Search results for "Knockout"

showing 10 items of 806 documents

Lack of GDAP1 induces neuronal calcium and mitochondrial defects in a knockout mouse model of Charcot-Marie-tooth neuropathy

2015

27 páginas, 9 figuras.

Mitochondrial proteinCancer Researchlcsh:QH426-470Nerve Tissue ProteinsBiologyMitochondrionCharcot-Marie-Tooth diseaseGDAP1 geneMiceGeneticsAutophagyAnimalsCalcium SignalingMolecular BiologyGenetics (clinical)Ecology Evolution Behavior and SystematicsCytoskeletonCalcium signalingGeneticsVoltage-dependent calcium channelEndoplasmic reticulumAutophagyBiología y Biomedicina / BiologíaAxonsCell biologyMitochondriaMitochondrialMice Inbred C57BLAlpha tubulinlcsh:Geneticsmitochondrial fusionKnockout mouseMitochondrial fissionCalcium ChannelsAnimal cellGene DeletionResearch Article
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Lactaturia and loss of sodium-dependent lactate uptake in the colon of SLC5A8-deficient mice.

2008

SLC5A8 is a member of the sodium/glucose cotransporter family. It has been proposed that SLC5A8 might act as an apical iodide transporter in the thyroid follicular cells or as a transporter of short chain monocarboxylates. We have directly addressed the functional role of SLC5A8 in vivo by generation of SLC5A8 mutant mice. We found that SLC5A8 is responsible for the re-absorption of lactate at the apical membrane of the kidney proximal tubules and of serous salivary gland ducts. In addition, SLC5A8 mediated the uptake of lactate into colonocytes under physiological conditions. We did not find any evidence of SLC5A8 being essential for the apical iodide transport in the thyroid gland, even i…

Monocarboxylic Acid Transportersmedicine.medical_specialtyColonButyrateBiologyBiochemistryIntestinal absorptionMiceInternal medicinemedicineAnimalsIodide transportLactic AcidMolecular BiologyCation Transport ProteinsMice KnockoutThyroidSodiumTransporterCell BiologyNeoplasms ExperimentalApical membraneTransport proteinButyratesMembrane Transport Structure Function and BiogenesisEndocrinologymedicine.anatomical_structureCell Transformation NeoplasticIntestinal AbsorptionCarcinogensKidney DiseasesCotransporterThe Journal of biological chemistry
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Lipid profiling following intake of the Omega 3 fatty acid DHA identifies the peroxidized metabolites F-4-Neuroprostanes as the best predictors of at…

2014

International audience; The anti-atherogenic effects of omega 3 fatty acids, namely eicosapentaenoic (EPA) and docosahexaenoic acids (DHA) are well recognized but the impact of dietary intake on bioactive lipid mediat or profiles remains unclear. Such a profiling effort may offer novel targets for future studies into the mechanism of action of omega 3 fatty acids. The present study aimed to determine the impact of DHA supplementation on the profiles of polyunsaturated fatty acids (PUFA) oxygenated metabolites and to investigate their contribution to therosclerosis prevention. A special emphasis was given to the non-enzymatic metabolites knowing the high susceptibility of DHA to free radical…

MouseBlood PressureCardiovascularBiochemistryMice0302 clinical medicineTandem Mass SpectrometryAetiologylcsh:Sciencechemistry.chemical_classificationLiquid0303 health sciences[CHIM.ORGA]Chemical Sciences/Organic chemistryFatty Acidsanti-atherogenic;omega 3 fatty acids;epa;dha;bioactive lipid;atherosclerosis;pufas;effects;molecularLipids3. Good healthFatty Acids UnsaturatedMedicinemedicine.medical_specialtyanti-atherogenicKnockoutAortic DiseasesMédecine humaine et pathologieGas Chromatography-Mass SpectrometryLDLDose-Response RelationshipLipid Mediators03 medical and health sciencesomega 3 fatty acids[ CHIM.ORGA ] Chemical Sciences/Organic chemistryacide gras n 3Complementary and Integrative HealtheffectsBiologybioactive lipidDose-Response Relationship DrugPreventionlcsh:RathéroscléroseEPALipid MetabolismPrevention of disease and conditionsmedicine.diseaseEndocrinologychemistryNeuroprostaneslcsh:QHuman health and pathologyBiomarkersand promotion of well-beinglcsh:Medicine030204 cardiovascular system & hematologymedicine.disease_causeOral and gastrointestinalHeart RateReceptorsBlood plasmaCluster Analysis2.1 Biological and endogenous factorsMice KnockoutUnsaturatedChromatographyMultidisciplinaryFatty liverAnimal ModelsDHALiverBiochemistryDocosahexaenoic acidlipids (amino acids peptides and proteins)DrugResearch ArticlePolyunsaturated fatty acidDocosahexaenoic AcidsClinical Research DesignGeneral Science & TechnologyBiologyModel OrganismsInternal medicinemedicineAnimalsNeuroprostanesAnimal Models of Diseasemolecular3.3 Nutrition and chemopreventionOmega 3 fatty acidNutrition030304 developmental biologyAnalysis of Varianceacide docosahexaénoiqueLipid metabolismAtherosclerosis[SDV.AEN] Life Sciences [q-bio]/Food and NutritionReceptors LDLPUFAs[SDV.AEN]Life Sciences [q-bio]/Food and NutritionOxidative stressChromatography Liquid
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Regulatory T Cells and IL-10 Independently Counterregulate Cytotoxic T Lymphocyte Responses Induced by Transcutaneous Immunization

2011

Background: The imidazoquinoline derivate imiquimod induces inflammatory responses and protection against transplanted tumors when applied to the skin in combination with a cognate peptide epitope (transcutaneous immunization, TCI). Here we investigated the role of regulatory T cells (Treg) and the suppressive cytokine IL-10 in restricting TCI-induced cytotoxic T lymphocyte (CTL) responses. Methodology/Principal Findings: TCI was performed with an ointment containing the TLR7 agonist imiquimod and a CTL epitope was applied to the depilated back skin of C57BL/6 mice. Using specific antibodies and FoxP3-diphteria toxin receptor transgenic (DEREG) mice, we interrogated inhibiting factors after…

Mouselcsh:MedicineEpitopes T-LymphocyteAdaptive ImmunityT-Lymphocytes RegulatoryImmune toleranceMiceMedicineCytotoxic T celllcsh:ScienceImmune ResponseSkinMice KnockoutB-LymphocytesMultidisciplinaryImiquimodFOXP3hemic and immune systemsForkhead Transcription FactorsAnimal ModelsFlow CytometryInterleukin-10Interleukin 10medicine.anatomical_structureAminoquinolinesCytokinesIntercellular Signaling Peptides and ProteinsImmunotherapyResearch ArticleHeparin-binding EGF-like Growth FactorT cellImmune CellsImmunologychemical and pharmacologic phenomenaImmune SuppressionImmunomodulationImmune systemModel OrganismsImmune ToleranceAnimalsBiologyB cellbusiness.industrylcsh:RImmunityMice Inbred C57BLCTL*Immune SystemImmunologyImmunologic Techniqueslcsh:QImmunizationbusinessT-Lymphocytes CytotoxicPLoS ONE
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FSHD muscular dystrophy region gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis.

2013

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant myopathy with a strong epigenetic component. It is associated with deletion of a macrosatellite repeat leading to over-expression of the nearby genes. Among them, we focused on FSHD region gene 1 (FRG1) since its over-expression in mice, Xenopus laevis and Caenorhabditis elegans, leads to muscular dystrophy-like defects, suggesting that FRG1 plays a relevant role in muscle biology. Here we show that, when over-expressed, FRG1 binds and interferes with the activity of the histone methyltransferase Suv4-20h1 both in mammals and Drosophila. Accordingly, FRG1 over-expression or Suv4-20h1 knockdown inhibits myogenesis. Moreov…

Muscle DevelopmentEvolution Molecular03 medical and health sciencesMice0302 clinical medicineGeneticsmedicineFacioscapulohumeral muscular dystrophyMyocyteAnimalsHumansEpigeneticsMuscular dystrophyMyopathyMolecular Biology030304 developmental biologyCell NucleusMice Knockout0303 health sciencesMuscle CellsbiologyMyogenesisMicrofilament ProteinsNuclear ProteinsProteinsRNA-Binding ProteinsCell DifferentiationCell BiologyGeneral MedicineHistone-Lysine N-MethyltransferaseMuscular Dystrophy Animalmedicine.diseaseMolecular biologyHistoneDrosophila melanogasterHEK293 CellsPhenotypeOrgan SpecificityHistone methyltransferaseEpigenetic deregulation by FRG1Gene Knockdown Techniquesbiology.proteinmedicine.symptomCarrier Proteins030217 neurology & neurosurgeryProtein BindingJournal of molecular cell biology
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Transcriptome comparison of murine wild-type and synaptophysin-deficient retina reveals complete identity

2005

Loss of synaptophysin, one of the major synaptic vesicle membrane proteins, is surprisingly well tolerated in knockout mice. To test whether compensatory gene transcription accounts for the apparent lack of functional deficiencies, comparative transcriptome analyses were carried out. The retina was selected as the most suitable tissue since morphological alterations were observed in mutant photoreceptors, most notably a reduction of synaptic vesicles and concomitant increase in clathrin-coated vesicles. Labeled cRNA was prepared in triplicate from retinae of age- and sex-matched wild-type and mutant litter mates and hybridized to high-density microarray chips. Only three differentially expr…

MutantSynaptophysinSynaptic vesicleRetinaTranscriptomeMiceMicroscopy Electron TransmissionGene expressionAnimalsPhotoreceptor CellsRNA MessengerEye ProteinsMolecular BiologyMice KnockoutbiologyReverse Transcriptase Polymerase Chain ReactionSynaptic vesicle membraneGeneral NeuroscienceWild typeGlucan 13-beta-GlucosidaseMicroarray AnalysisMolecular biologyClathrinMice Inbred C57BLGene Expression RegulationKnockout mouseSynaptophysinbiology.proteinSynaptic VesiclesNeurology (clinical)Developmental BiologyBrain Research
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Cutting Edge: IL-1α Is a Crucial Danger Signal Triggering Acute Myocardial Inflammation during Myocardial Infarction

2016

Abstract Myocardial infarction (MI) induces a sterile inflammatory response that contributes to adverse cardiac remodeling. The initiating mechanisms of this response remain incompletely defined. We found that necrotic cardiomyocytes released a heat-labile proinflammatory signal activating MAPKs and NF-κB in cardiac fibroblasts, with secondary production of cytokines. This response was abolished in Myd88−/− fibroblasts but was unaffected in nlrp3-deficient fibroblasts. Despite MyD88 dependency, the response was TLR independent, as explored in TLR reporter cells, pointing to a contribution of the IL-1 pathway. Indeed, necrotic cardiomyocytes released IL-1α, but not IL-1β, and the immune acti…

MyocarditisImmunologyInterleukin-1betaMyocardial InfarctionInflammation030204 cardiovascular system & hematologyArticleProinflammatory cytokine03 medical and health sciencesMice0302 clinical medicineImmune system[SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular systemIn vivoInterleukin-1alphamedicineImmunology and AllergyAnimalsMyocytes CardiacMyocardial infarction030304 developmental biologyInflammationMice Knockout0303 health sciencesbusiness.industryToll-Like Receptorsmedicine.disease[SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular systemMyocarditisIL1AImmunologyMyeloid Differentiation Factor 88Cancer researchmedicine.symptomSignal transductionbusinessSignal Transduction
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Reduced presynaptic efficiency of excitatory synaptic transmission impairs LTP in the visual cortex of BDNF-heterozygous mice

2006

The neurotrophin brain-derived neurotrophic factor (BDNF) plays an important role in neuronal survival, axonal and dendritic growth and synapse formation. BDNF has also been reported to mediate visual cortex plasticity. Here we studied the cellular mechanisms of BDNF-mediated changes in synaptic plasticity, excitatory synaptic transmission and long-term potentiation (LTP) in the visual cortex of heterozygous BDNF-knockout mice (BDNF(+/-)). Patch-clamp recordings in slices showed an approximately 50% reduction in the frequency of miniature excitatory postsynaptic currents (mEPSCs) compared to wild-type animals, in the absence of changes in mEPSC amplitudes. A presynaptic impairment of excita…

N-MethylaspartatePatch-Clamp TechniquesTime FactorsLong-Term PotentiationPresynaptic TerminalsAMPA receptorIn Vitro TechniquesSynaptic TransmissionMicePostsynaptic potentialQuinoxalinesExcitatory Amino Acid AgonistsAnimalsalpha-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic AcidVisual CortexMice KnockoutNeuronsBrain-derived neurotrophic factorDose-Response Relationship DrugPost-tetanic potentiationChemistryBrain-Derived Neurotrophic FactorGeneral NeuroscienceDose-Response Relationship RadiationLong-term potentiationElectric StimulationSynaptic fatigueAnimals Newbornnervous systemSynaptic plasticityExcitatory postsynaptic potentialCalciumExcitatory Amino Acid AntagonistsNeuroscienceEuropean Journal of Neuroscience
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Loss of Nrf2 in bone marrow-derived macrophages impairs antigen-driven CD8+ T cell function by limiting GSH and Cys availability

2015

NF-E2-related factor 2 (Nrf2), known to protect against reactive oxygen species, has recently been reported to resolve acute inflammatory responses in activated macrophages. Consequently, disruption of Nrf2 promotes a proinflammatory macrophage phenotype. In the current study, we addressed the impact of this macrophage phenotype on CD8(+) T cell activation by using an antigen-driven coculture model consisting of Nrf2(-/-) and Nrf2(+/+) bone marrow-derived macrophages (BMDMΦ) and transgenic OT-1 CD8(+) T cells. OT-1 CD8(+) T cells encode a T cell receptor that specifically recognizes MHC class I-presented ovalbumin OVA(257-264) peptide, thereby causing a downstream T cell activation. Interes…

NF-E2-Related Factor 2OvalbuminAntiporterT cellBlotting WesternReceptors Antigen T-CellApoptosisMice TransgenicCD8-Positive T-LymphocytesBiologyReal-Time Polymerase Chain Reactionenvironment and public healthBiochemistryAntioxidantsImmunoenzyme TechniquesMicechemistry.chemical_compoundBone MarrowPhysiology (medical)MHC class ImedicineAnimalsCytotoxic T cellRNA MessengerCells CulturedCell ProliferationMice KnockoutReverse Transcriptase Polymerase Chain ReactionGCLMMacrophagesHistocompatibility Antigens Class IGlutathionerespiratory systemFlow CytometryGlutathioneMolecular biologyMice Inbred C57BLOxidative Stressmedicine.anatomical_structurechemistrybiology.proteinCystineReactive Oxygen SpeciesIntracellularCD8Signal TransductionFree Radical Biology and Medicine
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Increased immunosuppressive function of CD4(+)CD25(+)Foxp3(+)GITR+ T regulatory cells from NFATc2((-/-)) mice controls allergen-induced experimental …

2012

The expansion of effector T cells is tightly controlled by transcription factors like nuclear factor of activated T cells (NFAT) family members that mediate early intracellular responses to T cell receptor-mediated signals. In this study we show that, after allergen challenge, NFATc2((-/-)) mice had augmented number of functionally intact CD4(+)CD25(++)GITR(++) T regulatory (T regs) cells in the lung. Anti-GITR antibody treatment inhibited T regulatory cell function and enhanced the number of activated lung CD4(+) T cells associated with increased IL-2 and pSTAT-5 in the airways of NFATc2((-/-)) mice in experimental allergic asthma. This agonistic treatment led to increased inflammation in …

NFATC2T cellImmunologyInflammationBiologyT-Lymphocytes RegulatoryInterleukin 21MiceTh2 CellsGlucocorticoid-Induced TNFR-Related ProteinmedicineImmunology and AllergyAnimalsIL-2 receptorLungImmunosuppression TherapyMice KnockoutNFATC Transcription FactorsInterleukin-2 Receptor alpha SubunitFOXP3NFATForkhead Transcription FactorsHematologyAllergensAsthmarespiratory tract diseasesDisease Models Animalmedicine.anatomical_structureImmunologyCD4 Antigensbiology.proteinCytokinesTh17 CellsFemalemedicine.symptomAntibodySpleenImmunobiology
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