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showing 10 items of 19001 documents

Molecular, clinical and neuropsychological study in 31 patients with Kabuki syndrome and KMT2D mutations

2017

IF 3.326; International audience; Kabuki syndrome (KS-OMIM 147920) is a rare developmental disease characterized by the association of multiple congenital anomalies and intellectual disability. This study aimed to investigate intellectual performance in children with KS and link the performance to several clinical features and molecular data. We recruited 31 children with KMT2D mutations who were 6 to 16 years old. They all completed the Weschler Intelligence Scale for Children, fourth edition. We calculated all indexes: the Full Scale Intellectual Quotient (FSIQ), Verbal Comprehension Index (VCI), Perceptive Reasoning Index (PRI), Processing Speed Index (PSI), and Working Memory Index (WMI…

0301 basic medicineMaleAdolescentVisual impairmentDNA Mutational AnalysisIntelligenceneuropsychologyDisease[SDV.GEN] Life Sciences [q-bio]/GeneticsNeuropsychological Testsgenotype-phenotype correlation03 medical and health sciencesIntensive careIntellectual disabilityGene OrderGeneticsmedicineHumansAbnormalities MultipleChildKMT2D mutationGenetics (clinical)AllelesGenetic Association Studies[SDV.GEN]Life Sciences [q-bio]/GeneticsKabuki syndromebusiness.industryWorking memoryNeuropsychologyWechsler Adult Intelligence Scalemedicine.diseaseHematologic Diseases3. Good healthNeoplasm ProteinsDNA-Binding Proteins030104 developmental biologyPhenotypeVestibular DiseasesGenetic LociFaceMutationFemalemedicine.symptombusinessKabuki syndromeClinical psychology
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Effects of a Lactobacillus salivarius mixture on performance, intestinal health and serum lipids of broiler chickens

2017

The ban or severe restriction on the use of antibiotics in poultry feeds to promote growth has led to considerable interest to find alternative approaches. Probiotics have been considered as such alternatives. In the present study, the effects of a Lactobacillus mixture composed from three previously isolated Lactobacillus salivarius strains (CI1, CI2 and CI3) from chicken intestines on performance, intestinal health status and serum lipids of broiler chickens has been evaluated. Supplementation of the mixture at a concentration of 0.5 or 1 g kg-1 of diet to broilers for 42 days improved body weight, body weight gain and FCR, reduced total cholesterol, LDL-cholesterol and triglycerides, inc…

0301 basic medicineMaleAntibioticsBlood lipidslcsh:MedicineWeight GainBiochemistryPoultrylaw.inventionProbioticRandom AllocationlawLactobacillusMedicine and Health SciencesGamefowlFood sciencelcsh:ScienceBifidobacteriumGlucuronidaseMultidisciplinarybiologyLactobacillus salivariusbeta-Glucosidasefood and beveragesAgriculture04 agricultural and veterinary sciencesLipidsIntestinesCholesterolVertebratesAnatomyResearch Articleanimal structuresmedicine.drug_classReal-Time Polymerase Chain ReactionMicrobiologyBirds03 medical and health sciencesmedicineAnimalsNutritionBacteriaProbioticslcsh:RGut Bacteria0402 animal and dairy scienceBroilerOrganismsBiology and Life Sciencesbiology.organism_classification040201 dairy & animal scienceAnimal FeedDietGastrointestinal MicrobiomeGastrointestinal TractLactobacillus030104 developmental biologyFowlAmniotesLigilactobacillus salivariuslcsh:QBifidobacteriumChickensDigestive SystemBacteriaPLoS ONE
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Exceptional human longevity is associated with a specific plasma phenotype of ether lipids

2019

A lipid profile resistant to oxidative damage is an inherent trait associated with animal lifespan. However, there is a lack of lipidomic studies on human longevity. Here we use mass spectrometry based technologies to detect and quantify 137 ether lipids to define a phenotype of healthy humans with exceptional lifespan. Ether lipids were chosen because of their antioxidant properties and ability to modulate oxidative stress. Our results demonstrate that a specific ether lipid signature can be obtained to define the centenarian state. This profile comprises higher level of alkyl forms derived from phosphatidylcholine with shorter number of carbon atoms and double bonds; and decreased content…

0301 basic medicineMaleAntioxidantmedicine.medical_treatmentClinical BiochemistryBiochemistryLipid peroxidationchemistry.chemical_compound0302 clinical medicineLongevitatlcsh:QH301-705.5media_commonlcsh:R5-920medicine.diagnostic_testLongevityLipidsFenotipEther lipidPhenotypeBiochemistryFemalelipids (amino acids peptides and proteins)lcsh:Medicine (General)Research PaperAdultmedia_common.quotation_subjectPlasmalogensLongevityEther03 medical and health sciencesCentenariansmedicineHumansFree-radical theory of agingAgedPhosphatidylethanolamineMass spectrometryOrganic ChemistryPhosphatidylethanolamineFatty acid unsaturationPhosphatidylcholine030104 developmental biologychemistryROC Curvelcsh:Biology (General)LípidsLipid profile030217 neurology & neurosurgeryAlkenyl phospholipidsAlkyl phospholipidsRedox Biology
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Identification of plasma biomarkers for discrimination between tuberculosis infection/disease and pulmonary non tuberculosis disease.

2018

We used the Luminex Bead Array Multiplex Immunoassay to measure cytokines, chemokines and growth factors responses to the same antigens used for RD1-based Interferon γ Release Assay (IGRA) test. Seventy-nine individuals, 27 active TB, 32 latent infection subsets, 20 individuals derivative purified protein (PPD) negative (subjects that do not have any indurative cutaneous reaction after 72 hrs of intradermal injection of PPD) and with other pulmonary disease were retrospectively studied. Forty-eight analytes were evaluated by Luminex Assay in plasma obtained from whole blood stimulated cells. The diagnostic accuracies of the markers detected were evaluated by ROC curve analysis and by the co…

0301 basic medicineMaleBacterial DiseasesPhysiologylcsh:MedicineBiochemistry0302 clinical medicineBiochemistry Genetics and Molecular Biology (all); Agricultural and Biological Sciences (all)Immune PhysiologyBlood plasmaMedicine and Health SciencesMultiplex030212 general & internal medicinelcsh:ScienceWhole bloodInnate Immune SystemMultidisciplinarymedicine.diagnostic_testLatent tuberculosisbiologyChemotaxis3. Good healthBody FluidsActinobacteriaCell MotilityInfectious DiseasesBloodCytokinesTuberculosis Diagnosis and ManagementChemokinesAnatomyResearch ArticleAdultTuberculosisSettore MED/17 - Malattie InfettiveImmunologyBlood PlasmaMycobacterium tuberculosis03 medical and health sciencesAntigenLatent TuberculosisDiagnostic MedicinemedicineSettore MED/05 - Patologia ClinicaHumansTuberculosisTuberculosis PulmonaryAntigens BacterialBiochemistry Genetics and Molecular Biology (all)Bacteriabusiness.industrylcsh:ROrganismsBiology and Life SciencesMycobacterium tuberculosisCell BiologyMolecular Developmentmedicine.diseasebiology.organism_classificationTropical Diseases030104 developmental biologyAgricultural and Biological Sciences (all)ImmunoassayImmune SystemImmunologylcsh:QbusinessBiomarkersDevelopmental BiologyPloS one
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Infant gut microbiota modulation by human milk disaccharides in humanized microbiome mice

2021

Human milk glycans present a unique diversity of structures that suggest different mechanisms by which they may affect the infant microbiome development. A humanized mouse model generated by infant fecal transplantation was utilized here to evaluate the impact of fucosyl-α1,3-GlcNAc (3FN), fucosyl-α1,6-GlcNAc, lacto-N-biose (LNB) and galacto-N-biose on the fecal microbiota and host–microbiota interactions. 16S rRNA amplicon sequencing showed that certain bacterial genera significantly increased (Ruminococcus and Oscillospira) or decreased (Eubacterium and Clostridium) in all disaccharide-supplemented groups. Interestingly, cluster analysis differentiates the consumption of fucosyl-oligosacc…

0301 basic medicineMaleBifidobacterium longuminfant fecal microbiotaMicrobiologiaRC799-869Gut floraAcetatesDisaccharidesFecesMice0302 clinical medicinelacto-n-biosefluids and secretionsRuminococcus gnavusRNA Ribosomal 16SEubacteriumgalacto-n–bioseBifidobacteriumbiologyGastroenterologyDiseases of the digestive system. Gastroenterologylacto-N-biosegalacto-N–biosefucosyl-α-1ButyratesInfectious Diseases030211 gastroenterology & hepatologyFemaleResearch ArticleResearch PaperMicrobiology (medical)AdultDNA Bacterialhumanized mouse modelInfants Malaltiesshort-chain fatty acidsMicrobiologyMicrobiology03 medical and health sciencesfucosyl-α-16-N-acetylglucosamineYoung AdultAnimalsHumans6-n-acetylglucosamineMicrobiomeBacteriaMilk HumanRuminococcusInfant NewbornInfantAkkermansiafucosyl-α-13-N-acetylglucosaminebiology.organism_classificationcytokinesGastrointestinal Microbiome3-n-acetylglucosamineMice Inbred C57BL030104 developmental biologyshort-chain fatty acidscytokineshuman milk oligosaccharides
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Substantial deficiency of free sialic acid in muscles of patients with GNE myopathy and in a mouse model

2017

GNE myopathy (GNEM), also known as hereditary inclusion body myopathy (HIBM), is a late- onset, progressive myopathy caused by mutations in the GNE gene encoding the enzyme responsible for the first regulated step in the biosynthesis of sialic acid (SA). The disease is characterized by distal muscle weakness in both the lower and upper extremities, with the quadriceps muscle relatively spared until the late stages of disease. To explore the role of SA synthesis in the disease, we conducted a comprehensive and systematic analysis of both free and total SA levels in a large cohort of GNEM patients and a mouse model. A sensitive LC/MS/MS assay was developed to quantify SA in serum and muscle h…

0301 basic medicineMaleBiopsylcsh:MedicineMuscle ProteinsBiochemistryPathogenesischemistry.chemical_compoundMice0302 clinical medicineTandem Mass SpectrometryMedicine and Health Scienceslcsh:ScienceMusculoskeletal SystemMultidisciplinarymedicine.diagnostic_testOrganic CompoundsMusclesGastrocnemius MusclesAnimal ModelsMuscle AnalysisMiddle AgedChemistrymedicine.anatomical_structureBioassays and Physiological AnalysisBiochemistryExperimental Organism SystemsPhysical SciencesFemalemedicine.symptomAnatomyResearch ArticleMuscle tissueAdultmedicine.medical_specialtyAdolescentMuscle TissueMouse ModelsSurgical and Invasive Medical ProceduresCreatineResearch and Analysis Methods03 medical and health sciencesYoung AdultModel OrganismsInternal medicineBiopsymedicineAnimalsHumansMyopathyMuscle SkeletalAgedHereditary inclusion body myopathybusiness.industrylcsh:ROrganic ChemistryChemical CompoundsBiology and Life SciencesProteinsmedicine.diseaseCreatineN-Acetylneuraminic AcidSialic acidDistal MyopathiesDisease Models Animal030104 developmental biologyEndocrinologyBiological TissuechemistrySkeletal Muscleslcsh:QbusinessN-Acetylneuraminic acid030217 neurology & neurosurgeryBiomarkersChromatography LiquidPLoS ONE
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Bone marrow B lymphocytes in multiple myeloma and MGUS: Focus on distribution of naïve cells and memory subsets.

2016

Multiple myeloma (MM) is caused by proliferation of clonal plasma cells (cPCs) in bone marrow (BM), associated with numerical and functional defects in immune subsets. An impairment of B cell compartment is involved in onset/progression of the disease.By flow cytometry, we studied distribution of naïve/transitional (IgD(+)CD27(-)), memory unswitched (IgD(+)CD27(+)), memory switched (IgD(-)CD27(+)) and double negative (DN) (IgD(-)CD27(-)) B lymphocytes in BM of control subjects, and responding and relapsing patients.We observed an increased percentage of IgD(+)CD27(+) B cells in healthy controls vs responding patients (p0.05). Treated non complete responders exhibited an expanded DN compartm…

0301 basic medicineMaleCancer ResearchB-Lymphocyte Subsetschemical and pharmacologic phenomenaBone Marrow CellsImmunoglobulin DMonoclonal Gammopathy of Undetermined SignificanceFlow cytometry03 medical and health sciencesImmune systemstomatognathic systemimmune system diseaseshemic and lymphatic diseasesmedicineHumansB cellMultiple myelomaB-Lymphocyte SubsetsB cellB-Lymphocytesmedicine.diagnostic_testbiologyhemic and immune systemsHematologyImmunoglobulin Dmedicine.diseaseFlow CytometryTumor Necrosis Factor Receptor Superfamily Member 7030104 developmental biologymedicine.anatomical_structureOncologyCase-Control StudiesImmunologybiology.proteinMGUSFemaleBone marrowMultiple MyelomaMonoclonal gammopathy of undetermined significanceLeukemia research
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Exome-wide somatic mutation characterization of small bowel adenocarcinoma

2018

Small bowel adenocarcinoma (SBA) is an aggressive disease with limited treatment options. Despite previous studies, its molecular genetic background has remained somewhat elusive. To comprehensively characterize the mutational landscape of this tumor type, and to identify possible targets of treatment, we conducted the first large exome sequencing study on a population-based set of SBA samples from all three small bowel segments. Archival tissue from 106 primary tumors with appropriate clinical information were available for exome sequencing from a patient series consisting of a majority of confirmed SBA cases diagnosed in Finland between the years 2003–2011. Paired-end exome sequencing was…

0301 basic medicineMaleCancer ResearchMICROSATELLITE INSTABILITYColorectal canceroncogenesReceptor ErbB-2medicine.disease_causeCOLORECTAL-CANCERACTIVATIONCohort Studies0302 clinical medicineAnimal CellsAdenocarcinomasMedicine and Health SciencesExomeFrameshift MutationExomeGenetics (clinical)Exome sequencingAged 80 and overSMALL-INTESTINEeducation.field_of_study1184 Genetics developmental biology physiologyCELIAC-DISEASENonsense MutationMiddle Aged3. Good healthsyöpägeenitOncology030220 oncology & carcinogenesissyöpätauditFemaleSIGNALING PATHWAYKRASCellular TypesResearch ArticleAdultProto-Oncogene Proteins B-raflcsh:QH426-470SEQUENCING DATAImmune CellsNonsense mutationPopulationImmunologyAntigen-Presenting CellsComputational biologysuolistosyövätBiologyAdenocarcinomata3111CarcinomasFrameshift mutation03 medical and health sciencesGermline mutationQUALITY-CONTROLGenetiikka kehitysbiologia fysiologia - Genetics developmental biology physiologySyöpätaudit - CancersIntestinal NeoplasmsmedicineGeneticsPoint MutationHumanseducationMolecular BiologyEcology Evolution Behavior and SystematicsAgedColorectal CancerBiology and Life SciencesCancers and Neoplasmscancerous diseasesCell Biologymedicine.diseaseta3122mutationsCOMPREHENSIVE MOLECULAR CHARACTERIZATIONlcsh:Genetics030104 developmental biologyMutationSomatic Mutationbowel cancer3111 BiomedicinemutaatiotHIGH-RESOLUTIONPLoS Genetics
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Galectin-3 is a marker of favorable prognosis and a biologically relevant molecule in neuroblastic tumors

2014

Childhood neuroblastic tumors are characterized by heterogeneous clinical courses, ranging from benign ganglioneuroma (GN) to highly lethal neuroblastoma (NB). Although a refined prognostic evaluation and risk stratification of each tumor patient is becoming increasingly essential to personalize treatment options, currently only few biomolecular markers (essentially MYCN amplification, chromosome 11q status and DNA ploidy) are validated for this purpose in neuroblastic tumors. Here we report that Galectin-3 (Gal-3), a β-galactoside-binding lectin involved in multiple biological functions that has already acquired diagnostic relevance in specific clinical settings, is variably expressed in m…

0301 basic medicineMaleCancer ResearchPathologyTime FactorsCellular differentiationGalectin 3ApoptosisPredictive Value of TestKaplan-Meier EstimateNeuroblastoma0302 clinical medicineRisk FactorsChildGanglioneuroblastomaGanglioneuroblastomaCell DifferentiationBlood ProteinsNeuroblastic TumorPhenotypeImmunohistochemistry3. Good healthGalectin-3030220 oncology & carcinogenesisChild PreschoolImmunohistochemistryOriginal ArticleFemaleHumanmedicine.medical_specialtyAdolescentTime FactorSchwannian stromaGalectinsImmunologyBiologyTransfectionNeural cell differentiationschwannian stroma; neuroblastoma prognostic factor; neural cell differentiation; neuroblastoma03 medical and health sciencesCellular and Molecular NeurosciencePredictive Value of TestsNeuroblastomaCell Line TumormedicineBiomarkers TumorCell AdhesionHumansGanglioneuromaNeuroblastoma prognostic factorCell ProliferationNeoplasm StagingRisk FactorInfant NewbornApoptosiInfantGanglioneuromaCell Biologymedicine.disease030104 developmental biologyCancer research
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Seroprevalence of SARs-CoV-2 among the staff and patients of a French cancer centre after first lockdown: the canSEROcov study

2021

Abstract Background In view of the potential gravity of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection for patients with cancer, epidemiological data are vital to assess virus circulation among patients and staff of cancer centres. We performed a prospective study to investigate seroprevalence of SARS-CoV-2 antibodies among staff and patients with cancer at a large cancer centre, at the end of the period of first national lockdown in France and to determine factors associated with the risk of SARS-CoV-2 infection. Methods After the first lockdown, all medical and non-medical staff, as well as all patients attending the medical oncology department were invited to unde…

0301 basic medicineMaleCancer ResearchSerology0302 clinical medicineHygieneSeroepidemiologic StudiesEpidemiologyMedicineProspective cohort studyChildmedia_commonOriginal ResearchAged 80 and overserodiagnosisseroprevalenceSocial distanceMiddle AgedSerologyOncology030220 oncology & carcinogenesisChild PreschoolCarrier StateFemaleFrancemedicine.symptomAdultmedicine.medical_specialtyAdolescentmedia_common.quotation_subjectHealth PersonnelSARS-COV-2Cancer Care FacilitiesAsymptomaticCOVID-19 Serological Testingstaff03 medical and health sciencesYoung AdultSeroprevalenceHumansAntibodyAgedbusiness.industryhealthcare workersCancerCOVID-19cancer centerCancer patientsmedicine.diseasePersonnel Hospital030104 developmental biologyFamily medicinebusinessEuropean Journal of Cancer
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