ABCMdb

PMID: 17237149

Bossard F, Robay A, Toumaniantz G, Dahimene S, Becq F, Merot J, Gauthier C
NHE-RF1 protein rescues DeltaF508-CFTR function.
Am J Physiol Lung Cell Mol Physiol. 2007 May;292(5):L1085-94. Epub 2007 Jan 19., [PubMed]

Sentences

No. Mutations Sentence Comment

61 ABCC7 p.Lys1468* ABCC7 p.Lys1468* To construct the ⌬F508-K1468X mutant plasmid, the KpnI/HpaI restriction fragment of the pcDNA3-⌬F508-CFTR plasmid, containing the ⌬F508 mutation, was cloned in frame in pcDNA3-CFTR-K1468X. Login to comment 174 ABCC7 p.Lys1468* As illustrated in Fig. 7, coexpression of ⌬F508-K1468X-CFTR with NHE-RF1 in MDCK cells did not result in any significant changes in the baseline or the forskolin-stimulated Cl-channel activity. Login to comment 190 ABCC7 p.Lys1468* Effect of NHE-RF1 overexpression on ⌬F508-K1468X in MDCK cells. Login to comment 191 ABCC7 p.Lys1468* MDCK cells were microinjected with ⌬F508-K1468X (30 ␮g/ml) and NHE-RF1 (50 ␮g/ml) cDNAs. Membrane permeability to halides (p, in min-1 ) was measured under baseline conditions (open bars) and after application of 10 ␮M Fsk (filled bars). Login to comment 197 ABCC7 p.Lys1468* Finally, the involvement of PDZ domains in such effect is strongly suggested by the use of ⌬F508-K1468X-CFTR, a ⌬F508 mutant lacking the 13 last amino acids involved in PDZ interaction with NHE-RF1. Login to comment 224 ABCC7 p.Lys1468* Indeed, ⌬F508-K1468X-CFTR exhibited a low basal Cl-channel activity that was insensitive to forskolin in the presence of NHE-RF1. Login to comment