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- W2171636319 endingPage "512" @default.
- W2171636319 startingPage "469" @default.
- W2171636319 abstract "Absorption of a photon by an opsin pigment causes isomerization of the chromophore from 11-cis-retinaldehyde to all-trans-retinaldehyde. Regeneration of visual chromophore following light exposure is dependent on an enzyme pathway called the retinoid or visual cycle. Our understanding of this pathway has been greatly facilitated by the identification of disease-causing mutations in the genes coding for visual cycle enzymes. Defects in nearly every step of this pathway are responsible for human-inherited retinal dystrophies. These retinal dystrophies can be divided into two etiologic groups. One involves the impaired synthesis of visual chromophore. The second involves accumulation of cytotoxic products derived from all-trans-retinaldehyde. Gene therapy has been successfully used in animal models of these diseases to rescue the function of enzymes involved in chromophore regeneration, restoring vision. Dystrophies resulting from impaired chromophore synthesis can also be treated by supplementation with a chromophore analog. Dystrophies resulting from the accumulation of toxic pigments can be treated pharmacologically by inhibiting the visual cycle, or limiting the supply of vitamin A to the eyes. Recent progress in both areas provides hope that multiple inherited retinal diseases will soon be treated by pharmaceutical intervention." @default.
- W2171636319 created "2016-06-24" @default.
- W2171636319 creator A5033350423 @default.
- W2171636319 creator A5044688323 @default.
- W2171636319 creator A5044954870 @default.
- W2171636319 creator A5045657649 @default.
- W2171636319 date "2007-02-01" @default.
- W2171636319 modified "2023-10-09" @default.
- W2171636319 title "Diseases Caused by Defects in the Visual Cycle: Retinoids as Potential Therapeutic Agents" @default.
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