Fig. 2: The Ush2adelG/delG model exhibits a late-onset decline in visual function.

A Optomotor response is significantly reduced at P180 in Ush2adelG/delG mice and persists at P500. Shown are mean ± SEM. N values: WT P45: 7; P180: 9; P500: 8. Ush2adelG/delG P45: 6; P180: 13; P500: 12. Statistical significance was determined by a two-tailed unpaired t-test. P values: P180: p = 0.0015 (**), P500: p = 0.0197 (*). B Maximum ERG a-wave and b-wave amplitudes plotted against the log of the eliciting flash intensities. Animals (P200) were flashed from weakest to brightest light intensities. Shown are mean ± SEM. N value: 7 WT mice were tested for each intensity in the measurement of both scotopic a- and b-waves. 5 Ush2adelG/delG mice were tested for each intensity in the measurement of both scotopic a- and b-waves. C–G Full-field ERGs were performed on WT and Ush2adelG/delG mice at the indicated ages. C, D Representative scotopic and photopic ERG waveforms are shown from P360 Ush2adelG/delG and WT mice. E–G Maximum scotopic a-, scotopic b-, and photopic b-wave amplitudes were measured at the indicated ages. Amplitudes are significantly decreased in the P360 and P500 Ush2adelG/delG mice. Shown are mean ± SEM. Significance was determined by a two-tailed unpaired t-test. P values: Scotopic a-wave: P360: p = 0.0001 (***) P500: p = 0.0006 (***). Scotopic b-wave: P360: p = 0.0001 (***) P500: p < 0.00001 (****). Photopic b-wave: P360: p = 0.0079 (**) P500: p = 0.0292 (*). N values: Scotopic a-wave: WT P30: 18; P180: 11; P270: 9; P360: 17; P500: 5. Ush2adelG/delG P30: 15; P180: 17; P270: 9; P360: 13; P500:12. Scotopic b-wave: WT P30: 18; P180: 11; P270: 9; P360: 17; P500:5. Ush2adelG/delG P30: 15; P180: 17; P270: 9; P360: 12; P500:12. Photopic b-wave: WT P30: 18; P180: 10; P270: 9; P360: 17; P500: 5. Ush2adelG/delG P30: 13; P180: 17; P270: 9; P360: 12; P500:12.