ABCMdb

PMID: 10744680

Yue H, Devidas S, Guggino WB
The two halves of CFTR form a dual-pore ion channel.
J Biol Chem. 2000 Apr 7;275(14):10030-4., 2000-04-07 [PubMed]

Sentences

No. Mutations Sentence Comment

5 ABCC7 p.Arg334Trp ABCC7 p.Arg347Pro In further studies, a "dual-R mutant," R334W and R347P in the transmembrane segment 6 of the first half of CFTR, severely impaired the large conductance channel without affecting the small conductance channel. Login to comment 76 ABCC7 p.Arg334Trp ABCC7 p.Arg347Pro We also tested the Cl- conduction mutations, R334W and R347P. Login to comment 79 ABCC7 p.Arg334Trp ABCC7 p.Arg347Pro As expected the "dual-arginine" mutants, R334W/R347P, in full-length CFTR severely reduced whole cell Cl- currents in airways cells transiently transfected with this mutant. Login to comment 122 ABCC7 p.Arg334Trp ABCC7 p.Arg347Pro Dual arginine mutants R334W and R347P in the transmembrane segment six of TMD1 eliminate the large conductance but retain the small conductance of CFTR. Login to comment 165 ABCC7 p.Thr1134Phe McDough observed (12) that wild-type CFTR and T1134F mutant CFTR occasionally showed a long-lived subconductance state with amplitude ϳ60% of the full conductance in Xenopus oocytes. Login to comment