Most BEST1 mutations associated with Best’s disease are missense mutations [54], but another bestrophinopathy is late onset, referred to as adult-onset vitelliform macular dystrophy (AVMD; OMIM: 608161), and in addition to mutations in BEST1 that lead to AVMD there are known mutations in three other genes associated with photoreceptors. Here, BEST1 is linked to Best vitelliform macular dystrophy.