grant
Rare Bronchiectatic Diseases Consortium
Organization UNIV OF NORTH CAROLINA CHAPEL HILLLocation CHAPEL HILL, UNITED STATESPosted 1 Sept 2025Deadline 31 May 2030
NIHUS FederalResearch GrantFY2025A1PIAAT deficiencyAccelerationAirway DiseaseAnti-Bacterial AgentsApicalBacterial InfectionsBile AcidsBiological MarkersBody TissuesBronchial DiseasesBronchiectasisBronchoscopyCAT scanCT X RayCT XrayCT imagingCT scanCell BodyCellsChronicClinical TrialsCollaborationsCollectionCommon CoreComputed TomographyCoughingDNA mutationDataDefectDevelopmentDiagnosisDiseaseDisorderDysfunctionER stressEctasiaElementsEpitheliumEsteroproteasesEventExhibitsFrequenciesFunctional disorderGene AlterationGene MutationGene TranscriptionGenesGenetic ChangeGenetic DiseasesGenetic TranscriptionGenetic defectGenetic mutationGoalsHealthHost DefenseHost Defense MechanismHyper-IgE SyndromeHyperimmunoglobulin E SyndromeHyperimmunoglobulin E-Recurrent Infection SyndromeHypoxiaHypoxicImmuneImmune GlobulinsImmunesImmunoglobulinsIn VitroInfectionInflammationInflammatoryInitiation FactorsInvestigationKartagener SyndromeKartagener TriadLifeLungLung DiseasesLung Respiratory SystemLung symptomMediatingModificationMouth microbiomeMucous body substanceMucusMutationNon-Polyadenylated RNAObstructionOrphan DiseaseOxygen DeficiencyPathogenesisPathologic DilatationPathological DilatationPathway interactionsPatientsPeptidasesPeptide HydrolasesPeptide Initiation FactorsPersonsPhysiopathologyPolynesian bronchiectasisPrevalencePrimary Ciliary DyskinesiasProductionProtease GeneProteasesProteinasesProteinsProteolytic EnzymesProteomicsPulmonary DiseasesPulmonary DisorderRNARNA ExpressionRNA Gene ProductsRare DiseasesRare DisorderRespiratory EpitheliumRibonucleic AcidRoleSTAT3STAT3 geneSamplingSputumStructure of respiratory epitheliumTechniquesTechnologyTestingTissuesTomodensitometryTranscriptionTranslation Initiation FactorTranslational Initiation FactorViral DiseasesVirusVirus DiseasesX-Ray CAT ScanX-Ray Computed TomographyX-Ray Computerized TomographyXray CAT scanXray Computed TomographyXray computerized tomographya1-antitrypsin deficiencyairflow limitationairflow obstructionairway epitheliumairway limitationairway microbiomeairway obstructionalpha 1 Antiproteasealpha 1-Antiproteinasealpha 1-Antitrypsinalpha 1-Antitrypsin Deficiencyalpha 1-Antitrypsin Trypsin Inhibitoralpha 1-Protease Inhibitoralpha 1-Proteinase Inhibitoralpha-1-anti-trypsin deficiencyalpha1-antitrypsin deficiencyanti-bacterialaspiratebacteria infectionbacterial diseasebio-markersbiologic markerbiomarkerbiomarker identificationbronchus disordercatscancomputed axial tomographycomputer tomographycomputerized axial tomographycomputerized tomographydesigndesigningdevelopmentaldisease of the lungdisorder of the lungendoplasmic reticulum stressexperiencegene defectgenetic conditiongenetic disordergenome mutationhypoimmunityidentification of biomarkersidentification of new biomarkersimmune deficiencyimmunodeficiencyimprovedin vitro testinglung disorderlung functionmarker identificationmorphometrymucousmucus clearancemutant allelenew drug treatmentsnew drugsnew markernew pharmacological therapeuticnew therapeuticsnew therapynext generation therapeuticsnon-contrast CTnoncontrast CTnoncontrast computed tomographynovelnovel biomarkernovel drug treatmentsnovel drugsnovel markernovel pharmaco-therapeuticnovel pharmacological therapeuticnovel therapeuticsnovel therapyobstructed airflowobstructed airwayoral microbial communityoral microbiomeoral microbiotaoral microfloraorphan disorderpathophysiologypathwaypatient populationpulmonarypulmonary functionpulmonary symptomrare genetic diseaserare genetic disorderrespiratoryrespiratory airway obstructionrespiratory microbiomerespiratory tract epitheliumrespiratory tract microbiomeresponsesocial rolespecific biomarkerstherapeutic agent developmenttherapeutic developmenttraitviral infectionvirus infectionvirus-induced diseaseα-1 anti-trypsin deficiencyα-1-antitrypsin deficiencyα1-Antitrypsinα1-Antitrypsin Deficiencyα1-Proteinase Inhibitor
Description preview
PROJECT 2 ABSTRACT
Rare genetic diseases are associated with bronchiectasis (BE) as a severe pulmonary disease manifestation.
People with rare genetic diseases with BE experience increased sputum production, loss of lung function, an
increased frequency of respiratory exacerbations, and life limitation. There is a great need for novel therapies…
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