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. 2024 May 20;111(6):1206–1221. doi: 10.1016/j.ajhg.2024.04.019

Figure 5.

Figure 5

Biophysical characterization of group 2 variants in a ternary configuration

(A) Inactivation time constants obtained by fitting the Kv4.1-mediated current decay kinetics with a double-exponential function (τ1: circles, τ2: triangles) and the relative amplitude of the total decay accounted for by τ1 (number of observations indicated).

(B) Recovery time constants obtained by fitting the kinetics of recovery from inactivation with a single-exponential function (number of observations indicated).

(C) Voltages of half-maximal inactivation (squares) and half-maximal activation (circles) and corresponding slope factors (kinact and kact, respectively; number of observations indicated).

(A–C) Gray symbols and dotted lines: Kv4.1 WT data from Figures 2F, 2G, and 3B; red symbols: variant ternary channel data from Figures 2F, 2G, and 3B that significantly differ from Kv4.1 WT when testing 15 variants, including p.His308Tyr; black symbols: values obtained for variant ternary channels with no difference compared to Kv4.1 WT (including part of the p.Asp115Asn and p.Lys450 data); purple symbols: values obtained for variant ternary channels (maternally inherited missense variants) that significantly differ from Kv4.1 WT; number of observations indicated; all statistics based on one way ANOVA with Dunnett’s post-hoc testing; significant differences compared to Kv4.1 WT are indicated with p < 0.05 or ∗∗p < 0.0001.