Figure 5:
Inhibition of FHL2 rescues enhanced Iks in TTN-T32756I iPSC-aCMs. (A) Co-immunoprecipitation revealed increased interaction between FHL2 and KCNQ1-KCNE1 (MinK) complex. (IP: KCNE1). Immunoblotting (IB) was performed with antibodies against FHL2 (32 kDa) and MinK (32 kDa) (n = 3) (B) qPCR data showing the inhibition of FHL2 gene by the siRNA in the WT and TTN-T32756I iPSC-aCMs (n=7). (C) I-V curves showing the rescue of the Iks TTN-T32756I iPSC-aCMs by the suppression of FHL2 (n=4–8). (D) Comparison of Iks current density at 50 mV (mean ± SEM). I Schematic showing the TTN-T32756I results in increased FHL2 binding with the KNCQ1-KCNE1 complex and enhanced Iks activity. *P<0.05, **P<0.01, ***P<0.001.