGeneral Pharmacology Toxicology and PharmaceuticsAlleleMolecular BiologyGeneGenetics (clinical)chemistry.chemical_classificationEpistasis GeneticGlutathioneCYP2E1gene antitubercolosis drug drug cytochrome geneticsSettore BIO/18 - Genetica030104 developmental biologyEnzymechemistryLiver030220 oncology & carcinogenesisMolecular MedicineFemalePharmacogenetics and genomics
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When do myopia genes have their effect? Comparison of genetic risks between children and adults

2016

Item does not contain fulltext Previous studies have identified many genetic loci for refractive error and myopia. We aimed to investigate the effect of these loci on ocular biometry as a function of age in children, adolescents, and adults. The study population consisted of three age groups identified from the international CREAM consortium: 5,490 individuals aged 25 years. All participants had undergone standard ophthalmic examination including measurements of axial length (AL) and corneal radius (CR). We examined the lead SNP at all 39 currently known genetic loci for refractive error identified from genome-wide association studies (GWAS), as well as a combined genetic risk score (GRS). …

0301 basic medicineAdultMalemedicine.medical_specialtyBiometryAdolescentGenotypeEpidemiologySingle-nucleotide polymorphismGenome-wide association studyBiologyPolymorphism Single NucleotideConnexinsSensory disorders Donders Center for Medical Neuroscience [Radboudumc 12]03 medical and health sciencesYoung Adult0302 clinical medicineRisk FactorsInternal medicineGenotypemedicineMyopiaSNPHumansAlleleYoung adult610 Medicine & healthChildGenetics (clinical)AllelesGenetic associationGenetics030104 developmental biologyGenetic Loci030221 ophthalmology & optometryPopulation studyFemaleRELamininGenome-Wide Association Study
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Vitamin D receptor polymorphisms and 25-hydroxyvitamin D in a group of Sicilian multiple sclerosis patients

2016

Multiple sclerosis (MS) is an auto-immune disease whose etiology remains controversial. Both genetic and environmental factors are thought to be involved in the risk of developing the disease. The purpose of our study was to assess the association of Vitamin D receptor (VDR) polymorphisms with MS and to investigate the interaction of these polymorphisms with vitamin D levels. A total of 179 Sicilian subjects, including 104 MS patients and 75 healthy controls, were studied. The most common VDR polymorphisms (Fok-I, Bsm-I, Taq-I and Apa-I) were genotyped by polymerase chain reaction (PCR) followed by restriction fragment length polymorphism (RFLP) analyses in both groups and serum 25-hydroxyv…

0301 basic medicineAdultMalemedicine.medical_specialtyMultiple SclerosisGenotypeVDR polymorphismsDermatologyCalcitriol receptor25(OH)D; Multiple sclerosis; VDR polymorphisms; Vitamin D; Adult; Female; Gene Frequency; Genotype; Humans; Male; Middle Aged; Multiple Sclerosis; Receptors Calcitriol; Sicily; Vitamin D; Polymorphism Restriction Fragment Length03 medical and health sciences0302 clinical medicineGene FrequencyCalcitriolInternal medicineGenotypeReceptorsmedicineGenetic predispositionVitamin D and neurologyHumansMultiple sclerosiAlleleVitamin DPolymorphismAllele frequencySicilyVDR25(OH)Dbusiness.industryMultiple sclerosisGeneral MedicineMiddle Agedmedicine.diseaseVitamin D 25(OH)DPsychiatry and Mental healthSettore BIO/12 - Biochimica Clinica E Biologia Molecolare Clinica030104 developmental biologyEndocrinologyRestriction Fragment LengthImmunologyReceptors CalcitriolSettore MED/26 - NeurologiaFemaleNeurology (clinical)Restriction fragment length polymorphismbusiness030217 neurology & neurosurgeryPolymorphism Restriction Fragment Length
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