ABCMdb

PMID: 16624886

Wang Y, Bartlett MC, Loo TW, Clarke DM
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones.
Mol Pharmacol. 2006 Jul;70(1):297-302. Epub 2006 Apr 19., [PubMed]

Sentences

No. Mutations Sentence Comment

30 ABCC7 p.His1085Arg Wild-type, ⌬F508, and H1085R CFTR cDNAs were inserted into the pcDNA3 vector (Invitrogen Canada Inc., Burlington, ON, Canada) as described previously (Loo et al., 2006). Login to comment 138 ABCC7 p.His1085Arg It has been reported that it was possible to specifically rescue CFTR processing mutants containing a processing mutation in the front half of the protein (⌬F508) by coexpression with a CFTR NH2-half molecule whereas processing mutations located in the back half of the protein (H1085R) could be rescued by coexpression with a CFTR COOH-half molecule (Cormet-Boyaka et al., 2004). Login to comment 140 ABCC7 p.His1085Arg Therefore, we tested whether a specific CFTR pharmacological chaperone such as CFpot-532 could rescue a CFTR processing mutant with a mutation in the COOH half of the molecule (H1085R). Login to comment 141 ABCC7 p.His1085Arg HEK 293 cells transiently expressing CFTR processing mutant H1085R were incubated in the presence or absence of 10 ␮M CFpot-532 for 48 h. Whole-cell extracts were subjected to immunoblot analysis. Login to comment 142 ABCC7 p.His1085Arg Figure 7 shows that expression of CFTR H1085R in the presence of 10 ␮M CFpot-532 promoted maturation of the protein. Login to comment 170 ABCC7 p.His1085Arg HEK 293 cells were transiently transfected with CFTR wild-type or H1085R cDNAs and then expressed in the absence (-) or presence (ϩ) of 10 ␮M CFpot-532 for 48 h. Whole-cell extracts were subjected to immunoblot analysis with a CFTR polyclonal antibody. Login to comment