ABCC7 p.Arg1128Gln

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PMID: 18449561 [PubMed] Zhou JJ et al: "Identification of positive charges situated at the outer mouth of the CFTR chloride channel pore."
No. Sentence Comment
62 To investigate the role of charge on these residues in controlling I-V shape, neutral substitutions (R104Q, R117Q, K335A, R1128Q) were also investigated.
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ABCC7 p.Arg1128Gln 18449561:62:122
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91 All mutants depicted (R104Q, R117Q, K335A, R1128Q, R104E, R117E, K335E, R1128E) showed rectification ratios significantly different from wild type (asterisk P<0.05).
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ABCC7 p.Arg1128Gln 18449561:91:43
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120 As shown in Fig. 8a, while Pt(NO2)4 2-induced strong inward rectification in wild type (as a result of strongly voltage-dependent current inhibition) [26], this effect was less marked in R104Q, K335A, and R1128Q.
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ABCC7 p.Arg1128Gln 18449561:120:205
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122 Considering the data at +80 mV, where the inhibitory effects of Pt(NO2)4 2- are strongest, suggests that Pt(NO2)4 2- inhibition is significantly weakened in R104Q, K335A, and (to a lesser extent) R1128Q but not significantly altered in K114C, R117Q, K329A, K829Q, or R899Q (Fig. 8c).
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ABCC7 p.Arg1128Gln 18449561:122:196
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125 Similar effects were observed in R117Q and R1128Q (Fig. 9b), consistent with the minor effects of these mutations on Pt(NO2)4 2- inhibition inferred from the rectification of macroscopic currents (Fig. 8).
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ABCC7 p.Arg1128Gln 18449561:125:43
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147 b Mean fraction of control normalized current remaining in the presence of 10 mM extracellular Pt(NO2)4 2at different membrane potentials in wild type (closed circles), R104Q (closed squares), K335A (open circles), and R1128Q (open squares), quantified as the IREL in the presence of 10 mM Pt(NO2)4 2- [IREL(block)] as a fraction of that in the absence of Pt(NO2)4 2- [IREL(control)].
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ABCC7 p.Arg1128Gln 18449561:147:219
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172 In contrast, data for R107Q and R1128Q were not significantly different from wild type at any voltage (P>0.05).
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ABCC7 p.Arg1128Gln 18449561:172:32
status: NEW
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PMID: 19448737 [PubMed] Zhou JJ et al: "Evidence that extracellular anions interact with a site outside the CFTR chloride channel pore to modify channel properties."
No. Sentence Comment
67 Similar weakening of block by internal Pt(NO2)4 2-was also observed in K114C, K329A, K335A, and R1128Q (Fig. 2), as well as R334Q (Zhou et al. 2007).
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ABCC7 p.Arg1128Gln 19448737:67:96
status: NEW
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PMID: 25277268 [PubMed] Broadbent SD et al: "The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor."
No. Sentence Comment
112 To explore the role of phosphorylation further, we studied the effect of deleting the R domain from CFTR (residues 634-836) [12, 7], which removes all the major PKA/PKC Table 1 Summary of the FSK stimulation of whole cell currents and Erev shifts observed with the CFTR constructs used in this study CFTR Construct n FSK Stimulation (%&#b1;SEM) Erev shift (mV&#b1;SEM) WT (50 bc;M ATP) 5 180&#b1;96 15.0&#b1;3.6 WT (100 bc;M ATP) 6 12,000&#b1;6,000 15.2&#b1;3.0 WT (300 bc;M ATP) 8 1,200&#b1;600 17.0&#b1;3.0 WT (1 mM ATP) 24 13,000&#b1;6,000 23.7&#b1;1.8 WT (1.3 mM ATP) 9 1,400&#b1;900 16.7&#b1;2.6 WT (2 mM ATP) 24 6,100&#b1;5,300 16.7&#b1;1.6 WT (5 mM ATP) 7 1,600&#b1;1,000 20.1&#b1;4.4 WT (50 bc;M ATP + 50 bc;M P-ATP) 7 224&#b1;130 15.3&#b1;1.0 WT + Genistein 4 7,600&#b1;5,200 26.1&#b1;5.4 WT + AMP-PNP 5 2,800&#b1;2,500 21.8&#b1;5.5 WT (3 mM MgCl2) 7 28,000&#b1;17,000 18.3&#b1;3.1 R104Q 5 4,600&#b1;1,600 28.6&#b1;4.7 K114C 5 12,000&#b1;6,700 29.2&#b1;3.0 R117Q 4 33,000&#b1;20,000 30.1&#b1;3.4 K329A 5 13,000&#b1;10,000 33.7&#b1;2.1 R334Q 9 13,000&#b1;6,700 27.3&#b1;2.9 K335A 5 3,200&#b1;1,500 20.8&#b1;7.1 W401G 7 2,600&#b1;1,800 18.5&#b1;4.8 Delta-R (No Stim) 5 - 25.1&#b1;2.7 Delta-R (No FSK, Genistein) 5 140&#b1;13 22.7&#b1;3.0 Delta-R (FSK, No Genistein) 4 89&#b1;14 15.6&#b1;6.0 Delta-R (FSK + Genistein) 6 639&#b1;432 25.1&#b1;4.9 Delta-R-E1371S (No FSK) 9 - 21.4&#b1;4.8 Delta-R-E1371S (FSK) 4 2,600&#b1;1,400 15.3&#b1;4.7 K892Q 7 16,000&#b1;9,500 36.8&#b1;4.8 R899E 4 1,200&#b1;400 25.0&#b1;2.7 R899K 4 1,600&#b1;900 26.6&#b1;2.9 R899Q 7 5,400&#b1;2,800 30.0&#b1;1.3 R899Q + AMP-PNP 4 72,000&#b1;50,000 15.2&#b1;2.8 R899Q-E1371Q (No FSK) 4 - 18.4&#b1;5.9 R899Q-E1371Q (FSK) 6 107&#b1;48 15.6&#b1;3.0 R1128Q 6 14,000&#b1;6,100 41.1&#b1;4.2 Y1219G 6 3,200&#b1;2,500 19.2&#b1;3.3 E1371Q (No FSK) 6 - 25.5&#b1;3.5 E1371Q (FSK) 8 -28&#b1;9 22.3&#b1;4.0 E1371Q (FSK, No ATP, No GTP) 8 270&#b1;130 19.4&#b1;4.5 E1371Q + AMP-PNP (No FSK) 4 - 24.7&#b1;6.5 E1371Q + AMP-PNP (FSK) 8 180&#b1;170 17.4&#b1;4.0 Vector Control 4 15&#b1;38 - FSK stimulation was calculated as the percentage increase in current density at -60 mV from the Erev, after 5-min exposure to 10 bc;M FSK.
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ABCC7 p.Arg1128Gln 25277268:112:1737
status: NEW
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