Assessment of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity in CFTR-Null Mice after Bone Marrow Transplantation

Several studies have demonstrated that bone marrow (BM)-derived cells give rise to rare epithelial cells in the gastrointestinal (GI) and respiratory tracts after BM transplantation into myeloablated recipients. We investigate whether, after transplantation of cystic fibrosis transmembrane conductan...

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Published inProceedings of the National Academy of Sciences - PNAS Vol. 103; no. 8; pp. 2965 - 2970
Main Authors Bruscia, Emanuela M., Price, Joanna E., Cheng, Ee-Chun, Weiner, Scott, Caputo, Christina, Ferreira, Elisa C., Egan, Marie E., Krause, Diane S.
Format Journal Article
LanguageEnglish
Published United States National Academy of Sciences 21.02.2006
National Acad Sciences
Subjects
Anatomy & physiology
Animals
Biological Sciences
Bone marrow
Bone Marrow Transplantation
Cystic fibrosis
Cystic Fibrosis - therapy
Cystic Fibrosis Transmembrane Conductance Regulator - analysis
Cystic Fibrosis Transmembrane Conductance Regulator - genetics
Epithelial cells
Epithelium
Gastric Mucosa - chemistry
Gastrointestinal tract
Genetic Therapy
Intestinal Mucosa - chemistry
Irradiation
Lungs
Messenger RNA
Mice
Mice, Inbred CFTR
Nasal Mucosa - chemistry
Proteins
RNA, Messenger - analysis
Rodents
Stem cells
Tissue transplantation
Tissues
Transplantation
Transplants & implants
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Summary:Several studies have demonstrated that bone marrow (BM)-derived cells give rise to rare epithelial cells in the gastrointestinal (GI) and respiratory tracts after BM transplantation into myeloablated recipients. We investigate whether, after transplantation of cystic fibrosis transmembrane conductance regulator (CFTR)-positive BM-derived cells, BM-derived GI and airway epithelial cells can provide CFTR activity in the GI tract and nasal epithelium of recipient cystic fibrosis mice. CFTR-/-mice were transplanted with wild-type BM after receiving different doses of irradiation, and CFTR activity was assessed in vivo in individual mice over time by using rectal and nasal potential difference analyses and in vitro by Ussing chamber analysis. The data suggest that rare BM-derived epithelial cells in the GI and nasal epithelium detected in CFTR-/transplanted mice provide a modest level of CFTR-dependent chloride secretion. Detection of CFTR mRNA and protein in tissues of transplanted CFTR-/-mice supports these data.
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Communicated by Steven C. Hebert, Yale University School of Medicine, New Haven, CT, December 21, 2005
Author contributions: E.M.B., M.E.E., and D.S.K. designed research; E.M.B., J.E.P., E.-C.C., S.W., C.C., and E.C.F. performed research; E.M.B. contributed new reagents/analytic tools; E.M.B., J.E.P., E.-C.C., S.W., C.C., E.C.F., M.E.E., and D.S.K. analyzed data; and E.M.B., M.E.E., and D.S.K. wrote the paper.
ISSN:0027-8424
1091-6490
DOI:10.1073/pnas.0510758103