Supplementary Figure 1: Activation, deactivation and Inactivation of wild-type and mutant KCNH1 channels expressed in Xenopus oocytes. | Nature Genetics

Supplementary Figure 1: Activation, deactivation and Inactivation of wild-type and mutant KCNH1 channels expressed in Xenopus oocytes.

From: Mutations in the voltage-gated potassium channel gene KCNH1 cause Temple-Baraitser syndrome and epilepsy

Supplementary Figure 1

(a) Representative families of whole-cell currents showing voltage-dependent activation of wild-type (WT) and mutant human KCNH1 channels expressed in Xenopus oocytes. Currents were elicited by steps from –120 mV to +80 mV in 20-mV increments, using a holding potential of –100 mV. Inset in the final panel: schematic of the pulse protocol used for the activation experiments. (b) Representative families of whole-cell currents showing only minor steady-state inactivation of WT and mutant KCNH1 channels expressed in Xenopus oocytes. Inset in the final panel: schematic of the pulse protocol used for the inactivation experiments. (c) Activation current-voltage relationship (± s.e.m.) for WT, p.Lys217Asn, p.Leu489Phe, p.Ile494Val and p.Gln503Arg channels expressed in Xenopus oocytes. Currents were measured at the end of the activation test pulse. (d) Fast and slow time constants (τ) of deactivation for WT and mutant KCNH1 channels expressed in Xenopus oocytes, analyzed from the tail current at –90 mV following a maximally activating prepulse to +80 mV. (e) Steady-state inactivation current-voltage relationship (± s.e.m.) for WT and mutant KCNH1 channels expressed in Xenopus oocytes. Currents were measured at the end of the +30 mV activation test pulse. Data are presented as mean ± s.e.m. with the numbers of experiments indicated in parentheses. P values were calculated in comparison to WT using an unpaired t test with Welch’s correction. *P < 0.05, **P < 0.01, ***P < 0.001.

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