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1 November 2006 9-cis Retinal Increased in Retina of RPE65 Knockout Mice with Decrease in Coat Pigmentation
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Abstract

The protein RPE65 is essential for the generation of the native chromophore, 11-cis retinal, of visual pigments. However, the Rpe65 knockout (Rpe65−/−) mouse shows a minimal visual response due to the presence of a pigment, isorhodopsin, formed with 9-cis retinal. Isorhodopsin accumulates linearly with prolonged dark-rearing of the animals. The majority of Rpe65−/− mice have an agouti coat color. A tan coat color subset of Rpe65−/− mice was found to have an enhanced visual response as measured by electroretinograms. The enhanced response was found to be due to increased levels of 9-cis retinal and isorhodopsin pigment levels. Animals of both coat colors reared in cyclic light have minimal levels of regenerated pigment and show photoreceptor degeneration. On dark-rearing, pigment accumulates and photoreceptor degeneration is decreased. In the tan Rpe65−/− mice, the level of photoreceptor degeneration is less than in the agouti animals, which have an increased pigment and decreased free opsin level. Therefore, photoreceptor damage correlates with the amount of the apoprotein present, supporting findings that the activity from unregenerated opsin can lead to photoreceptor degeneration.

Jie Fan, Bill X. Wu, Tadeusz Sarna, Baerbel Rohrer, T. Michael Redmond, and Rosalie K. Crouch "9-cis Retinal Increased in Retina of RPE65 Knockout Mice with Decrease in Coat Pigmentation," Photochemistry and Photobiology 82(6), 1461-1467, (1 November 2006). https://doi.org/10.1562/2006-02-02-RA-793
Received: 2 February 2006; Accepted: 1 March 2006; Published: 1 November 2006
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