2006
Engraftment of Donor‐Derived Epithelial Cells in Multiple Organs Following Bone Marrow Transplantation into Newborn Mice
Bruscia EM, Ziegler EC, Price JE, Weiner S, Egan ME, Krause DS. Engraftment of Donor‐Derived Epithelial Cells in Multiple Organs Following Bone Marrow Transplantation into Newborn Mice. Stem Cells 2006, 24: 2299-2308. PMID: 16794262, DOI: 10.1634/stemcells.2006-0166.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnimals, NewbornBone Marrow TransplantationCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsFemaleFluorescent Antibody TechniqueHematopoietic Stem Cell TransplantationIn Situ Hybridization, FluorescenceMaleMiceMice, Inbred C57BLMice, Inbred StrainsMice, TransgenicRNA, MessengerY ChromosomeConceptsBone marrow-derived cellsMarrow-derived epithelial cellsBone marrow transplantationNewborn miceEpithelial cellsMarrow transplantationGI tractBone marrow-derived epithelial cellsDonor-derived epithelial cellsDoses of busulfanMarrow-derived cellsEngraftment of donorIrradiated adult recipientsMyeloablative regimenPreparative regimenAdult recipientsDifferent regimensEngrafted miceHematopoietic engraftmentGastrointestinal tractSurvival advantageTherapeutic benefitAdult miceMultiple organsBone marrowAssessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation
Bruscia EM, Price JE, Cheng EC, Weiner S, Caputo C, Ferreira EC, Egan ME, Krause DS. Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation. Proceedings Of The National Academy Of Sciences Of The United States Of America 2006, 103: 2965-2970. PMID: 16481627, PMCID: PMC1413802, DOI: 10.1073/pnas.0510758103.Peer-Reviewed Original ResearchConceptsCftr-/- miceEpithelial cellsNasal epitheliumBM-derived cellsBone marrow transplantationWild-type BMAirway epithelial cellsCystic fibrosis transmembrane conductance regulator (CFTR) activityCystic fibrosis miceRare epithelial cellsCftr-null miceMarrow transplantationBM transplantationFibrosis miceRespiratory tractCFTR activityGI tractBone marrowGastrointestinalChloride secretionCFTR-dependent chloride secretionIndividual miceTransplantationDifferent dosesMice
2002
Isolation of CF cell lines corrected at ΔF508-CFTR locus by SFHR-mediated targeting
Bruscia E, Sangiuolo F, Sinibaldi P, Goncz KK, Novelli G, Gruenert DC. Isolation of CF cell lines corrected at ΔF508-CFTR locus by SFHR-mediated targeting. Gene Therapy 2002, 9: 683-685. PMID: 12032687, DOI: 10.1038/sj.gt.3301741.Peer-Reviewed Original ResearchConceptsSmall fragment homologous replacementAllele-specific PCRPrimer extension productsCell linesCFTR mRNAHuman airway epithelial cellsAllele-specific PCR amplificationΔF508 mutationCF transmembrane conductance regulator (CFTR) proteinExon 10CF cell lineMutant cell linesPCR amplificationAirway epithelial cellsEpithelial cellsGenomic DNA fingerprintingEndogenous CFTRHomologous replacementRegulator proteinDNA fingerprint analysisGenomic DNAWT mRNADNA fragmentsΔF508 alleleSequence analysis