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Article

In conditions of limited chromophore supply rods entrap 11-cis-retinal leading to loss of cone function and cell death.

Human molecular genetics - 1 Apr 2009

Samardzija Marijana, Tanimoto Naoyuki, Kostic Corinne, Beck Susanne, Oberhauser Vitus, Joly Sandrine, Thiersch Markus, Fahl Edda, Arsenijevic Yvan, von Lintig Johannes, Wenzel Andreas, Seeliger Mathias W, Grimm Christian

Abstract excerpt

RPE65 is a retinoid isomerase required for the production of 11-cis-retinal, the chromophore of both cone and rod visual pigments. We recently established an R91W knock-in mouse strain as homologous animal model for patients afflicted by this mutation in RPE65. These mice have impaired vision and can only synthesize minute amounts of 11-cis-retinal. Here, we investigated the consequences of this chromophore...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 19147682
DOI: 10.1093/hmg/ddp026

Genes

RPE65

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Related research

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Click a neighbor to travelStep 1 · 12 closest

In conditions of limited chromophore supply rods entrap 11-cis-retinal leading to loss of cone function and cell death.PMID 19147682Read ↗

R91W mutation in Rpe65 leads to milder early-onset retinal dystrophy due to the generation of low levels of 11-cis-retinalSemantic proximity Read ↗

<i>RPE65</i> knockout <i>Xenopus laevis</i> have a compromised but detectable electroretinogram and altered visual responses, without retinal degeneration or altered melanophore dispersionSemantic proximity Read ↗

Nrl-knockout mice deficient in Rpe65 fail to synthesize 11-cis retinal and cone outer segments.Semantic proximity Read ↗

Pathways and disease-causing alterations in visual chromophore production for vertebrate visionSemantic proximity Read ↗

Retinal cone and rod photoreceptor cells exhibit differential susceptibility to light‐induced damageSemantic proximity Read ↗

Explant cultures of Rpe65-/- mouse retina: a model to investigate cone opsin trafficking.Semantic proximity Read ↗

Rpe65 is necessary for production of 11-cis-vitamin A in the retinal visual cycle.Semantic proximity Read ↗

Cone Opsins and Inherited Retinal Disease.Semantic proximity Read ↗

An S-opsin knock-in mouse (F81Y) reveals a role for the native ligand 11-cis-retinal in cone opsin biosynthesis.Semantic proximity Read ↗

Human cone photoreceptor dependence on RPE65 isomeraseSemantic proximity Read ↗

A Dominant Mutation in Rpe65, D477G, Delays Dark Adaptation and Disturbs the Visual Cycle in the Mutant Knock-In Mice.Semantic proximity Read ↗

RPE Visual Cycle and Biochemical Phenotypes of Mutant Mouse Models.Semantic proximity Read ↗

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