BEST1 and Best vitelliform macular dystrophy: This is exemplified by the bestrophinopathies, which are linked to variants in the bestrophin-1 (BEST1) gene but are inherited in either an autosomal dominant (as seen in Best vitelliform macular dystrophy and vitreoretinochoroidopathy) or in an autosomal recessive manner (as seen in bestrophinopathy, autosomal recessive)23.