BEST1 and autosomal recessive bestrophinopathy: On the other hand, gain-of-function mutations such as D203A, I205T, and Y236C represent the ‘strongest’ class, which predominates over the WT BEST1 even at a 1:4 ratio (presumably one protomer per channel, Figure 2a–c and Figure 2—figure supplement 1b), although it remains unclear if they are specifically linked to a certain type of bestrophinopathy.