ABCMdb

PMID: 21594776

Loo TW, Clarke DM
Repair of CFTR folding defects with correctors that function as pharmacological chaperones.
Methods Mol Biol. 2011;741:23-37., [PubMed]

Sentences

No. Mutations Sentence Comment

147 ABCC7 p.His1085Arg ABCC7 p.Gln1071Pro HEK 293 cells expressing the CFTR processing mutants, F508del, Q1071P, or H1085R, were incubated with media containing no corrector (-) or 0.05 mM VX-325 plus 0.015 mM corr-4a (+). Login to comment 247 ABCC7 p.Val510Ala To improve the maturation efficiency of Cys-less CFTR, cysteines 590 and 592 were replaced with leucines (16) and valine 510 was changed to alanine (17). Login to comment 252 ABCC7 p.Val510Cys ABCC7 p.Ala1067Cys The following protocols describe disulfide cross-linking between cysteines introduced into NBD1 (V510C) and the fourth intracellular loop in TMD2 (A1067C) of a F508del-CFTR processing mutant expressed in the presence or the absence of correctors. Login to comment 263 ABCC7 p.Val510Cys ABCC7 p.Ala1067Cys The next day, add 0.17 mg of (V510C)/(A1067C)/ F508del-CFTR cDNA to sterile water to give a total volume of 7.65 ml. Login to comment 282 ABCC7 p.Val510Cys Effect of correctors on cross-linking of CFTR mutant F508del/V510C (NBD1)/A1067C(TMD2). Login to comment