Figure 1.
Normal rod function and loss of cone function in CNGB3m/m- and CNGB3−/−-mutant dogs and short-term restoration of cone ERG signals following a single subretinal treatment with rAAV5-PR2.1-hCNGB3. Representative, ERG traces evoked by full-field white flashes under dark-adapted (rod and mixed cone-rod responses) and light-adapted (cone 1 and 29 Hz) conditions are shown. Compared with an age-matched normal wild-type dog, the treated eye of the CNGB3−/− dog (M606; see Table 1) showed restoration of cone function as elicited by single and 29 Hz flicker light flashes 7 weeks after subretinal injection. The smaller amplitude of the restored cone function compared with the normal dog can be explained by the fact that the subretinal bleb covered ∼30% of the entire retina.