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Isomerase activity of the missense mutation RPE65/D477G. (A) HPLC of retinoids from cell lysates incubated with all-trans retinol (peak 3), showing presence of 11-cis retinol (peak 1) and 13-cis retinol (peak 2). Inset shows representative chromatography for cells transfected with either canine WT RPE65, RPE65/D477G, or a mixture of WT and RPE65/D477G. (B) Comparison of total 11-cis retinol extracted from transfected cells (mean and SEM; n=4).
