ABCMdb

PMID: 11307750

Scheid P, Kempster L, Griesenbach U, Davies JC, Dewar A, Weber PP, Colledge WH, Evans MJ, Geddes DM, Alton EW
Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.
Eur Respir J. 2001 Jan;17(1):27-35., [PubMed]

Sentences

No. Mutations Sentence Comment

12 ABCC7 p.Gly551Asp Cos-7 cell transfection with plasmids expressing DF508 or G551D mutant CFTR protein resulted in increased activation of a p50-containing NF-kB complex, but IL-8 secretion was similar to wild-type cells. Login to comment 110 ABCC7 p.Gly551Asp Transfections The 6.2 kb wild-type, DF508 and G551D CFTR complementary deoxyribonucleic acids (cDNAs) were cloned into the Not I site of the eukaryotic expression vector pCMVb (Clontech Laboratories UK Ltd, Basingstoke, UK), from which the b-galactosidase reporter gene was removed through Not I digestion. Login to comment 140 ABCC7 p.Gly551Asp ABCC7 p.Gly542* ABCC7 p.Gly542* Group 1 (n=13) with mistraf- ®cking mutations on both alleles (DF508 homozygotes and a DF508/I507 compound heterozygote), group 2 (n=6) with only one mistraf®cking mutation (DF508 compound heterozygotes, the second mutation being R553X62, 1717-1GAA, 621+1GAT, G551D62), and group 3 (n=3) without mistraf®cking mutations (genotypes being G542X/3849+10kB GAT, G542X/ R533X, 1717-1GAA/G551D). Login to comment 166 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp The effects of transfection with wild-type, DF508 and G551D cystic ®brosis transmembrane conductance regulator on nuclear factor-kB activity and interleukin-8 secretion Cos-7 cells transfected with either DF508 or G551D CFTR plasmids, exhibited a signi®cant increase in p50-containing complex II when compared to wild-type transfectants (n=10, p<0.01) (®g. 7). Login to comment 175 ABCC7 p.Gly551Asp Overexpression of DF508 and G551D mutant CFTR increased p50 activity in Cos-7 cells when compared to overexpression of wild-type CFTR; however, this increase was not accompanied by an increase in IL-8 secretion. Login to comment 224 ABCC7 p.Gly551Asp In an attempt to further determine the effect of ER overload, the present study transiently overexpressed wild-type, DF508 or G551D CFTR protein in Cos-7 cells. Login to comment 226 ABCC7 p.Gly551Asp A signi®cant increase in a p50 containing protein/DNA complex (complex II) was detected after DF508 or G551D overexpression when compared to overexpression of the wild-type protein. Login to comment