Retinal Disease in Rpe 65-Deficient Mice : Comparison to Human Leber Congenital Amaurosis Due to RPE 65 Mutations

METHODS. Full-field electroretinograms (ERGs) were recorded from wild-type (C57BL/6; Rpe65 / ) and Rpe65 / mice at ages ranging from 1 month to 2 years. A physiologically based model of rod phototransduction activation was used to determine photoreceptor (P3) cell components of ERG photoresponses. A bipolar (P2) cell component was also derived. Photoreceptor and inner retinal thickness measurements were made by using optical coherence tomography in human RPE65-LCA.

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