ABCC7 p.Arg134Gln

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PMID: 17043152 [PubMed] Aubin CN et al: "Positive charges at the intracellular mouth of the pore regulate anion conduction in the CFTR chloride channel."
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131 However, both R134Q and R1030Q did yield functional channels (Fig. 10).
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ABCC7 p.Arg134Gln 17043152:131:14
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132 R134Q Figure 7. Modification of CFTR by intracellular MTSES.
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ABCC7 p.Arg134Gln 17043152:132:0
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143 Measurable single channel activity was not observed after R134Q expression; however, Fig. 10 C shows an example of a very small apparent single channel opening frequently observed in a patch excised from an R134Q-expressing cell.
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ABCC7 p.Arg134Gln 17043152:143:58
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ABCC7 p.Arg134Gln 17043152:143:207
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144 This tiny current suggests that R134Q has an extremely small unitary conductance below the accurate resolution of single channel recording.
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ABCC7 p.Arg134Gln 17043152:144:32
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172 (A) Example leak-subtracted macroscopic I-V relationships from R134Q and R1030Q-CFTR in inside-out membrane patches with symmetrical 154 mM Cl- solu- tions, after maximal channel activation.
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ABCC7 p.Arg134Gln 17043152:172:63
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174 * indicates a significant difference from wild type at the same Cl-concentration (P < 10-4, two-tailed t test), † indicates a significant difference from R134Q at 154 mM Cl- (P < 0.005, two-tailed t test).
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ABCC7 p.Arg134Gln 17043152:174:161
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177 For R134Q, the tiny current associated with a putative channel opening is identified by a line above it.
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ABCC7 p.Arg134Gln 17043152:177:4
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207 R134Q and R1030Q do not appear consistent with loss of an intracellular surface charge.
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ABCC7 p.Arg134Gln 17043152:207:0
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208 R134Q is associated with outward rectification (Fig. 10, A and B); however, this effect shows a complex Cl-dependence that, unlike that of R303E, R303Q, and R352Q (Fig. 3 B), is not consistent with a simple surface charge effect. Furthermore, R134Q shows an extremely small apparent unitary conductance (Fig. 10 C).
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ABCC7 p.Arg134Gln 17043152:208:0
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ABCC7 p.Arg134Gln 17043152:208:243
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