Lentiviral Gene Transfer-Mediated Cone Vision Restoration in RPE65 Knockout Mice
In photoreceptors, the photo-isomerization of 11-cis-retinal chromophore into alltrans-retinal is the first step of the photo-transduction cascade. To regain light sensitivity after light exposure, photoreceptors need to recycle their chromophore. This is done through the retinoid visual cycle, which involves a series of enzymatic reactions taking place in photoreceptors as well as in the cells of the retinal-pigmented epithelium (RPE) (reviewed by JC Saari (Saari, 2000)). Rpe65, a gene specifically expressed in RPE, has recently been identified as the isomerohydrolase (Jin et al., 2005; Moiseyev et al., 2005), one of the key enzymes of the retinoid visual cycle. As a consequence, when Rpe65 is mutated, the retinoid visual cycle is disrupted, and the sensitivity to light of the photoreceptors is drastically reduced.
KeywordsRetinal Pigment Epithelium Cone Photoreceptor Intraocular Injection Optomotor Response Photopic Condition
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