BEST1 and Best vitelliform macular dystrophy: Mutations in BEST1 gene have been described in a variety of ocular disease phenotypes including autosomal recessive bestrophinopathy (ARB, MIM 611809)1,2, Best vitelliform macular dystrophy (MIM 153700)3,4, autosomal dominant vitreoretinochoroidopathy (MIM 193220)5,6, autosomal dominant microcornea, rod-cone dystrophy, early-onset cataract posterior staphyloma syndrome and retinitis pigmentosa (MIM 613194)7,8.