Fig. 1
From: Possible mechanisms for sensorineural hearing loss and deafness in patients with propionic acidemia
a. Propionic acid effects on IKs current in KCNQ1/KCNE1-CHO cells. Representative whole cell current traces at baseline, after the application of PA (5 mM, blue), and after brief wash-out (grey), demonstrating an acute reduction of IKs by 50%. Voltage clamp protocol is shown in the inset. b. Propionic acid effects on action potential in human iPSC-CMs. Superimposed representative AP traces at baseline and after PA (5 mM, blue), indicating an AP-prolongation