Skip to main content
. Author manuscript; available in PMC: 2024 Jul 1.
Published in final edited form as: J Physiol. 2023 Feb 20;601(13):2711–2731. doi: 10.1113/JP283976

Figure 4. Simulation of the trafficking-deficient Kv11.1-p.(A57P) mutation associated with long-QT syndrome type 2.

Figure 4.

A, Relative changes in membrane stability (top panel) and forward trafficking (bottom panel) over time induced by the Kv11.1-p.(A57P) mutation (red circles, Exp. MT) compared to wild-type Kv11.1 (black squares, Exp. WT) in experimental recordings (Kanner et al., 2018). B, Similar to panel A for simulations with the default parameters representing wild-type Kv11.1 (black line, model WT) and 4 different parameter sets obtained through optimization on the relative differences between wild-type and Kv11.1-p.(A57P) after a perturbation in each of the four rates. C, The number of membrane channels for the wild-type model and each of the 4 parameter sets that was optimized to mimic the Kv11.1-p.(A57P) behaviour. D, Action-potential duration of the human ventricular cardiomyocyte model incorporating the Kv11.1 trafficking component during steady-state pacing at a basic cycle of 60s for the wild-type parameters (black) and each of the 4 parameter sets corresponding to Kv11.1-A57P.