Fig. 4.
SUDC DNMs G402S and V396L of the CACNA1C gene lead to decreased Ba2+ current density and slowed inactivation. (A) A schematic diagram showing the positions of the two SUDC DNMs in CACNA1C. Gray highlight connotes a subtle change, white highlight, a more drastic alteration. (B) I-V curves showing the overall activation profile with 30 mM Ba2+ as charge carrier. (C) Averaged traces from wild-type, G402S, and V396L cells were superimposed to show the differences in inactivation. (D) Quantification of the ratio between the residual current at 1,000 ms and the peak current (r1000). **P < 0.01, ***P < 0.001 by unpaired two-tailed Student’s t test. (E) Activation curves for WT and V396L obtained by Boltzmann fit to normalized tail current amplitudes (Vh = -2.9 mV and -2.2 mV, k = 12.71 and 16.86, respectively).