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IRE1α mediated dysregulation of the pulmonary epithelium in lung fibrosis

Organization UNIVERSITY OF CALIFORNIA, SAN FRANCISCOLocation SAN FRANCISCO, UNITED STATESPosted 15 Mar 2021Deadline 28 Feb 2026 ⚠️
NIHUS FederalResearch GrantFY2025Airway failureApoptosisApoptosis PathwayBasal Transcription FactorBasal transcription factor genesBindingBioavailabilityBiologicalBiological AvailabilityBiologyBleoBleomycinBody TissuesBone-Derived Transforming Growth FactorCareer Development AwardsCareer Development Awards and ProgramsCareer Development Programs K-SeriesCausalityCell AgingCell BodyCell Communication and SignalingCell CountCell DeathCell FunctionCell NumberCell PhysiologyCell ProcessCell SenescenceCell SignalingCellsCellular AgingCellular FunctionCellular PhysiologyCellular ProcessCellular SenescenceCellular StressCellular Stress ResponseCessation of lifeChemicalsCicatrixClinical EvaluationClinical TestingCollaborationsDNA DamageDNA InjuryDataDeathDevelopmental BiologyDiagnosisDiseaseDisorderDysfunctionER stressEndoplasmic ReticulumEndoribonucleasesEnvironmentEnvironmental ExposureEpithelial CellsEpitheliumEquilibriumEquipmentErgastoplasmEtiologyEventExpression SignatureFamilyFamily memberFibrosing AlveolitisFibrosisFibrotic lesions in lungFlow CytofluorometriesFlow CytofluorometryFlow CytometryFlow MicrofluorimetryFlow MicrofluorometryFunctional disorderGene AlterationGene Down-RegulationGene ExpressionGene Expression ProfileGene MutationGeneral Transcription Factor GeneGeneral Transcription FactorsGenesGeneticGenetic ModelsGenetic predisposing factorHistologicHistologicallyHumanHyperactivityIn-bleomycinIntegrinsIntegrins Extracellular MatrixInternationalIntracellular Communication and SignalingK-AwardsK-Series Research Career ProgramsKinasesKnock-outKnockoutLaboratoriesLinkLungLung Alveolar EpitheliaLung DiseasesLung Respiratory SystemLung Tissue FibrosisLung damageLung scarLung tissue regenerationLung tissue scarMediatingMediatorMembraneMentorsMentorshipMessenger RNAMiceMice MammalsMicroRNAsMilk Growth FactorMissionModelingModern ManMolecular InteractionMurineMusOutcomePathogenicityPathologicPathway interactionsPatientsPhosphotransferase GenePhosphotransferasesPhysiciansPhysiologicPhysiologic AvailabilityPhysiologicalPhysiopathologyPlatelet Transforming Growth FactorPopulationProductivityProfibrotic factorProfibrotic signalProgenitor CellsProgrammed Cell DeathProgressive DiseaseProteinsPulmonary DiseasesPulmonary DisorderPulmonary FibrosisPulmonary ScarPulmonary Tissue fibrosisRNA NucleasesRNA SplicingRNA endonucleaseRNaseRecordsReplicative SenescenceResearchResearch Career ProgramRespiratory FailureRibonuclease Family ProteinRibonucleasesScarring at the lungScarring in the lungScarsScientistSeminalSignal TransductionSignal Transduction SystemsSignalingSingle cell seqSplicingStressSubcellular ProcessTGF BTGF-betaTGF-βTGFbetaTGFβTelomere ShorteningTestingTissuesTrainingTranscription Factor Proto-OncogeneTranscription RepressionTranscription factor genesTransforming Growth Factor betaTransforming Growth Factor-Beta Family GeneTranslationsTransphosphorylasesXBP1XBP1 geneaberrant protein foldingabnormal protein foldingalveolar epitheliumbalancebalance functionbiologicbiological signal transductioncareercausationcell stressclinical testconferenceconventiondiffuse interstitial pulmonary fibrosisdisease causationdisease of the lungdisorder of the lungendoplasmic reticulum stressepithelial injuryepithelial progenitorepithelial progenitor cellepithelial stem cellfibrosis in the lungfibrotic lungflow cytophotometrygene defectgene expression patterngene expression signaturegene repressiongene signaturesgenetic risk factorgenetic signatureidiopathic pulmonary fibrosisin vitro Modelindium-bleomycininherited factorinnovateinnovationinnovativeinterstitialkinase inhibitorlung developmentlung disorderlung fibrosislung injurylung regenerationlung repairlung tissue repairmRNAmembrane structuremiRNAmouse modelmurine modelmutant allelenecrocytosisoverexpressoverexpressionpathologic protein foldingpathophysiologypathwayprematureprematurityprotein foldingprotein misfoldingpulmonarypulmonary damagepulmonary injurypulmonary regenerationpulmonary repairpulmonary tissue damagepulmonary tissue injuryregeneration biologyregeneration potentialregenerative biologyregenerative potentialrepairrepairedreplicative agingresearch clinical testingresponsesecretory proteinsenescencesenescence and its associated secretory phenotypesenescence associated secretomesenescence associated secretory factorssenescence associated secretory pathwaysenescence associated secretory phenotypesenescence associated secretory programsenescence associated secretory proteinssenescentsenescent associated secretomesenescent associated secretory phenotypesingle cell next generation sequencingsingle cell sequencingskillsstem cellssummitsymposiasymposiumtelomere attritiontranscription factortranscriptional profiletranscriptional signaturetranslation

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Project Summary / Abstract
Idiopathic pulmonary fibrosis (IPF) is a disease of progressive interstitial fibrosis, which leads to severe

debilitation and eventually respiratory failure and death. Protein folding stress in the endoplasmic reticulum (ER

stress) triggers the unfolded protein response (UPR), which has been implicated in IPF. The most…

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