Finally, mutations in the gene BEST1 are associated with five clinically distinct retinal degenerative diseases, which are referred to as "bestrophinopathies", and are: Best vitelliform macular dystrophy, autosomal recessive bestrophinopathy, adult-onset vitelliform macular dystrophy, autosomal dominant vitreoretinochoroidopathy, and retinitis pigmentosa [122]. This evidence concerns the gene BEST1 and autosomal recessive bestrophinopathy.