Figure 4.

Long-Term Preservation of Retinal Function after Gene Therapy with AAV2/5-GRK1-hRPGRstb in Dogs Treated at Mid-stage Disease

(A) Representative ERG traces of rod (−1.74 log cd.s.m⁻²), mixed rod-cone (1.01 log cd.s.m⁻²) recorded dark adapted, and cone (1.01 log cd.s.m⁻²) responses to single stimuli or 29-Hz cone flicker (0.76 log cd.s.m⁻²) recorded light adapted at 103 weeks of age in an RPGR mutant dog treated at 12 weeks of age with 150 μL of a low viral titer (1.51 × 10¹¹ vg/mL). (B) Mean (± SD) of all rod and cone ERG results recorded at 103 weeks of age from three RPGR mutant dogs treated at mid-stage disease with 150 μL of a low viral titer (1.51 × 10¹¹ vg/mL). (C) Representative ERG traces in an RPGR mutant dog treated at 12 weeks of age with 150 μL of a high viral titer (1.51 × 10¹² vg/mL). (D) Mean (± SD) of all rod and cone ERG results recorded at 103 weeks of age from three RPGR mutant dogs treated at mid-stage disease with 150 μL of a high viral titer (1.51 × 10¹² vg/mL). Tx, treated; Ctrl, contralateral BSS injected; ^♦p ≤ 0.07; *p < 0.05; **p < 0.001 from paired t test between treated and contralateral eyes.