Search results for "genomics"

showing 10 items of 1255 documents

Mutations in the gene encoding the basal body protein RPGRIP1L, a nephrocystin-4 interactor, cause Joubert syndrome.

2007

Peters, T.A./0000-0001-8443-5500; van Beersum, Sylvia E.C./0000-0002-4552-2908; Cremers, Frans/0000-0002-4954-5592; Roepman, Ronald/0000-0002-5178-8163 WOS: 000247619800019 PubMed: 17558407 Protein- protein interaction analyses have uncovered a ciliary and basal body protein network that, when disrupted, can result in nephronophthisis ( NPHP), Leber congenital amaurosis, Senior- Loken syndrome ( SLSN) or Joubert syndrome ( JBTS)(1-6). However, details of the molecular mechanisms underlying these disorders remain poorly understood. RPGRIP1- like protein ( RPGRIP1L) is a homolog of RPGRIP1 ( RPGR-interacting protein 1), a ciliary protein defective in Leber congenital amaurosis(7,8). We show t…

AdultMaleHealth aging / healthy living [IGMD 5]Eye DiseasesGenetics and epigenetic pathways of disease [NCMLS 6]TMEM67Molecular Sequence DataMembrane transport and intracellular motility [NCMLS 5]Biologymedicine.disease_causeJoubert syndromeCell LineGenomic disorders and inherited multi-system disorders [IGMD 3]NephronophthisisCerebellar DiseasesGeneticsmedicinePerception and Action [DCN 1]Basal bodyAnimalsHumansNeurosensory disorders [UMCN 3.3]CiliaAdaptor Proteins Signal TransducingRenal disorder [IGMD 9]GeneticsMutationCiliumCiliary transition zoneProteinsSyndromemedicine.diseasePedigreeRatsCytoskeletal ProteinsGenetic defects of metabolism [UMCN 5.1]RPGRIP1LFemaleKidney DiseasesFunctional Neurogenomics [DCN 2]Ciliary Motility Disorders
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Perilipin polymorphism interacts with saturated fat and carbohydrates to modulate insulin resistance

2010

Macronutrient intakes and genetic variants have been shown to interact to alter the risk of insulin resistance, but replication of gene-nutrient interactions across independent populations may be difficult to achieve. Saturated fat and carbohydrate were previously shown to interact with genotype for insulin resistance for a variant of perilipin (PLIN), the major adipocyte-associated protein and a regulator of adipocyte metabolism. We investigated the same interaction for PLIN 11482G>A (rs894160) on insulin resistance in US men(n=462) and women(n=508) (mean ±SD, 49±16 years). In multivariate linear regression models, we found a significant (PA for HOMA-IR (homeostasis model assessment of ins…

AdultMaleHeterozygotePerilipin-1medicine.medical_specialtyAdolescentMinnesotaEndocrinology Diabetes and MetabolismSaturated fatmedicine.medical_treatmentMedicine (miscellaneous)BiologyPolymorphism Single NucleotideWhite PeopleArticleYoung Adultchemistry.chemical_compoundNutrigenomicsInsulin resistanceUtahAdipocyteInternal medicineDietary CarbohydratesmedicineHumansInsulinGenetic Association StudiesAgedAged 80 and overSex CharacteristicsNutrition and DieteticsInsulinMetabolismMiddle AgedCarbohydratePhosphoproteinsmedicine.diseaseDietary FatsEndocrinologychemistryPerilipinFemaleInsulin ResistanceCarrier ProteinsCardiology and Cardiovascular MedicineHomeostasisNutrition, Metabolism and Cardiovascular Diseases
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REEP1 mutation spectrum and genotype/phenotype correlation in hereditary spastic paraplegia type 31.

2008

Contains fulltext : 71291.pdf (Publisher’s version ) (Closed access) Mutations in the receptor expression enhancing protein 1 (REEP1) have recently been reported to cause autosomal dominant hereditary spastic paraplegia (HSP) type SPG31. In a large collaborative effort, we screened a sample of 535 unrelated HSP patients for REEP1 mutations and copy number variations. We identified 13 novel and 2 known REEP1 mutations in 16 familial and sporadic patients by direct sequencing analysis. Twelve out of 16 mutations were small insertions, deletions or splice site mutations. These changes would result in shifts of the open-reading-frame followed by premature termination of translation and haploins…

AdultMaleMutation rateAdolescentGenotypeHereditary spastic paraplegiaDNA Mutational AnalysisBiologymedicine.disease_causeArticleCognitive neurosciences [UMCN 3.2]Gene duplicationGenotypemedicinePerception and Action [DCN 1]HumansCopy-number variationAge of OnsetMutation frequencyChildAgedAged 80 and overGeneticsMutationHereditary cancer and cancer-related syndromes [ONCOL 1]Spastic Paraplegia HereditaryInfantMembrane Transport ProteinsMiddle Agedmedicine.diseasePedigreePhenotypeChild PreschoolMutationFemaleNeurology (clinical)HaploinsufficiencyFunctional Neurogenomics [DCN 2]
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Variation in genomic landscape of clear cell renal cell carcinoma across Europe

2014

The incidence of renal cell carcinoma (RCC) is increasing worldwide, and its prevalence is particularly high in some parts of Central Europe. Here we undertake whole-genome and transcriptome sequencing of clear cell RCC (ccRCC), the most common form of the disease, in patients from four different European countries with contrasting disease incidence to explore the underlying genomic architecture of RCC. Our findings support previous reports on frequent aberrations in the epigenetic machinery and PI3K/mTOR signalling, and uncover novel pathways and genes affected by recurrent mutations and abnormal transcriptome patterns including focal adhesion, components of extracellular matrix (ECM) and …

AdultMaleOncogene Proteins FusionRNA SplicingGeneral Physics and AstronomyBiologymedicine.disease_causeGeneral Biochemistry Genetics and Molecular BiologyCohort StudiesTranscriptomePhosphatidylinositol 3-KinasesMutation RatemedicineHumansEpigeneticsCarcinoma Renal CellAgedAged 80 and overGeneticsFocal AdhesionsMutationMultidisciplinaryGenome HumanGene Expression ProfilingGenetic VariationGenomicsSequence Analysis DNAGeneral ChemistryMiddle Agedmedicine.diseaseHuman genetics3. Good healthEuropeGene Expression Regulation NeoplasticGene expression profilingClear cell renal cell carcinomaMutationFemaleCarcinogenesisClear cellSignal TransductionNature Communications
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Pro-inflammatory gene variants in myocardial infarction and longevity: implications for pharmacogenomics.

2008

Inflammation and genetics play an important role in the pathogenesis of coronary heart disease (CHD). However, despite the increasing appreciation of the role of genetics in CHD and myocardial infarction (MI) pathogenesis, pharmacogenomic approaches to uncover drug target have not been extensively explored. Cyclo-oxygenases (COXs) and 5-lipoxygenase (5-LO) are the key enzymes in the conversion of arachidonic acid to prostaglandins (PG) and leukotrienes (LT) and are implicated in a wide variety of inflammatory disorders, including atherosclerosis. In fact, PGE2 activates Matrix Metallo-proteinases whereas LTB4 is a chemoactractant for monocytes and activates gene expression in inflammatory c…

AdultMalePathologymedicine.medical_specialtymedia_common.quotation_subjectLongevityMyocardial InfarctionIMMUNOGENETICSINFARCTIONINFLAMMATIONLONGEVITYPHARMACOGENOMICSInflammationDiseaseBioinformaticsPathogenesisYoung AdultDrug Delivery SystemsRisk FactorsDrug DiscoverymedicineHumansGenetic Predisposition to DiseaseMyocardial infarctionAlleleAllelesmedia_commonAged 80 and overInflammationPharmacologyArachidonate 5-Lipoxygenasebusiness.industryAge FactorsLongevityMiddle Agedmedicine.diseasePhenotypeCyclooxygenase 2PharmacogeneticsPharmacogenomicsFemalemedicine.symptombusinessPharmacogenetics
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Examining facial emotion recognition as an intermediate phenotype for psychosis: Findings from the EUGEI study

2022

The EUGEI project was supported by the European Community’s Seventh Framework Program under grant agreement No. HEALTH-F2- 2009-241909 (Project EU-GEI). Dr. Arango was supported by the Spanish Ministry of Science and Innovation; Instituto de Salud Carlos III (SAM16-PE07CP1, PI16/02012, PI19/024); CIBERSAM (...)

AdultMalePsychosisGENETIC RISKInterviews as Topic03 medical and health sciencesSTRUCTURED INTERVIEW0302 clinical medicinePolygenic risk scoreRisk FactorsSocial cognitionIMPUTATIONmedicineHumansPOLYGENIC RISKEmotion recognitionAssociation (psychology)Biological PsychiatryEmotionPharmacologyIntermediate phenotypebusiness.industrySiblingsUNAFFECTED SIBLINGSRegression analysisASSOCIATIONGenomicsmedicine.diseaseSocial cognition030227 psychiatrySchizotypal traitsINDIVIDUALSPolygenic risk scoresPhenotypePsychotic DisordersSchizophreniaRELIABILITYStructured interviewSchizophreniaFemalebusinessFacial Recognition030217 neurology & neurosurgeryClinical psychologyProgress in Neuro-Psychopharmacology and Biological Psychiatry
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Genetic risk prediction and neurobiological understanding of alcoholism.

2014

We have used a translational Convergent Functional Genomics (CFG) approach to discover genes involved in alcoholism, by gene-level integration of genome-wide association study (GWAS) data from a German alcohol dependence cohort with other genetic and gene expression data, from human and animal model studies, similar to our previous work in bipolar disorder and schizophrenia. A panel of all the nominally significant P-value SNPs in the top candidate genes discovered by CFG  (n=135 genes, 713 SNPs) was used to generate a genetic  risk prediction score (GRPS), which showed a trend towards significance (P=0.053) in separating  alcohol dependent individuals from controls in an independent German…

AdultMaleRiskCandidate geneAlcohol abuseContext (language use)Single-nucleotide polymorphismGenome-wide association studyBioinformaticsPolymorphism Single NucleotideMice03 medical and health sciencesCellular and Molecular Neuroscience0302 clinical medicineGermanyAnimalsHumansMedicineGenetic Predisposition to DiseaseBiological Psychiatry030304 developmental biologyMice KnockoutGenetics0303 health sciencesbusiness.industryAlcohol dependenceGenomics16. Peace & justicemedicine.diseaseUnited States3. Good healthAlcoholismDisease Models AnimalPsychiatry and Mental healthBehavioral medicineCohortOriginal ArticleFemaleCorrigendumbusiness030217 neurology & neurosurgeryGenome-Wide Association Study
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Active and secreted IgA-coated bacterial fractions from the human gut reveal an under-represented microbiota core

2013

AbstractHost-associated microbiota varies in distribution depending on the body area inhabited. Gut microbes are known to interact with the human immune system, maintaining gut homoeostasis. Thus, we studied whether secreted-IgA (S-IgA) coat specific microbial taxa without inducing strong immune responses. To do so, we fractionated gut microbiota by flow cytometry. We found that active and S-IgA-coated bacterial fractions were characterized by a higher diversity than those observed in raw faecal suspensions. A long-tail effect was observed in family distribution, revealing that rare bacteria represent up to 20% of total diversity. While Firmicutes was the most abundant phylum, the majority …

AdultMaleSequence analysisFirmicutesGut floraArticleMicrobiologyYoung AdultImmune systemDNA Barcoding TaxonomicHumansMultidisciplinaryBacteriabiologyPhylumMicrobiotaComputational BiologyBiodiversitySequence Analysis DNAbiology.organism_classificationImmunoglobulin AGastrointestinal TractSphingomonadaceaeMetagenomicsMetagenomeFemaleBacteriaScientific Reports
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The experience of physicians in pharmacogenomic clinical decision support within eight German university hospitals.

2017

Aim: The aim of this study was to assess the physicians’ attitude, their knowledge and their experience in pharmacogenomic clinical decision support in German hospitals. Materials & methods: We conducted an online survey to address physicians of 13 different medical specialties across eight German university hospitals. In total, 564 returned questionnaires were analyzed. Results: The remaining knowledge gap, the uncertainty of test reimbursement and the physicians’ lack of awareness of existing pharmacogenomic clinical decision support systems (CDSS) are the major barriers for implementing pharmacogenomic CDSS into German hospitals. Furthermore, pharmacogenomic CDSS are most effective …

AdultMalemedicine.medical_specialtyDecision support systemAttitude of Health PersonnelClinical decision support systemGermanHospitals University03 medical and health sciencesYoung Adult0302 clinical medicinePhysiciansSurveys and QuestionnairesGeneticsMedicineHumans030212 general & internal medicineReimbursementAgedPharmacologybusiness.industryMiddle AgedUniversity hospitalDecision Support Systems Clinicallanguage.human_languageTest (assessment)Pharmacogenetics030220 oncology & carcinogenesisPharmacogenomicsFamily medicinelanguageMolecular MedicineFemalebusinessPharmacogenomics
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Respiratory chain polymorphisms and obesity in the Spanish population, a cross-sectional study

2019

ObjectiveTo study the association of genes involved in the mitochondrial respiratory chain (MRC) pathway with body mass index (BMI) and obesity risk.DesignThis work studies three cross-sectional populations from Spain, representing three provinces: HORTEGA (Valladolid, Northwest/Centre), SEGOVIA (Segovia, Northwest/centre) and PIZARRA (Malaga,South).SettingForty-eight single nucleotide polymorphisms (SNPs) from MRC genes were selected and genotyped by SNPlex method. Association studies with BMI and obesity risk were performed for each population. These associations were then verified by analysis of the studied population as a whole (3731 samples).ParticipantsA total of 3731 Caucasian indivi…

AdultMaleobesityGenotypeCross-sectional studyPopulationRespiratory chainmitochondrial respiratory chain030209 endocrinology & metabolismSingle-nucleotide polymorphismPolymorphism Single NucleotideWhite PeopleBody Mass IndexMitochondrial Proteins03 medical and health sciences0302 clinical medicineRisk FactorsMedicineHumans1506educationAlleles030304 developmental biologyGenetic associationAged0303 health scienceseducation.field_of_studybusiness.industryResearch1697Genetics and GenomicssnpGeneral MedicineMiddle Agedmedicine.diseaseObesityMitochondrial respiratory chainCross-Sectional StudiesElectron Transport Chain Complex ProteinsSpainFemalebusinessBody mass indexDemographyBMJ Open
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