Mutations in BEST1 therefore affect RPE metabolism and cause a collection of bestrophinopathies, such as Best disease [9], adult-onset Best vitelliform macular dystrophy [10], ARB [11], Retinitis pigmentosa 50/concentric retinitis pigmentosa [12], autosomal dominant vitreoretinochoroidopathy (ADVIRC) [13] and microcornea, rod-cone dystrophy, cataract, and posterior staphyloma syndrome (MRCS) [14]. This evidence concerns the gene BEST1 and autosomal recessive bestrophinopathy.