ABCMdb

PMID: 14532265

Zhang XM, Wang XT, Yue H, Leung SW, Thibodeau PH, Thomas PJ, Guggino SE
Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.
J Biol Chem. 2003 Dec 19;278(51):51232-42. Epub 2003 Oct 7., 2003-12-19 [PubMed]

Sentences

No. Mutations Sentence Comment

43 ABCC7 p.Trp1282* Cell Culture-The IB3-1 (IB3) cell line is an SV40-transformed line derived from the bronchial epithelium of a cystic fibrosis patient with the ⌬F508/W1282X genotype (X ϭ stop mutation). Login to comment 44 ABCC7 p.Trp1282* The W1282X mutation produces an unstable mRNA (16). Login to comment 136 ABCC7 p.Trp1282* Gentamicin enhances read-through of the W1282X deletion (24). Login to comment 143 ABCC7 p.Trp1282* Because IB3 cells have a W1282X allele that could complicate the data because of potential gentamicin-mediated read-through, we used another cell line. Login to comment 157 ABCC7 p.Trp1282* Although there could be enhanced read-through of the W1282X allele of the CFTR gene in these cells, the major effect of treatment with organic solutes seemed to be an increase in the ratio of the band C compared with the amount of band B. Login to comment