Rescue KCNQ4 Surface Expression in Cells Mimicking the Heterozygous Condition of DFNA2 Patients.

<p><i>A. & B. Representative Western blot</i> HEK293T cells were transfected with WT and a mutant KCNQ4 channel at a ratio 1:1 plus various amount of HSP90β as indicated. 24 hrs post transfection, KCNQ4 channels on cell surface were labeled with a mouse monoclonal antibody against the HA tag in the first extracellular loop of KCNQ4 channels prior to cell lysis; then isolated using Dynabeads Protein G and analyzed by Western blot. <i>C. & D. Summary data</i> Each data point in the bar graphs represents the mean ±SD of three experiments (* p≤0.05, ** p≤0.01). Surface expression in cells co-expressing WT/L274H or WT/W276S could be restored to the level of WT KCNQ4 by over-expression of HSP90β.