Fig. 7

Plot of regression equation of Best Disease Severity Index (BDSI) versus modulus of relative energy of dimerization |ΔΔEdim| of variant BEST1 subunits: BDSI = 0.24935 · |ΔΔEdim| + 6.56527, Eq. (6). BDSI was derived from clinically evaluated BCVA (best-corrected visual acuity in logMAR scale) and age of 27 probands, Eq. (5), Table 2; ΔΔEdim was computed by molecular mechanics from the 3D model of human CaCC. Statistical parameters of the correlation: number of available data points: n = 20, squared correlation coefficient: R2 = 0.854, leave-one-out cross-validated correlation coefficient: \({\text{R}}^{ 2}_{\text{xv}}\) = 0.827, Fischer F-test: F = 76.164, significance of correlation: α > 95%, number of outliers removed: no = 5