Currents were recorded from cells using an internal (pipette) solution containing a [Cl−] of 5 mm. From a holding potential of 0 mV, currents were elicited in 20 mV intervals over the voltage range −145 to +95 mV followed by a fixed pulse of −105 mV. A, a representative current family from a cell expressing WT hClC-1 channels. B, the averaged current traces from six cells expressing Q552R hClC-1 channels. C, voltage dependence of the instantaneous current amplitude for Q552R hClC-1-expressing cells at high (▪, n = 7) and low (□, n = 9) [Cl−]i. D, voltage dependence of the relative Po for WT (▵, n = 8) and Q552R (□, n = 9) at a [Cl−]i of 5 mm. The continuous lines represent fits of a Boltzmann distribution.