BEST1 and autosomal recessive bestrophinopathy: Remarkably, the endogenous BEST1 protein was diminished with BVSi 3–8 treatment (Figure 4—figure supplement 1d) in the mutant hPSC-RPE cells, concomitant with abolished Ca2+-dependent Cl- currents in these cells at 1.2 μM [Ca2+]i (Figure 5c–d), while co-expression of the wobble WT BEST1-mCherry restored Cl- currents to the WT levels at all tested [Ca2+]is (Figure 5c–f, Figure 4—figure supplement 1d), providing a proof-of-concept for the cure of bestrophinopathies associated with BEST1 gain-of-function mutations.