Synthetic 9-cis-beta-carotene inhibits photoreceptor degeneration in cultures of eye cups from rpe65rd12 mouse model of retinoid cycle defect
| Autor: | Sara Peri-Chen, Adi Tzameret, Ifat Sher, Ehud Gazit, Ygal Rotenstreich, Ludmila Buzhansky, Alon Sayer, Michael Ioffe, Victoria Edelshtain |
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| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
genetic structures medicine.drug_class lcsh:Medicine Chemistry Techniques Synthetic Biology Article 03 medical and health sciences chemistry.chemical_compound Mice 0302 clinical medicine Retinal Diseases beta-Carotene Retinitis pigmentosa medicine Animals Retinoid lcsh:Science Gene Cells Cultured chemistry.chemical_classification Multidisciplinary Provitamins Retinal Degeneration lcsh:R Dystrophy Retinal medicine.disease beta Carotene eye diseases Cell biology Disease Models Animal 030104 developmental biology Enzyme chemistry 030221 ophthalmology & optometry lcsh:Q sense organs Retinal Dystrophies Retinitis Pigmentosa Photoreceptor Cells Vertebrate |
| Zdroj: | Scientific Reports, Vol 8, Iss 1, Pp 1-9 (2018) Scientific Reports |
| ISSN: | 2045-2322 |
| Popis: | The retinoid cycle enzymes regenerate the visual chromophore 11-cis retinal to enable vision. Mutations in the genes encoding the proteins of the retinoid cycle are the leading cause for recessively inherited retinal dystrophies such as retinitis pigmentosa, Leber congenital amaurosis, congenital cone-rod dystrophy and fundus albipunctatus. Currently there is no treatment for these blinding diseases. In previous studies we demonstrated that oral treatment with the 9-cis-β-carotene rich Dunaliella Bardawil algae powder significantly improved visual and retinal functions in patients with retinitis pigmentosa and fundus albipunctatus. Here we developed a convenient and economical synthetic route for biologically active 9-cis-β-carotene from inexpensive building materials and demonstrated that the molecule is stable for at least one month. Synthetic 9-cis-β-carotene rescued cone photoreceptors from degeneration in eye cup cultures of mice with a retinoid cycle genetic defect. This study suggests that synthetic 9-cis-β-carotene may serve as an effective treatment for retinal dystrophies involving the retinoid cycle. |
| Databáze: | OpenAIRE |
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