ABCMdb

PMID: 15130785

Gong X, Linsdell P
Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions.
Arch Biochem Biophys. 2004 Jun 1;426(1):78-82., [PubMed]

Sentences

No. Mutations Sentence Comment

35 ABCC7 p.Arg334Leu ABCC7 p.Arg334Gln ABCC7 p.Arg334Cys ABCC7 p.Arg334His ABCC7 p.Arg334Glu ABCC7 p.Arg334Lys Results and discussion Previously we characterized the properties of six different R334 mutants (R334C, R334E, R334H, R334K, R334L, and R334Q) [19]. Login to comment 37 ABCC7 p.Arg334Trp In the present study, we have added a seventh (R334W), however the expression of this mutant in BHK cells appeared weak and we were not able to characterize its macroscopic current properties as previously described for the other mutants [19]. Login to comment 38 ABCC7 p.Arg334Ala ABCC7 p.Arg334Thr Two other mutants, R334A and R334T, did not express any detectable Cl current in BHK cells. Login to comment 43 ABCC7 p.Arg334Trp ABCC7 p.Arg334Lys Each of these traces shows the activity of two active CFTR channels, except for R334K and R334W (one active channel). Login to comment 50 ABCC7 p.Arg334His ABCC7 p.Arg334Lys Comparison of unitary current amplitude at )100 mV showed that, compared to wild type, mean current was reduced by between 53 (in R334K) and 91% (in R334H) (Fig. 2B). Login to comment 51 ABCC7 p.Arg334Trp ABCC7 p.Arg334Cys Others have previously shown that both R334C [20] and R334W [24] are associated with reduced unitary conductance. Login to comment 59 ABCC7 p.Arg334Trp R334W is not included in this analysis since it did not give large enough macroscopic currents to allow these functional properties to be characterized. Login to comment 65 ABCC7 p.Arg334Leu ABCC7 p.Arg334Cys ABCC7 p.Arg334His ABCC7 p.Arg334Glu ABCC7 p.Arg334Lys (A) Unitary current-voltage relationships for each of the channel variants shown in Fig. 1: (d) wild type, (r) R334C, (j) R334E, (}) R334H, (s) R334K, () R334L, (.) Login to comment 66 ABCC7 p.Arg334Trp ABCC7 p.Arg334Gln R334Q, and (,) R334W. Login to comment 98 ABCC7 p.Arg334Trp ABCC7 p.Arg334Leu ABCC7 p.Arg334Gln Interestingly, three of these mutations-R334L, R334Q, and R334W-have been identified in cystic fibrosis patients [29]. Login to comment