Search results for "EB"

showing 10 items of 15658 documents

9q33.3q34.11 microdeletion: new contiguous gene syndrome encompassing STXBP1, LMX1B and ENG genes assessed using reverse phenotyping

2016

International audience; The increasing use of array-CGH in malformation syndromes with intellectual disability could lead to the description of new contiguous gene syndrome by the analysis of the gene content of the microdeletion and reverse phenotyping. Thanks to a national and international call for collaboration by Achropuce and Decipher, we recruited four patients carrying de novo overlapping deletions of chromosome 9q33.3q34.11, including the STXBP1, the LMX1B and the ENG genes. We restrained the selection to these three genes because the effects of their haploinsufficency are well described in the literature and easily recognizable clinically. All deletions were detected by array-CGH …

0301 basic medicineMale[ SDV.MHEP.PED ] Life Sciences [q-bio]/Human health and pathology/PediatricsHaploinsufficiencycerebral hypomyelinationwest-syndromeBioinformaticsCraniofacial Abnormalities0302 clinical medicineIntellectual disabilitySTXBP1ChildGenetics (clinical)Nail patella syndromeGeneticsEndoglinSyndrome3. Good healthdevelopmental delayPhenotypeintellectual disabilityMedical geneticsFemaleChromosome DeletionHaploinsufficiencyChromosomes Human Pair 9medicine.medical_specialtyAdolescentLIM-Homeodomain ProteinsBiologyContiguous gene syndromeArticle03 medical and health sciencesMunc18 ProteinsGenetic linkageGeneticsmedicineHumansde-novo mutations[SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/PediatricsdiseaseEpilepsyinfantile epileptic encephalopathyassociationdeletionsmedicine.diseaseHuman genetics030104 developmental biologynail-patella syndrome030217 neurology & neurosurgeryTranscription Factors
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The neuroanatomy of Eml1 knockout mice, a model of subcortical heterotopia

2018

Symposium issue: Human Cortex Developmentidentifiant wos: 000482426800014; International audience; The cerebral cortex is a highly organized structure responsible for advanced cognitive functions. Its development relies on a series of steps including neural progenitor cell proliferation, neuronal migration, axonal outgrowth and brain wiring. Disruption of these steps leads to cortical malformations, often associated with intellectual disability and epilepsy. We have generated a new resource to shed further light on subcortical heterotopia, a malformation characterized by abnormal neuronal position. We describe here the generation and characterization of a knockout (KO) mouse model for Eml1,…

0301 basic medicineMale[SDV.MHEP.AHA] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO]heterotopiaHistology[SDV.BA] Life Sciences [q-bio]/Animal biologyClassical Lissencephalies and Subcortical Band HeterotopiasBiologyCorpus callosum03 medical and health sciences0302 clinical medicinemedicine[SDV.MHEP.AHA]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO]Animals[SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]Progenitor cellMolecular BiologyEcology Evolution Behavior and SystematicsMice Knockout[SDV.BA]Life Sciences [q-bio]/Animal biologyBrainHeterozygote advantageCell BiologyOriginal Articlesmouse model of developmental disordersmedicine.diseasecortical malformationsCorticogenesisDisease Models Animal030104 developmental biologymedicine.anatomical_structureHeterotopia (medicine)Cerebral cortexKnockout mouseFemale[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]AnatomyNeuroscienceMicrotubule-Associated Proteins030217 neurology & neurosurgeryDevelopmental BiologyNeuroanatomy
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Association of metformin administration with gut microbiome dysbiosis in healthy volunteers

2018

Background Metformin is a widely used first-line drug for treatment of type 2 diabetes. Despite its advantages, metformin has variable therapeutic effects, contraindications, and side effects. Here, for the very first time, we investigate the short-term effect of metformin on the composition of healthy human gut microbiota. Methods We used an exploratory longitudinal study design in which the first sample from an individual was the control for further samples. Eighteen healthy individuals were treated with metformin (2 × 850 mg) for 7 days. Stool samples were collected at three time points: prior to administration, 24 hours and 7 days after metformin administration. Taxonomic composition of…

0301 basic medicineMaleendocrine system diseasesPhysiologylcsh:MedicineType 2 diabetesGut floraPathology and Laboratory MedicineOpportunistic Pathogens0302 clinical medicineRNA Ribosomal 16SMedicine and Health SciencesLongitudinal Studieslcsh:ScienceData ManagementMultidisciplinarybiologydigestive oral and skin physiologyHigh-Throughput Nucleotide SequencingGenomicsHealthy VolunteersMetformin3. Good healthMetforminBacterial PathogensTolerabilityMedical MicrobiologyFemalePathogensmedicine.drugResearch ArticleMicrobial TaxonomyAdultDNA BacterialEscherichiaComputer and Information SciencesClostridiaceae030209 endocrinology & metabolismMicrobial GenomicsPlaceboDNA RibosomalMicrobiologyDrug Administration Schedule03 medical and health sciencesYoung AdultEnterobacteriaceaeAdverse ReactionsmedicineGeneticsHumansMicrobiomeMicrobial PathogensTaxonomyPharmacologyClostridiumBacteriabusiness.industryPeptostreptococcusTherapeutic effectlcsh:RGut BacteriaOrganismsBiology and Life SciencesSequence Analysis DNAmedicine.diseasebiology.organism_classificationGastrointestinal Microbiome030104 developmental biologyDysbiosislcsh:QMicrobiomebusinessDysbiosisPLOS ONE
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Effect of different oxidative stress degrees generated by hydrogen peroxide on motility and DNA fragmentation of zebrafish (Danio rerio) spermatozoa.

2018

An increase in reactive oxygen species (ROS) or decrease in antioxidant barriers can provoke lipid peroxidation of the membranes or DNA damage of the spermatozoa. The aim of this work is to study the effect of the different degrees of oxidative stress generated by H2 O2 incubation on total motility, kinetics, and DNA fragmentation of zebrafish (Danio rerio) spermatozoa. For this process, experimental groups were incubated in 50 µM (Low; L) and 200 µM (High; H) H2 O2 , respectively, for 20 min at 4oC. Sperm motility parameters were obtained with a computer-assisted sperm analysis (CASA) system. Sperm DNA fragmentation (SDF) was assessed using the sperm chromatin dispersion test. Both low and…

0301 basic medicineMaleendocrine systemDNA damageMotilitymedicine.disease_causeAndrologyLipid peroxidation03 medical and health scienceschemistry.chemical_compound0302 clinical medicineEndocrinologymedicineImage Processing Computer-AssistedAnimalsSperm motilityZebrafishchemistry.chemical_classificationReactive oxygen species030219 obstetrics & reproductive medicineurogenital systemHydrogen PeroxideSpermSpermatozoaOxidative Stress030104 developmental biologychemistrySperm MotilityDNA fragmentationAnimal Science and ZoologyOxidative stressBiotechnologyDNA DamageReproduction in domestic animals = Zuchthygiene
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Effect of nutritional supplementations on physical performance and muscle strength parameters in older people: A systematic review and meta-analysis

2019

Malnutrition plays a role in the development of poor physical performance, frailty and sarcopenia. The use of nutritional supplementations for improving physical performance and muscle strength parameters in older people is unclear. We therefore aimed to summarize the effect of nutritional supplementations compared to placebo on physical performance (i.e. tests more investigating physical function, utilising aerobic capacity & muscle power) and muscle strength (i.e. tests depending on muscle power) outcomes in older people in randomized controlled trials (RCTs). A literature search in major databases was undertaken until the 01st September 2018. Eligible studies were RCTs investigating …

0301 basic medicineMalemedicine.medical_specialtyAgingSarcopeniaNutritional SupplementationPlaceboBiochemistrylaw.inventionPhysical performance03 medical and health sciencesA systematic review and meta-analysis.- Ageing research reviews cilt.51 ss.48-54 2019 [VERONESE N. STUBBS B. PUNZI L. Soysal P. INCALZI R. SALLER A. MAGGI S. -Effect of nutritional supplementations on physical performance and muscle strength parameters in older people]0302 clinical medicineRandomized controlled triallawmedicineHumansMolecular BiologyAgedRandomized Controlled Trials as TopicNutritionAged 80 and overHand Strengthbusiness.industryMuscle strengthPhysical Functional Performancemedicine.diseaseConfidence intervalMalnutritionMeta-analysis030104 developmental biologyTreatment OutcomeNeurologyPhysical performanceSarcopeniaMeta-analysisDietary SupplementsPhysical therapyFemalebusiness030217 neurology & neurosurgeryBiotechnology
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GABA-containing compound gammapyrone protects against brain impairments in Alzheimer's disease model male rats and prevents mitochondrial dysfunction…

2018

Neuroinflammation, oxidative stress, decreased glucose/energy metabolism, and disrupted neurotransmission are changes that occur early in sporadic Alzheimer's disease (AD), manifesting as mild cognitive impairment. Recently, the imbalanced function of the gamma-aminobutyric acid (GABA) system was identified as a critical factor in AD progression. Thus, maintaining balance among neurotransmitter systems, particularly the GABA system, can be considered a beneficial strategy to slow AD progression. The present study investigated the effects of the compound gammapyrone, a molecule containing three GABA moieties: "free" moiety attached to the position 4 of the 1,4-dihydropyridine (DHP) ring, and…

0301 basic medicineMalemedicine.medical_specialtyAllosteric regulationbioenergetics; GABA; intracerebroventricular streptozocin; PC12 cells; protein expression; spatial learning/memoryNeurotransmissionspatial learning/memorymedicine.disease_causebioenergeticsNeuroprotection03 medical and health sciencesCellular and Molecular NeuroscienceGABA0302 clinical medicineReceptors GABAAlzheimer DiseaseMemoryInternal medicinemedicineAnimalsRats WistarReceptorMaze Learningprotein expressionNeuroinflammationCells Culturedgamma-Aminobutyric AcidGABAA receptorChemistryGlutamate DecarboxylasePC12 cellsBrainintracerebroventricular streptozocinMitochondriaStreptozocinDisease Models Animal030104 developmental biologyEndocrinologyNeuroprotective AgentsAstrocytesAcetylcholinesteraseEncephalitisMicroglia030217 neurology & neurosurgeryOxidative stressJournal of neuroscience research
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Effect of Omega-3 and Vitamins E + C Supplements on the Concentration of Serum B-Vitamins and Plasma Redox Aminothiol Antioxidant Status in Elderly M…

2015

<b><i>Background:</i></b> Data on redox plasma aminothiol status in individuals on strength training are very limited. Therefore, we studied the effect of omega-3 and vitamins E + C supplementation on the concentration of B-vitamins and redox aminothiol status in elderly men after strength training for 3 months. <b><i>Methods:</i></b> Healthy men, age 60 ± 6 (mean ± SD) were randomly divided into 3 groups: group I received placebo (n = 17), group II consumed omega-3 (700 mg, n = 17), and group III consumed vitamins E + C (235 mg +1 g, n = 16) daily for 3 months. All participants completed a strength training program for the same period. <b&…

0301 basic medicineMalemedicine.medical_specialtyAntioxidantPyridoxalStrength trainingmedicine.medical_treatmentMedicine (miscellaneous)Ascorbic Acid030204 cardiovascular system & hematologyPlaceboAntioxidants03 medical and health scienceschemistry.chemical_compound0302 clinical medicineFolic AcidInternal medicineFatty Acids Omega-3medicineHumansVitamin ESulfhydryl CompoundsPyridoxalAgedNutrition and DieteticsVitamin EResistance TrainingVitaminsMiddle AgedAscorbic acidUric AcidB vitaminsVitamin B 12030104 developmental biologyEndocrinologyBiochemistrychemistryDietary SupplementsVitamin B ComplexUric acidAnnals of nutritionmetabolism
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Effect of a Nutritional Intervention on the Intestinal Microbiota of Vertically HIV-Infected Children: The Pediabiota Study

2020

This article belongs to the Special Issue Role of Prebiotics and Probiotics in Health and Disease.

0301 basic medicineMalemedicine.medical_specialtyChildren and adolescentsTime FactorsHuman immunodeficiency virus (HIV)HIV Infectionslcsh:TX341-641Diseasemedicine.disease_causedigestive systemArticle03 medical and health sciences0302 clinical medicineDouble-Blind MethodIntervention (counseling)Internal medicineHiv infectedmedicinemicrobiotaHumans030212 general & internal medicineChildSymbiosisNutrition and Dieteticsbusiness.industryMicrobiotaProbioticsHIVInfectious Disease Transmission VerticalGastrointestinal MicrobiomePilots030104 developmental biologyPrebioticsAnti-Retroviral Agentschildren and adolescentsChild PreschoolDietary SupplementsVertical transmissionDysbiosisFemalevertical transmissionbusinessChild Nutritional Physiological Phenomenalcsh:Nutrition. Foods and food supplyFood ScienceNutrients
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Anxiolytic effects of muscarinic acetylcholine receptors agonist oxotremorine in chronically stressed rats and related changes in BDNF and FGF2 level…

2017

Rationale: In depressive disorders, one of the mechanisms proposed for antidepressant drugs is the enhancement of synaptic plasticity in the hippocampus and cerebral cortex. Previously, we showed that the muscarinic acetylcholine receptor (mAChR) agonist oxotremorine (Oxo) increases neuronal plasticity in hippocampal neurons via FGFR1 transactivation. Objectives: Here, we aimed to explore (a) whether Oxo exerts anxiolytic effect in the rat model of anxiety-depression-like behavior induced by chronic restraint stress (CRS), and (b) if the anxiolytic effect of Oxo is associated with the modulation of neurotrophic factors, brain-derived neurotrophic factor (BDNF) and fibroblast growth factor-2…

0301 basic medicineMalemedicine.medical_specialtyElevated plus mazemedicine.drug_classBehavioral testPrefrontal CortexHippocampal formationAnxietyMuscarinic AgonistsAnxiolyticHippocampus03 medical and health sciences0302 clinical medicineInternal medicineMuscarinic acetylcholine receptormedicineOxotremorineMuscarinic acetylcholine receptor M4AnimalsElevated plus maze testRats WistarPrefrontal cortexmAChRChronic restraint streForced swimming testPharmacologyNeuronsChemistryBrain-Derived Neurotrophic FactorOxotremorineCerebral cortexRats030104 developmental biologymedicine.anatomical_structureEndocrinologyAnti-Anxiety AgentsCerebral cortexFibroblast Growth Factor 2Anxiety; Behavioral test; Cerebral cortex; Chronic restraint stress; Elevated plus maze test; Forced swimming test; mAChR; Neurotrophins; Novelty suppressed feeding test; PharmacologyNeurotrophinNovelty suppressed feeding testNeuroscience030217 neurology & neurosurgeryStress Psychologicalmedicine.drugPsychopharmacology
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Mechanisms involved in the increased sensitivity of the rabbit basilar artery to atrial natriuretic peptide in diabetes.

2017

Atrial natriuretic peptide (ANP) is a vasodilator with significant regional differences and controversial effects in the cerebral circulation, a vascular bed particularly prone to diabetes-induced complications. The present study has investigated how alloxan-induced diabetes modifies the mechanisms involved in the response of the rabbit basilar artery to ANP. ANP (10(-12) -10(-7) M) relaxed precontracted basilar arteries, with higher potency in diabetic than in control rabbits. In arteries from both groups of animals, endothelium removal reduced ANP-induced relaxations. Inhibition of NO-synthesis attenuated ANP-induced relaxation but this attenuation was lower in diabetic than in control ra…

0301 basic medicineMalemedicine.medical_specialtyEndotheliummedicine.drug_classRabbit basilar arteryVasodilationProstanoidsNitric OxidePotassium channelsDiabetes Mellitus ExperimentalGlibenclamide03 medical and health sciencesCerebral circulationAtrial natriuretic peptidemedicine.arteryInternal medicinemedicineBasilar arteryNatriuretic peptideAnimalsAtrial natriuretic peptidePharmacologyDose-Response Relationship Drugbusiness.industryDiabetesNitric oxideIberiotoxin030104 developmental biologyEndocrinologymedicine.anatomical_structureBasilar Arterycardiovascular systemProstaglandinsRabbitsbusinessReceptors Atrial Natriuretic Factorhormones hormone substitutes and hormone antagonistsAtrial Natriuretic Factormedicine.drugEuropean journal of pharmacology
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