Figure 1.
Pulmonary arterial hypertension (PAH)‐associated mutant KCNK3 channels demonstrate mutation‐specific severity of loss of function, across a broad pH range in COS7 cells. A, Typical voltage clamp recordings of wildtype (WT, top) and V221L (bottom) KCNK3. Sample current traces at pH 6.4 (blue), 7.4 (black), and 10.4 (red) are shown. A voltage ramp (top) was applied, −120 mV to +60 mV over 0.5 s, every 3 s, from a holding potential of −80 mV for all voltage clamp recordings in this study. B, Summary of current density (pA/pF at 60 mV) of cells expressing WT or one of the PAH‐associated mutant KCNK3 channels (n=3–9 cells at pH 6.4; n=6–33 cells at pH 7.4; n=6–32 cells at pH 10.4). Bars show mean±SEM. *P<0.05 at pH 7.4; # P<0.05 at pH 10.4, for the comparison of WT and each KCNK3 mutant channel by 1‐way ANOVA (P<0.05) and post hoc Tukey test.