Electroretinographic study of the C57BL/6-mivit/mivit mouse model of retinal degeneration.

PURPOSE

The C57BL/6-mivit/mivit mouse model of retinal degeneration is characterized by slow progressive loss of photoreceptor cells, concomitant loss of rhodopsin, and uneven pigmentation of the retinal pigment epithelium. The purpose of this study was to determine how these alterations affected the electroretinogram (ERG).

METHODS

Scotopic ERGs were measured in two litters of mivit/mivit mice beginning at 4 weeks and continued in the same animals at 2-week intervals through 18 weeks.

RESULTS

The mean of the maximum b-wave amplitude (Vmax) at 4 weeks was 234 +/- 14 microV in mivit/mivit mice, which did not differ significantly from controls (266 +/- 26 microV). With increasing age, all components of the ERG decreased and by 12 weeks, the mean of the Vmax had decreased to 170 microV. At 18 weeks, the mean Vmax was 75 microV, and the b- to a-wave ratio was still > 1.0. Comparison of these physiologic data to previously reported morphologic and biochemical data showed a high correlation between the b-wave amplitude and the number of photoreceptor cell nuclei (r = 0.9772) as well as the b-wave amplitude and rhodopsin levels (r = 0.9019).

CONCLUSIONS

The loss of all components of the ERG and the lack of a negative-type ERG suggested that the primary cells altered in the mivit/mivit mouse were the photoreceptors. The high correlations between the ERG amplitude and the number of photoreceptor nuclei indicate that the Vmax of the ERG is a good measure of the degree of photoreceptor loss.