Improvement in Rod and Cone Function in Mouse Model of Fundus albipunctatus after Pharmacologic Treatment with 9-cis-Retinal.
PURPOSE: To assess changes in rod and cone visual functions in a mouse model of Fundus albipunctatus with disrupted 11-cis-retinol dehydrogenase ( RDH) genes after pharmacologic treatment with an artificial retinal chromophore. METHODS: Retinoid levels and photoreceptor functions of Rdh5(-/-)Rdh11(-/-) mice at a variety of light intensities were analyzed with normal-phase HPLC and ERG techniques. Production of 11-cis-retinal, the visual pigment chromophore, was suppressed with a potent inhibitor of the retinoid cycle, all-trans-retinylamine (Ret-NH(2)). The chromophore was replaced by a functional geometric isomer, 9-cis-retinal, delivered by oral gavage. RESULTS: Aberrant cone responses were detected in 12-month-old Rdh5(-/-)Rdh11(-/-) mice raised in a 12-hour light/12-hour dark cycle. This cone defect was exacerbated in conditions of low levels of 11-cis-retinal. Administration of 9-cis-retinal increased the rate of dark adaptation and improved cone function in Rdh5(-/-)Rdh11(-/-) mice. CONCLUSIONS: Disruption of 11-cis-RDHs causes a slowly developing cone dystrophy caused by inefficient cone pigment regeneration. Rod and cone visual function improved significantly in the mouse model of F. albipunctatus after treatment with 9-cis-retinal, suggesting a potential approach to slow the progression of cone dystrophy in affected humans.[1]References
- Improvement in Rod and Cone Function in Mouse Model of Fundus albipunctatus after Pharmacologic Treatment with 9-cis-Retinal. Maeda, A., Maeda, T., Palczewski, K. Invest. Ophthalmol. Vis. Sci. (2006) [Pubmed]
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