ABCMdb

PMID: 10970820

Rajan S, Cacalano G, Bryan R, Ratner AJ, Sontich CU, van Heerckeren A, Davis P, Prince A
Pseudomonas aeruginosa induction of apoptosis in respiratory epithelial cells: analysis of the effects of cystic fibrosis transmembrane conductance regulator dysfunction and bacterial virulence factors.
Am J Respir Cell Mol Biol. 2000 Sep;23(3):304-12., [PubMed]

Sentences

No. Mutations Sentence Comment

44 ABCC7 p.Trp1282* IB-3 cells (∆F508/ W1282X), a human bronchial epithelial cell line, and C-38 cells, the corrected line which expresses an episomal copy of a truncated form of CFTR and exhibits normal physiology but lacks the first extracellular domain of CFTR (17), were obtained from P. Zeitlin (Johns Hopkins University, Baltimore, MD). Login to comment 84 ABCC7 p.Ser489* Immunohistochemistry Mice homozygous for the CFTR S489X (null) mutation or wild-type littermate controls (cftr -/- and ϩ/ϩ) were inoculated with P. aeruginosa impregnated in agar beads as previously described (10). Login to comment 156 ABCC7 p.Trp1282* Thus, cells with CFTR dysfunction, whether due to the ∆F508/W1282X or homozygous ∆F508 mutations expected to accumulate in the ER, Cl-channel dysfunction due to overexpression of the R-domain with normal CFTR localization on the cell surface, or lack of CFTR expression, were all similarly susceptible to the induction of apoptosis and did not differ significantly from control strains with normal CFTR activity. Login to comment 219 ABCC7 p.Trp1282* Mistrafficking of ∆F508/ W1282X or ∆F508 CFTR did not prevent apoptosis after PAO1 infection, suggesting that localization of normal CFTR on the cell surface (32) is not involved in the induction of apoptosis. Login to comment