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Role of the ER stress transcription factor XBP1S in the development of idiopathic pulmonary fibrosis
Organization UNIV OF NORTH CAROLINA CHAPEL HILLLocation CHAPEL HILL, UNITED STATESPosted 15 Dec 2020Deadline 30 Nov 2025 ⚠️
NIHUS FederalResearch GrantFY20253-D3-Dimensional3DAffectAgingAllelesAllelomorphsAlveolusApoptosisApoptosis PathwayAssayAutoprothrombin IIIBasal Transcription FactorBasal transcription factor genesBindingBinding ProteinsBioassayBiochemicalBiological AssayBleoBleomycinBlood Coagulation Factor XBronchial AlveolusCausalityCell BodyCell Communication and SignalingCell FunctionCell PhysiologyCell ProcessCell SignalingCellsCellular FunctionCellular PhysiologyCellular ProcessCellular StressCellular Stress ResponseCoagulation Factor XCystDNA mutationDataDefectDevelopmentDisease ProgressionDistalDysfunctionER stressEarly DiagnosisElectrophysiologyElectrophysiology (science)Environmental FactorEnvironmental Risk FactorEpithelial CellsEpitheliumEtiologyExposure toFactor XFibroblastsFibrosing AlveolitisFibrosisFunctional disorderGene ExpressionGene variantGeneral Transcription Factor GeneGeneral Transcription FactorsGenesGeneticGenetic ChangeGenetic defectGenetic mutationGenotypeGrippeH1N1H1N1 VirusHost DefenseHypoxiaHypoxicImpairmentIn VitroIndividualInfectionInflammationInfluenzaInfluenza A Virus, H1N1 SubtypeInjuryIntracellular Communication and SignalingLaboratoriesLeadLigand Binding ProteinLigand Binding Protein GeneLineage TracingLungLung Alveolar EpitheliaLung DiseasesLung Respiratory SystemLung Tissue FibrosisMG1MUC5BMUC5B geneMaintenanceMediatingMessenger RNAMiceMice MammalsMinorModelingMolecular InteractionMucinsMucociliary ClearanceMucociliary TransportMucous body substanceMucusMucus GlycoproteinMurineMusMutationMyofibroblastNatural regenerationNeurophysiology / ElectrophysiologyOnset of illnessOxygen DeficiencyPb elementPeripheralPersonsPhenotypePhysiopathologyPredispositionProbabilityProductionProgenitor CellsProgrammed Cell DeathProtein BindingProteinsPrower factorPulmonary DiseasesPulmonary DisorderPulmonary FibrosisRNA SplicingRegenerationRespiratory EpitheliumRiskRisk FactorsRoleSignal TransductionSignal Transduction SystemsSignalingSplicingStructure of respiratory epitheliumStuart FactorStuart-Prower FactorSubcellular ProcessSusceptibilitySystemTelomeraseTestingTimeTranscription Factor Proto-OncogeneTranscription factor genesType II PneumocyteVariantVariationXBP1XBP1 geneairway epithelial stem cellsairway epitheliumairway progenitorairway remodelingairway stem cellsallelic variantalveolar epitheliumalveolar type II cellbiological signal transductionbiophysical characteristicsbiophysical characterizationbiophysical measurementbiophysical parametersbiophysical propertiesbound proteincausationcell lineage analysiscell lineage mappingcell lineage tracingcell lineage trackingcell stresscellular lineage mappingcellular lineage trackingdevelopmentaldiffuse interstitial pulmonary fibrosisdisease causationdisease of the lungdisease onsetdisorder of the lungdisorder onsetearly detectioneffective therapyeffective treatmentelectrophysiologicalendoplasmic reticulum stressenvironmental riskepithelial injuryfibrosis in the lunggenetic variantgenome mutationgenomic variantheavy metal Pbheavy metal leadhuman modelidiopathic pulmonary fibrosisin vitro Assayin vivoinjuriesinjury and repairinjury responselung disorderlung fibrosislung repairlung tissue repairmRNAmodel of humanmouse geneticsmouse modelmucousmurine modelnew markernotchnotch proteinnotch receptorsnovelnovel biomarkernovel markeroverexpressoverexpressionpathophysiologypreventpreventingprogenitorprogenitor cell functionprogenitor cell homeostasisprogenitor functionpromoterpromotorpulmonarypulmonary repairregeneraterepairrepairedrespiratory progenitorrespiratory stem cellrespiratory tract epitheliumresponseresponse to injurysocial rolestem and progenitor cell functionstem and progenitor functionstem cell functionstem cell homeostasisstem cellsstem cells in the airwaysurfactanttherapeutic targetthree dimensionaltranscription factor
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Description preview
Idiopathic pulmonary fibrosis (IPF) is a progressive and end-stage lung disease of unknown etiology and
no cure. It is likely that genetic changes increase a person's risk of developing IPF, and then exposure to certain
environmental factors and/or aging trigger the onset of the disease. The MUC5B promoter variant rs35705950
is present in ∼50% of…
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