PMID: 10922041

Pier GB
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Proc Natl Acad Sci U S A. 2000 Aug 1;97(16):8822-8., 2000-08-01 [PubMed]
Sentences
No. Mutations Sentence Comment
133 ABCC7 p.Gly551Asp
X
ABCC7 p.Gly551Asp 10922041:133:110
status: NEW
view ABCC7 p.Gly551Asp details
Recent studies in transgenic CF mice with a stop mutation in Cftr (48), a ⌬F508 Cftr (49) allele, or a G551D Cftr allele (50) have confirmed the association between decreased lung cell internalization of P. aeruginosa in CF mice and increased bacterial burdens in lung tissue (unpublished data). Login to comment
134 ABCC7 p.Gly551Asp
X
ABCC7 p.Gly551Asp 10922041:134:43
status: NEW
view ABCC7 p.Gly551Asp details
The fact that transgenic Cftr knockout and G551D mice, both representative of alleles in humans with severe CF, have the same phenotype as ⌬F508 Cftr mice indicates that the defect in epithelial cell uptake of P. aeruginosa is not restricted to one allelic variant of Cftr. Login to comment