Figure 1 | Scientific Reports

Figure 1

From: Substitutions of the S4DIV R2 residue (R1451) in NaV1.4 lead to complex forms of paramyotonia congenita and periodic paralyses

Figure 1

Whole-cell properties of the WT, R1451C, and R1451L channels. (A) Representative whole-cell traces recorded from tsA201 cells expressing WT, R1451C, or R1451L channel. Currents were elicited from a holding potential of −140 mV and were depolarized to potentials ranging from −140 to +60 mV in 10-mV increments in 50-ms steps (see protocol in the inset under the R1451L traces). The dashed line represents zero current. (B) Normalized current/voltage relationships of the WT (n = 10), R1451C (n = 18), and R1451L (n = 18) channels. Current densities were measured by normalizing current amplitudes to membrane capacitance and were plotted against voltage. The experimental protocol was the same as in (A). (C) Histogram showing the average peak current densities recorded at −10 mV of the WT, R1451C, and R1451L channels. The R1451C and R1451L current densities decreased by 63% (***P < 0.001) and 46% (***P < 0.001), respectively, compared to the WT channel. R1451C current density decreased by 31% (*P < 0.05) compared to the R1451L channel.

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