Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2000-8-31
pubmed:abstractText
We have permanently reversed the lethal phenotype in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR)-deficient (knockout) mouse after in utero gene therapy with an adenovirus containing the cftr gene. The gene transfer targeted somatic stem cells in the developing lung and intestine, and these epithelial surfaces demonstrated permanent developmental changes after treatment. The survival statistics from the progeny of heterozygote-heterozygote matings after in utero cftr gene treatment demonstrated an increased mortality in the homozygous normal pups, indicating that overexpression during development was detrimental. The lungs of these pups revealed accelerated secretory cell proliferation and differentiation. The extent of proliferation and differentiation in the secretory cells of the lung parenchyma after in utero transfer of the cftr gene was evaluated with morphometric and biochemical analyses. These studies provide further support of the regulatory role of the cftr gene in the development of the secretory epithelium.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
1040-0605
pubmed:author
pubmed:issnType
Print
pubmed:volume
279
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
L333-41
pubmed:dateRevised
2006-4-21
pubmed:meshHeading
pubmed-meshheading:10926557-Adenoviridae, pubmed-meshheading:10926557-Animals, pubmed-meshheading:10926557-Cell Differentiation, pubmed-meshheading:10926557-Cell Division, pubmed-meshheading:10926557-Cystic Fibrosis, pubmed-meshheading:10926557-Cystic Fibrosis Transmembrane Conductance Regulator, pubmed-meshheading:10926557-DNA, pubmed-meshheading:10926557-Disease Models, Animal, pubmed-meshheading:10926557-Drug Administration Routes, pubmed-meshheading:10926557-Epithelial Cells, pubmed-meshheading:10926557-Fetal Diseases, pubmed-meshheading:10926557-Gene Therapy, pubmed-meshheading:10926557-Gene Transfer Techniques, pubmed-meshheading:10926557-Genetic Vectors, pubmed-meshheading:10926557-Heterozygote, pubmed-meshheading:10926557-Homozygote, pubmed-meshheading:10926557-Intestines, pubmed-meshheading:10926557-Lung, pubmed-meshheading:10926557-Mice, pubmed-meshheading:10926557-Mice, Knockout, pubmed-meshheading:10926557-Stem Cells, pubmed-meshheading:10926557-Survival Rate
pubmed:year
2000
pubmed:articleTitle
CFTR modulates lung secretory cell proliferation and differentiation.
pubmed:affiliation
Laboratory of Molecular Genetics, Alton Ochsner Medical Foundation, New Orleans 70121, USA. janlarson@hotmail.com
pubmed:publicationType
Journal Article