BEST1 and Best vitelliform macular dystrophy: Here we comprehensively examined six BEST1 dominant disease-causing mutations (A10T, R218H, L234P, A243T, Q293K and D302A) derived from BVMD patients in an interdisciplinary platform, including whole-cell patch clamp with patient-derived iPSC-RPEs and HEK293 cells expressing the mutant channels, immunodetection of endogenous BEST1 in iPSC-RPEs, structural analyses with human homology models, and virus-mediated BEST1 gene supplementation.