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. 2015 Feb;145(2):93–106. doi: 10.1085/jgp.201411304

Figure 8.

Figure 8.

The gating pore current generated by R222Q and R225W mutations is permeant in both activated and resting states. The VSD of domain I of mutated Nav1.5 is shown in their resting (green), activated (red), and immobilized (orange) states. These states are represented during the cardiac AP. The gating pore permeation pathway is open during the plateau phase of the AP, leading to an outward K+ leak and an inward Na+ leak. Because of S4 immobilization, the gating pore is also permeant to Na+ at the end of the AP.