Figure 3: Opposing shifts in channel activation in TS channels.
From: Arrhythmogenesis in Timothy Syndrome is associated with defects in Ca2+-dependent inactivation
(a) Exemplar on-cell current recordings in response to a voltage ramp (top). Channel openings are fit by the GHK equation (grey dashed line). Scale bar, 1 pA. (b) Addition of Bay K 8644 increases mode 2 openings, verifying channel number and GHK fit. (c) Current averaged over multiple patches gives the current voltage relation (black). The grey dashed relation is now the GHK relation scaled down by PO/max. Scale bar, 0.2 pA. (d) PO as a function of voltage, averaged across cells (red) with Boltzmann fit (black). Grey displays ±s.e.m. G402S (middle) is clearly right shifted (case 1) compared with WT displayed as dashed line for comparison. G406R (right) shifts left (case 2) as compared with WT and G402S (dashed curves). n=13, 8 and 7 for WT, G402S and G406R, respectively.
