Downregulation of Cone-Specific Gene Expression and Degeneration of Cone Photoreceptors in the<i>Rpe65</i>^−/−Mouse at Early Ages

Abstract

In the Rpe65(-/-) mouse, the expression of cone-specific genes is downregulated and is accompanied by cone degeneration at early ages. Early administration of 9- or 11-cis retinal can partially prevent cone loss, suggesting that the absence of 11-cis chromophore may be responsible for the early cone degeneration.