ABCMdb

PMID: 12878584

Cannon CL, Kowalski MP, Stopak KS, Pier GB
Pseudomonas aeruginosa-induced apoptosis is defective in respiratory epithelial cells expressing mutant cystic fibrosis transmembrane conductance regulator.
Am J Respir Cell Mol Biol. 2003 Aug;29(2):188-97., [PubMed]

Sentences

No. Mutations Sentence Comment

3 ABCC7 p.Gly551Asp Mice with a WT Cftr allele had apoptotic cells in their lungs after P. aeruginosa infections, whereas mice homozygous for the ⌬F508 or G551D Cftr alleles showed little apoptosis in response to acute infection. Login to comment 37 ABCC7 p.Trp1282* IB3 cells are bronchial epithelial cells from a ⌬F508/W1282X patient that are adeno-12-SV40 immortalized (17). Login to comment 78 ABCC7 p.Trp1282* Similar flow cytometry experiments were performed using the cell line IB3, an adeno-12-SV40 immortalized bronchial epithelial cell line derived from a ⌬F508/W1282X patient, and the S9 derivative of the IB3 cells generated by transfection with a recombinant adeno-associated viral vector encoding WT CFTR. Login to comment 110 ABCC7 p.Gly551Asp Equivalent areas examined for TUNEL-positive cells showed a striking absence in mice homozygous for either the ⌬F508 or G551D Cftr alleles (Figures 4A and 4C), whereas heterozygous littermates had TUNEL-positive cells both in the lumen of bronchi (Figures 4B and 4D, arrows) or scattered among the columnar epithelial cells lining the bronchi (Figure 4D). Login to comment 123 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp D and E show the lungs of an infected mouse heterozygous for WT and G551D cftr, and F depicts the lungs of a homozygous G551D littermate. Login to comment 248 ABCC7 p.Gly551Asp They also thank Gloria Meulini and Fadie Coleman for help in breeding the ⌬F508 and G551D mice and Ervin Meulini for sectioning the mouse lungs. Login to comment