grant
Alveolar Epithelial Cell Dysfunction in Pulmonary Fibrosis: Leveraging SFTPC Mutations for Discovery of Molecular and Cellular Targets
Organization UNIVERSITY OF PENNSYLVANIALocation PHILADELPHIA, UNITED STATESPosted 15 Jul 2019Deadline 30 Jun 2028
NIHUS FederalResearch GrantFY202521+ years oldATF6ATF6 geneActivating Transcription Factor 6AdultAdult HumanAffectAgingAirway failureAlveoli progenitorAlveoli stem cellArchitectureAutophagocytosisBioenergeticsBiogenesisBiologic ModelsBiological ModelsBody TissuesCell BodyCell Communication and SignalingCell ReprogrammingCell SignalingCellsCellular Metabolic ProcessCessation of lifeChronicCicatrixConsensusCoupledDNA mutationDataDeathDistalDysfunctionER stressElementsEndoplasmic ReticulumEngineering / ArchitectureEpithelial CellsEpitheliumEquilibriumEquipoiseErgastoplasmEsbrietEventFibrosing AlveolitisFibrosisFibrotic lesions in lungFunctional disorderFundingGasesGene AlterationGene MutationGene TranscriptionGene variantGenerationsGenesGeneticGenetic ChangeGenetic TranscriptionGenetic defectGenetic mutationGlycolysisHumanHypoxemic Respiratory FailureHypoxic Respiratory FailureImpairmentIn VitroIntermediary MetabolismInterstitial Lung DiseasesIntracellular Communication and SignalingIsoformsKI miceKinasesKnock-in MouseLungLung Alveolar EpitheliaLung DiseasesLung Respiratory SystemLung Tissue FibrosisLung scarLung tissue scarMediatingMediatorMedicalMembraneMetabolicMetabolic ControlMetabolic ProcessesMetabolismMiceMice MammalsMitochondriaModel SystemModelingModern ManMolecularMolecular FingerprintingMolecular ProfilingMolecular TargetMorbidityMorbidity - disease rateMurineMusMutationOfevOrigin of LifeOutcomePathogenesisPathologicPathway interactionsPatientsPhenotypePhosphotransferase GenePhosphotransferasesPhysiologyPhysiopathologyPirfenidonePopulationPre-Clinical ModelPreclinical ModelsPreclinical dataProgress ReportsProtein IsoformsProteinsPublishingPulmonary DiseasesPulmonary DisorderPulmonary FibrosisPulmonary ScarPulmonary Surfactant Protein CPulmonary Surfactant-Associated Protein CPulmonary Surfactant-Associated Protein SP-CPulmonary Tissue fibrosisQuality ControlRNA ExpressionRefractoryRespirationRespiratory FailureRoleSP-C peptideSP-C proteinScarring at the lungScarring in the lungScarsSecondary toSeminalSignal PathwaySignal TransductionSignal Transduction SystemsSignalingSocial Support SystemSubgroupSupport SystemSurfactant Polypeptide SP-CTestingTherapeuticTissuesTranscriptionTransitional CellTranslationsTransphosphorylasesTransplantationWorkadulthoodallelic variantalveolar epitheliumalveolar progenitoralveolar stem cellarmautophagybalancebalance functionbiological signal transductioncell metabolismcellular metabaolismcellular reprogrammingcellular targetingdiffuse interstitial pulmonary fibrosisdisease of the lungdisorder of the lungendophenotypeendoplasmic reticulum stressepigenomicsfibrogenesisfibrosis in the lungfibrotic lunggene defectgenetic variantgenome mutationgenomic variantglobal gene expressionglobal transcription profilehuman diseaseidiopathic pulmonary fibrosisin vitro Modelin vivoinduced pluripotent stem cells derived from patientsinduced pluripotent stem cells from patientsinjury and repairknockin micelung disorderlung fibrosismembrane structuremitochondrialmitochondrial dysfunctionmolecular profilemolecular signaturemortalitymouse modelmurine modelmutantmutant allelenew drug treatmentsnew drugsnew pharmacological therapeuticnew therapeuticsnew therapynext generation therapeuticsnintedanibnovelnovel drug treatmentsnovel drugsnovel pharmaco-therapeuticnovel pharmacological therapeuticnovel therapeuticsnovel therapyolder adultolder adulthoodpathophysiologypathwaypatient derived human iPSpatient derived human iPSCpatient derived human induced pluripotent stem cellpatient derived iPSpatient derived iPSCpatient derived induced pluripotent cellspatient derived induced pluripotent stem cellspatient-derived pluripotent stem cellspharmacologicpreclinical findingspreclinical informationprogenitor cell functionprogenitor functionprogramsprotein homeostasisproteostasisprototyperepairrepairedrespiratory mechanismresponsesocial rolestem and progenitor cell functionstem and progenitor functionstem cell functionsurfactant protein Ctooltranscriptometranscriptomicstranslationtransplant
Description preview
ABSTRACT
Idiopathic Pulmonary fibrosis (IPF) is a devastating interstitial lung disease (ILD) characterized by disruption of
distal lung architecture that results in scar formation, abnormal gas exchange, and respiratory failure. Barriers
to better IPF outcomes have included an incomplete understanding of its pathophysiologic underpinnings and
a…
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