ABCMdb

PMID: 14688350

Kowalski MP, Pier GB
Localization of cystic fibrosis transmembrane conductance regulator to lipid rafts of epithelial cells is required for Pseudomonas aeruginosa-induced cellular activation.
J Immunol. 2004 Jan 1;172(1):418-25., 2004-01-01 [PubMed]

Sentences

No. Mutations Sentence Comment

243 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp There are even some CFTR mutant proteins that are expressed on the cell surface, such as the G551D protein (41, 42), but are still associated with a high susceptibility of patients to P. aeruginosa infection. These non-⌬F508 CFTR mutant alleles may not properly respond to P. aeruginosa, as we recently showed that transgenic mice homozygous for the G551D Cftr allele are unable to produce a lung epithelial cell apoptotic reaction in response to P. aeruginosa infection (22). Login to comment 245 ABCC7 p.Gly551Asp That it is CFTR in epithelial cells that is key to resistance to P. aeruginosa lung infection was shown in an elegant experiment by Wainwright and colleagues (43), who expressed wild-type CFTR selectively in either the lung epithelial cells or alveolar macrophages of mice homozygous for the G551D allele of Cftr and found that recovery of resistance to P. aeruginosa infection approaching that of wild-type mice was only achieved in the mice expressing wild-type CFTR in the epithelium. Login to comment