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Literature summary for 2.7.11.14 extracted from
- Trafficking of membrane-associated proteins to cone photoreceptor outer segments requires the chromophore 11-cis-retinal (2008), J. Neurosci., 28, 4008-4014.
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | Mus musculus | mutant Lrat-/- and Rpe65-/- mice, models of human liver congenital amaurosis, show disrupted retinoid cycle and no production of 11-cis-retinal, the chromophore of visual pigments. The Lrat-/- and Rpe65-/- retina phenotype presents with rapid sectorial cone degeneration, and the visual pigments, S-opsin and M/L-opsin, fail to traffic to cone outer segments appropriately. In contrast, rod opsin traffics normally in mutant rods. Concomitantly, guanylate cyclase 1, cone Talpha-subunit, cone phosphodiesterase 6alpha', and GRK1 are not transported to Lrat-/- and Rpe65-/- cone outer segments, overview | ? | - |
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Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Mus musculus | - |
mutant Lrat-/- and Rpe65-/- mice, models of human Leber congenital amaurosis | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| eye | - |
Mus musculus | - |
| retina | - |
Mus musculus | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | mutant Lrat-/- and Rpe65-/- mice, models of human liver congenital amaurosis, show disrupted retinoid cycle and no production of 11-cis-retinal, the chromophore of visual pigments. The Lrat-/- and Rpe65-/- retina phenotype presents with rapid sectorial cone degeneration, and the visual pigments, S-opsin and M/L-opsin, fail to traffic to cone outer segments appropriately. In contrast, rod opsin traffics normally in mutant rods. Concomitantly, guanylate cyclase 1, cone Talpha-subunit, cone phosphodiesterase 6alpha', and GRK1 are not transported to Lrat-/- and Rpe65-/- cone outer segments, overview | Mus musculus | ? | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| G-protein-coupled receptor kinase 1 | - |
Mus musculus |
| GRK1 | - |
Mus musculus |
| opsin kinase | - |
Mus musculus |
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Release 2023.1 (January 2023)
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