Figure 1.
S versus L/M cone function and disease progression in patients with NR2E3 R311Q mutations. (A) Psychophysically measured S and L/M cone sensitivities and their ratio summarized over three annular regions corresponding to eccentricities of 3–10°, 10–34°, and >34°, respectively (I, II, III shown in Inset). Patient data (thin black bars) are ordered (left to right) in declining S cone sensitivity in region I. S to L/M cone sensitivity ratios are normalized by the mean normal ratio in each region. Data from the eye donor at age 70 years are shown as white. Normal data are gray except in the bottom row, where mean normal is 0 log. Error bars, 2 SEM. (B) Serial data over 11 years in a patient between the ages of 40 and 51 showing S and L/M cone sensitivities in regions I and II. (C) Serial dark-adapted ERG a-wave amplitudes in the same patient. Insets (ordinate range 100 μV; abscissa −10 to 160 ms) show two representative waveforms.