ABCMdb

ABCC7 p.Glu1126Arg

None has been submitted yet.

Publications

PMID: 25024266 [PubMed] Cui G et al: "Three charged amino acids in extracellular loop 1 are involved in maintaining the outer pore architecture of CFTR."

No. Sentence Comment

118 Fig. S6 shows representative I-V plots of double mutants R104E/ E116R- and R117E/E1126R-CFTR and their rectification ratio. Login to comment
224 Tab l e 1 Reversal potentials of WT-CFTR and mutants in ND96 bath solution CFTR n Vrev mV WT 14 &#e032;27.75 &#b1; 0.78 R334A 6 &#e032;12.15 &#b1; 1.64a R117A 6 &#e032;22.51 &#b1; 0.85a E116R 5 &#e032;21.45 &#b1; 1.14a K114D 5 &#e032;24.68 &#b1; 3.22 D110R 5 &#e032;27.64 &#b1; 3.29 R104E 5 &#e032;21.15 &#b1; 1.08a R899C 4 &#e032;25.30 &#b1; 3.94 D891C 6 &#e032;25.81 &#b1; 2.44 K892E 5 &#e032;23.70 &#b1; 3.62 E1124R 5 &#e032;18.32 &#b1; 0.43a E1126R 5 &#e032;20.67 &#b1; 3.16b R117E/E1126R 6 &#e032;23.06 &#b1; 1.37b R104E/E116R 6 &#e032;27.17 &#b1; 1.08 Values are mean &#b1; SEM. Login to comment
380 Sample current traces for both E1126R- and R117E/ E1126R-CFTR are shown in Fig. 12 A. Login to comment
403 We also compared the fractional burst duration represented by s1, s2, and f states for E1126R- and R117E/E1126R-CFTR and found that E1126R exhibited a significantly higher fraction of both s1 and s2 states than WT, whereas the distribution for double mutant R117E/E1126R-CFTR was similar to WT-CFTR, exhibiting mainly the f state (a nearly complete rescue; Fig. 12 D). Login to comment
405 E1126R-CFTR mainly opened to the full open state but with frequent brief transitions to the s1 and s2 states. Login to comment
406 R117E/E1126R-CFTR opened to a full open state much more often compared with R117A-CFTR (Fig. 2). Login to comment
407 Mean burst duration for both E1126R- and R117E/ E1126R-CFTR are summarized in Fig. 12 B. Login to comment
408 The mean burst duration of R117E/E1126R-CFTR was significantly longer than that of R117A- and R117C-CFTR. Login to comment
410 (A) Representative single-channel current traces of E1126R-, R117E/E1126R-, R117C-, and R117C/E1126C-CFTR recorded under the same experimental conditions as Fig. 2 and their all-points amplitude histograms (right). Login to comment
412 #, P < 0.01 indicates a significant difference between WTand R117C-CFTR; **, P < 0.01 indicates a significant difference between WTand E1126R-CFTR, between R117C and R117E/E1126R-CFTR, and between R117C and R117C/E1126C. Login to comment
417 (D) Mean fraction of open burst duration is plotted for each open conductance state of E1126R- and R117E/E1126R-CFTR. Login to comment