Search results for "Polymorphism"

showing 10 items of 1968 documents

Generation of a disease-specific iPS cell line derived from a patient with Charcot-Marie-Tooth type 2K lacking functional GDAP1 gene

2016

Human CMT2-FiPS4F1 cell line was generated from fibroblasts of a patient with Charcot-Marie-Tooth disease harbouring the following mutations in the GDAP1 gene in heterozygosis: p.Q163X/p.T288NfsX3. This patient did not present mutations in the PM22, MPZ or GJB genes. Human reprogramming factors OCT3/4, KLF4, SOX2 and C-MYC were delivered using a non-integrative methodology that involves the use of Sendai virus.

0301 basic medicineMaleHeterozygoteCellular differentiationCèl·lulesDNA Mutational AnalysisGenetic VectorsInduced Pluripotent Stem CellsKaryotypeNerve Tissue ProteinsBiologyPolymorphism Single NucleotideSendai virusCell Line03 medical and health sciencesKruppel-Like Factor 4stomatognathic systemCharcot-Marie-Tooth DiseaseHumansInduced pluripotent stem cellGeneTranscription factorMedicine(all)GeneticsBase SequenceHeterozygote advantageCell DifferentiationCell BiologyGeneral MedicineFibroblastsbiology.organism_classificationCellular ReprogrammingSendai virus030104 developmental biologyMicroscopy FluorescenceKLF4embryonic structuresSistema nerviós MalaltiesReprogrammingDevelopmental BiologyTranscription Factors
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The Effect of a Novel c.820C>T (Arg274Trp) Mutation in the Mitofusin 2 Gene on Fibroblast Metabolism and Clinical Manifestation in a Patient

2017

Charcot-Marie-Tooth disease type 2A (CMT2A) is an autosomal dominant axonal peripheral neuropathy caused by mutations in the mitofusin 2 gene (MFN2). Mitofusin 2 is a GTPase protein present in the outer mitochondrial membrane and responsible for regulation of mitochondrial network architecture via the fusion of mitochondria. As that fusion process is known to be strongly dependent on the GTPase activity of mitofusin 2, it is postulated that the MFN2 mutation within the GTPase domain may lead to impaired GTPase activity, and in turn to mitochondrial dysfunction. The work described here has therefore sought to verify the effects of MFN2 mutation within its GTPase domain on mitochondrial and e…

0301 basic medicineMaleHydrolasesMutantMFN2lcsh:MedicineGTPaseMitochondrionmedicine.disease_causeEndoplasmic ReticulumBiochemistryGTP Phosphohydrolases0302 clinical medicineMental RetardationAnimal CellsCharcot-Marie-Tooth DiseaseMedicine and Health SciencesMissense mutationlcsh:ScienceEnergy-Producing OrganellesCells CulturedConnective Tissue CellsGeneticsMutationMultidisciplinarySecretory PathwayOrganic CompoundsMonosaccharidesTryptophanMitochondrial DNACell biologyMitochondriaEnzymesNucleic acidsChemistryNeurologyConnective TissueCell ProcessesPhysical SciencesCellular Structures and OrganellesCellular TypesAnatomyResearch ArticleForms of DNACarbohydratesMutation MissenseBiologyBioenergeticsArgininePolymorphism Single NucleotideMitochondrial Proteins03 medical and health sciencesMitofusin-2Young AdultmedicineGeneticsHumansEndoplasmic reticulumlcsh:ROrganic ChemistryChemical CompoundsBiology and Life SciencesProteinsCell BiologyDNAFibroblastsGuanosine Triphosphatase030104 developmental biologyBiological TissueGlucoseAmino Acid SubstitutionCase-Control StudiesMutationEnzymologylcsh:Q030217 neurology & neurosurgeryPLoS ONE
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Genome-Wide Association Study of the Modified Stumvoll Insulin Sensitivity Index Identifies BCL2 and FAM19A2 as Novel Insulin Sensitivity Loci.

2016

Genome-wide association studies (GWAS) have found few common variants that influence fasting measures of insulin sensitivity. We hypothesized that a GWAS of an integrated assessment of fasting and dynamic measures of insulin sensitivity would detect novel common variants. We performed a GWAS of the modified Stumvoll Insulin Sensitivity Index (ISI) within the Meta-Analyses of Glucose and Insulin-Related Traits Consortium. Discovery for genetic association was performed in 16,753 individuals, and replication was attempted for the 23 most significant novel loci in 13,354 independent individuals. Association with ISI was tested in models adjusted for age, sex, and BMI and in a model analyzing t…

0301 basic medicineMaleInsulin Receptor Substrate ProteinsEndocrinology Diabetes and MetabolismLocus (genetics)Genome-wide association studyPolymorphism Single Nucleotide03 medical and health sciencesEndocrinology & MetabolismInsulin resistanceGenotypeInternal MedicinemedicineHumansGenetic Predisposition to DiseaseGenetic associationGeneticsbusiness.industryInsulin sensitivityGenetics/Genomes/Proteomics/Metabolomics11 Medical And Health Sciencesmedicine.diseaseIRS1030104 developmental biologyProto-Oncogene Proteins c-bcl-2Chemokines CCInsulin Receptor Substrate ProteinsFemaleInsulin ResistancebusinessGenome-Wide Association Study
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Clinical response to non-surgical periodontal treatment in patients with interleukin-6 and interleukin-10 polymorphisms

2017

Background Genetic polymorphisms are commonly associated with altered transcriptional activity and possibly make individuals more susceptible to periodontal disease development, increased disease severity and poor treatment outcome. The study aimed to determine the effect of Interleukin-6 (IL-6) -572 G/C (rs1800796) and IL-10 -592 C/A (rs1800872) polymorphisms on the outcomes of non-surgical periodontal therapy in a Caucasian population. Material and Methods Sixty-eight patients with chronic periodontal disease were grouped according to their genotype: IL-6, IL-10, IL-6 and IL-10 susceptible (SCP) and non-susceptible (NSCP). All individuals were clinically evaluated at the first visit, and …

0301 basic medicineMaleLongitudinal studymedicine.medical_specialtyGenotype03 medical and health sciences0302 clinical medicineInternal medicineGenotypeMedicineHumansLongitudinal StudiesAlleleInterleukin 6General DentistryGenotypingPeriodontal DiseasesOral Medicine and PathologyPolymorphism Geneticbiologybusiness.industryInterleukin-6ResearchInterleukin030206 dentistryMiddle Aged:CIENCIAS MÉDICAS [UNESCO]SurgeryInterleukin-10030104 developmental biologyTreatment OutcomeOtorhinolaryngologyInclusion and exclusion criteriaChronic DiseaseUNESCO::CIENCIAS MÉDICASbiology.proteinSurgeryFemaleGene polymorphismbusiness
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MicroRNAs and Drinking : Association between the Pre-miR-27a rs895819 Polymorphism and Alcohol Consumption in a Mediterranean Population

2016

Recently, microRNAs (miRNA) have been proposed as regulators in the different processes involved in alcohol intake, and differences have been found in the miRNA expression profile in alcoholics. However, no study has focused on analyzing polymorphisms in genes encoding miRNAs and daily alcohol consumption at the population level. Our aim was to investigate the association between a functional polymorphism in the pre-miR-27a (rs895819 A>G) gene and alcohol consumption in an elderly population. We undertook a cross-sectional study of PREvención con DIeta MEDiterránea (PREDIMED)-Valencia participants (n = 1007, including men and women aged 67 7 years) and measured their alcohol consumption (to…

0301 basic medicineMaleMicro RNAsMediterranean dietCross-sectional studyPhysiologyAlcoholmiR27aMediterraneanCOLORECTAL-CANCERFUNCTIONAL POLYMORPHISMlcsh:Chemistrychemistry.chemical_compoundPolymorphism (computer science)GenotypeMedicineMolecular geneticslcsh:QH301-705.5SpectroscopyGeneticsRISKeducation.field_of_studyMediterranean RegionalcoholGeneral MedicineMiddle AgedComputer Science ApplicationsmicroRNAsDrinking of alcoholic beveragesSINGLE NUCLEOTIDE POLYMORPHISMSMENDELIAN RANDOMIZATIONMir27aConsum d'alcoholFemaleAlcoholAlcohol DrinkingGenotypePopulationGENETIC VARIANTHEART-DISEASEPolymorphism Single NucleotideCatalysisArticleGenètica molecularInorganic Chemistry03 medical and health sciencesMediterranean cookingUSE DISORDERSmicroRNACuina mediterràniaHumansPhysical and Theoretical ChemistryeducationMolecular BiologyAgedCHINESE POPULATIONbusiness.industryOrganic ChemistrymicroRNAs; alcohol; miR27a; Mediterraneanmedicine.diseaseObesityMicroRNAs030104 developmental biologyCross-Sectional Studieschemistrylcsh:Biology (General)lcsh:QD1-999GASTRIC-CANCER SUSCEPTIBILITYbusiness
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MICA-129 Dimorphism and Soluble MICA Are Associated With the Progression of Multiple Myeloma

2018

Natural killer (NK) cells are immune innate effectors playing a pivotal role in the immunosurveillance of multiple myeloma (MM) since they are able to directly recognize and kill MM cells. In this regard, among activating receptors expressed by NK cells, NKG2D represents an important receptor for the recognition of MM cells, being its ligands expressed by tumor cells, and being able to trigger NK cell cytotoxicity. The MHC class I-related molecule A (MICA) is one of the NKG2D ligands; it is encoded by highly polymorphic genes and exists as membrane-bound and soluble isoforms. Soluble MICA (sMICA) is overexpressed in the serum of MM patients, and its levels correlate with tumor progression. …

0301 basic medicineMaleModels MolecularProtein ConformationGenotypeImmunology and AllergyReceptorpredictive biomarkerOriginal ResearchAged 80 and overnatural killer cellsbiologyChemistryMiddle AgedImmunosurveillanceGene Expression Regulation NeoplasticKiller Cells Naturalmultiple myelomaNK Cell Lectin-Like Receptor Subfamily KDisease ProgressionFemaleNKG2D receptorProtein Bindinglcsh:Immunologic diseases. AllergyGenotypeImmunologyEnzyme-Linked Immunosorbent AssayMICA polymorphismImmunophenotyping03 medical and health sciencesStructure-Activity RelationshipImmune systemMHC class IHumansGenetic Predisposition to DiseaseAllelesGenetic Association StudiesAgedPolymorphism GeneticHistocompatibility Antigens Class INKG2DMolecular biologyMolecular Typingstomatognathic diseases030104 developmental biologyAmino Acid SubstitutionTumor progressionbiology.proteinmultiple myeloma natural killer cells NKG2D receptor MICA polymorphism predictive biomarkerGene polymorphismlcsh:RC581-607Frontiers in Immunology
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Holocene Selection for Variants Associated With General Cognitive Ability: Comparing Ancient and Modern Genomes

2017

Human populations living during the Holocene underwent considerable microevolutionary change. It has been theorized that the transition of Holocene populations into agrarianism and urbanization brought about culture-gene co-evolution that favored via directional selection genetic variants associated with higher general cognitive ability (GCA). To examine whether GCA might have risen during the Holocene, we compare a sample of 99 ancient Eurasian genomes (ranging from 4.56 to 1.21 kyr BP) with a sample of 503 modern European genomes (Fst= 0.013), using three different cognitive polygenic scores (130 SNP, 9 SNP and 11 SNP). Significant differences favoring the modern genomes were found for al…

0301 basic medicineMaleMultifactorial InheritanceSingle-nucleotide polymorphismBiologyGenomePolymorphism Single NucleotideEvolution Molecular03 medical and health sciences0302 clinical medicineCognitionSNPHumansAlleleSelection GeneticGenetics (clinical)Selection (genetic algorithm)HoloceneModels GeneticDirectional selectionGenome HumanObstetrics and GynecologyOdds ratio030104 developmental biologyEvolutionary biologyPediatrics Perinatology and Child HealthFemale030217 neurology & neurosurgery
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Microevolution of bank voles (Myodes glareolus) at neutral and immune-related genes during multiannual dynamic cycles: consequences for Puumala hanta…

2017

ABSTRACTUnderstanding how host dynamics, including variations of population size and dispersal, may affect the epidemiology of infectious diseases through ecological and evolutionary processes is an active research area. Here we focus on a bank vole (Myodes glareolus) metapopulation surveyed in Finland between 2005 and 2009. Bank vole is the reservoir of Puumala hantavirus (PUUV), the agent of nephropathia epidemica (NE, a mild form of hemorrhagic fever with renal symptom) in humans.M glareoluspopulations experience multiannual density fluctuations that may influence the level of genetic diversity maintained in bank voles, PUUV prevalence and NE occurrence. We examine bank vole metapopulati…

0301 basic medicineMaleMyxovirus Resistance Proteins0106 biological sciencesSELECTIONPopulation geneticsPopulation DynamicsGene ExpressionPopulation geneticsNATURAL-POPULATIONPuumala virus01 natural sciencesRodent DiseasesNephropathia epidemicaFinlandGeneticsMolecular Epidemiology0303 health scienceseducation.field_of_studyRodentArvicolinaeMicroevolutionBiological EvolutionBank voleInfectious DiseasesMHC DIVERSITYHemorrhagic Fever with Renal SyndromeHost-Pathogen Interactions[SDV.IMM]Life Sciences [q-bio]/ImmunologyFemaleDisease SusceptibilityDensity fluctuationsMicrobiology (medical)Gene FlowPopulationMetapopulationBiologyMicrobiology010603 evolutionary biology03 medical and health sciencesGenetic driftGeneticsmedicineImmunogeneticsAnimalsHumansPOPULATION-STRUCTUREAdaptationeducationMolecular BiologyEcology Evolution Behavior and SystematicsDisease Reservoirs030304 developmental biologyTOLL-LIKE RECEPTORSHost-pathogen interactionPolymorphism Genetic[SDV.GEN.GPO]Life Sciences [q-bio]/Genetics/Populations and Evolution [q-bio.PE]MX2 PROTEINGenetic DriftNECROSIS-FACTOR-ALPHADENSITY-FLUCTUATIONSmedicine.diseasebiology.organism_classificationEUROPEAN ROE DEERToll-Like Receptor 4030104 developmental biologyToll-Like Receptor 7Evolutionary biology3121 General medicine internal medicine and other clinical medicineVoleRODENT HOST[SDV.EE.IEO]Life Sciences [q-bio]/Ecology environment/Symbiosis
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A Genome-Wide Association Meta-Analysis of Attention-Deficit/Hyperactivity Disorder Symptoms in Population-Based Pediatric Cohorts

2016

OBJECTIVE: The aims of this study were to elucidate the influence of common genetic variants on childhood attention-deficit/hyperactivity disorder (ADHD) symptoms, to identify genetic variants that explain its high heritability, and to investigate the genetic overlap of ADHD symptom scores with ADHD diagnosis. METHOD: Within the EArly Genetics and Lifecourse Epidemiology (EAGLE) consortium, genome-wide single nucleotide polymorphisms (SNPs) and ADHD symptom scores were available for 17,666 children (<13 years of age) from nine population-based cohorts. SNP-based heritability was estimated in data from the three largest cohorts. Meta-analysis based on genome-wide association (GWA) analyses w…

0301 basic medicineMaleNetherlands Twin Register (NTR)attention problemsPopulation/methodsCHILDHOODAdhd Symptoms ; Gwa ; Snp Heritability ; Attention Problems ; Meta-analysisGenome-wide association studyCHILDRENCohort Studies0302 clinical medicineDevelopmental and Educational PsychologyGENETIC INFLUENCESNETHERLANDS TWIN REGISTERChildGeneticsRISKeducation.field_of_studyGenetics Population/methods3. Good healthPsychiatry and Mental healthPERSONALITY CONSORTIUM/dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_beingFemalePsychologyAttention Deficit Disorder with Hyperactivity/geneticsAdolescentDEFICIT HYPERACTIVITY DISORDERPopulationSingle-nucleotide polymorphismGWAPROFILEGenetic correlationADHD symptomsArticle150 000 MR Techniques in Brain FunctionSNP heritabilityBEHAVIOR PROBLEMS03 medical and health sciencesSDG 3 - Good Health and Well-beingmedicine/dk/atira/pure/keywords/cohort_studies/netherlands_twin_register_ntr_GeneticsSNPAttention deficit hyperactivity disorderADHDHumanseducationGenetic associationNeurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7]Heritabilitymedicine.diseasemeta-analysis030104 developmental biologyGenetics PopulationTrastorn per dèficit d'atenció amb hiperactivitatAttention Deficit Disorder with HyperactivityCase-Control Studies030217 neurology & neurosurgeryGenèticaGenome-Wide Association Study
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Combined effects of the PNPLA3 rs738409, TM6SF2 rs58542926, and MBOAT7 rs641738 variants on NAFLD severity: a multicenter biopsy-based study.

2016

The PNPLA3 p.I148M, TM6SF2 p.E167K, and MBOAT7 rs641738 variants represent genetic risk factors for nonalcoholic fatty liver disease (NAFLD). Here we investigate if these polymorphisms modulate both steatosis and fibrosis in patients with NAFLD. We recruited 515 patients with NAFLD (age 16–88 years, 280 female patients). Liver biopsies were performed in 320 patients. PCR-based assays were used to genotype the PNPLA3, TM6SF2, and MBOAT7 variants. Carriers of the PNPLA3 and TM6SF2 risk alleles showed increased serum aspartate aminotransferase and alanine transaminase activities (P 0.05). The MBOAT7 variant was solely associated with increased fibrosis (P = 0.046). In the multivariate model, v…

0301 basic medicineMalePathologyBiopsyBiochemistryGastroenterologySeverity of Illness Index0302 clinical medicineEndocrinologyFibrosisNon-alcoholic Fatty Liver DiseaseGenotypeNonalcoholic fatty liver diseaseAged 80 and overeducation.field_of_studybiologyFatty liverMiddle AgedLiver030211 gastroenterology & hepatologyFemaleAdultmedicine.medical_specialtyAdolescentGenotypePolymorphism Single Nucleotide03 medical and health sciencesYoung AdultInternal medicinemedicineHumansAdiponutrinGenetic Predisposition to DiseaseeducationAllelesGenetic Association StudiesAgedbusiness.industryMembrane ProteinsCell BiologyLipasemedicine.diseaseFibrosisFatty Liver030104 developmental biologyAlanine transaminasebiology.proteinSteatosisbusinessPatient-Oriented and Epidemiological ResearchAcyltransferasesTM6SF2Journal of lipid research
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