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http://purl.uniprot.org/citations/17003450http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/17003450http://www.w3.org/2000/01/rdf-schema#comment"

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-NH2). 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."xsd:string
http://purl.uniprot.org/citations/17003450http://purl.org/dc/terms/identifier"doi:10.1167/iovs.06-0215"xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/author"Maeda A."xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/author"Maeda T."xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/author"Palczewski K."xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/date"2006"xsd:gYear
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/name"Invest Ophthalmol Vis Sci"xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/pages"4540-4546"xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/title"Improvement in rod and cone function in mouse model of Fundus albipunctatus after pharmacologic treatment with 9-cis-retinal."xsd:string
http://purl.uniprot.org/citations/17003450http://purl.uniprot.org/core/volume"47"xsd:string
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