grant
Patient-specific iPSCs to model and treat the inception of pulmonary fibrosis
Organization BOSTON UNIVERSITY MEDICAL CAMPUSLocation BOSTON, UNITED STATESPosted 1 Mar 2024Deadline 28 Feb 2029
NIHUS FederalResearch GrantFY202521+ years old5'-Adenylic acidATP-protein phosphotransferaseAdenosine MonophosphateAdenylic AcidAdultAdult HumanAgonistAirAlveoli progenitorAlveoli stem cellAutomobile DrivingAutophagocytosisAutoregulationBiologic ModelsBiological ModelsBiologyCRISPR interferenceCRISPR-dCas9-mediated repressionCRISPR/dCas9 interferenceCRISPR/dCas9-mediated transcriptional inhibitionCRISPRiCell BodyCellsCessation of lifeChildhoodClustered Regularly Interspaced Short Palindromic Repeats interferenceCo-cultureCocultivationCocultureCoculture TechniquesDataDeathDevelopmentDiagnosisDiseaseDisorderDistalDysfunctionEpitheliumExperimental ModelsFibrosing AlveolitisFibrosisFunctional disorderGWA studyGWASGasesGene x Environment InteractionGenesGeneticGlycolysisGoalsGxE interactionHomeostasisHumanIdiopathic Interstitial PneumoniaImpairmentIn VitroIn vivo analysisInflammatoryInterstitial Lung DiseasesKinase Family GeneLiquid substanceLungLung Alveolar EpitheliaLung DiseasesLung ParenchymaLung Respiratory SystemLung TissueLung Tissue FibrosisMediatingMesenchymalMesenchymasMesenchymeMetabolicMethodsMitochondriaModel SystemModelingModern ManOxidative PhosphorylationOxidative Phosphorylation PathwayPathogenesisPathway interactionsPatientsPhenotypePhysiological HomeostasisPhysiopathologyPluripotent Stem CellsPredispositionProtein KinasePublishingPulmonary DiseasesPulmonary DisorderPulmonary FibrosisRoleShort interfering RNASmall Interfering RNAStem Cell likeStructure of parenchyma of lungSusceptibilitySystemTestingTherapeuticThyroid Gland HormoneThyroid HormonesTimeVariantVariationadulthoodalveolar epitheliumalveolar progenitoralveolar stem cellautophagycigarette smoke exposuredevelopmentaldiffuse interstitial pulmonary fibrosisdisease causing variantdisease modeldisease of the lungdisease-causing alleledisease-causing mutationdisorder modeldisorder of the lungdrivingdruggable targeteffective therapyeffective treatmentefficacy testingenvironment effect on geneexposure to cigarette smokefibrogenesisfibrosis in the lungfluidgene environment interactiongenome wide associationgenome wide association scangenome wide association studygenomewide association scangenomewide association studyglycogen synthase a kinasehuman diseasehuman modelhydroxyalkyl protein kinaseiPSiPSCiPSCsidiopathic pulmonary fibrosisin vivoin vivo evaluationin vivo testinginduced pluripotent cellinduced pluripotent stem cellinducible pluripotent cellinducible pluripotent stem cellknock-downknockdownliquidlung disorderlung fibrosismitochondrialmitochondrial dysfunctionmodel of humanmouse modelmurine modelmutantnoveloverexpressoverexpressionpathogenic allelepathogenic variantpathophysiologypathwaypediatricphosphorylase b kinase kinasepluripotent progenitorprogenitor capacityprogenitor cell likeprogenitor cell modelprogenitor modelprogenitor-likeprotein homeostasisproteostasisrepressing CRISPR-dCas9 systemrestorationscRNA sequencingscRNA-seqself-renewself-renewalsiRNAsingle cell RNA-seqsingle cell RNAseqsingle cell expression profilingsingle cell transcriptomic profilingsingle-cell RNA sequencingsocial rolestem and progenitor cell modelstem cell based modelstem cell characteristicsstem cell derived modelstem cell modelstem-likestemnessvectorwhole genome association analysiswhole genome association study
Description preview
Project Summary
Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis leading to disruption of the gas
exchange unit and death within an average of four years from the time of diagnosis. A poor understanding of IPF
pathogenesis, in part due to a lack of reliable human disease models, has been a major hurdle to developing…
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