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. 2021 Jul 28;53(7):1192–1204. doi: 10.1038/s12276-021-00653-4

Fig. 6. Schematic illustration of different pathophysiological mechanisms of KCNQ4 variants on DFNA2.

Fig. 6

a Loss-of-function caused by KCNQ4 p.R331Q and its restoration following phospholipid phosphatidylinositol 4,5-bisphosphate (PIP2) activator (i.e., PIP5K) and channel openers. b Gain of function caused by KCNQ4 p.G319D and its restoration following interference of PIP2 activation by intracellular application of a polycation poly-L-lysine (PIP2 inhibitor) and channel inhibitor.