2. Academic Validation
  3. Synthetic 9-cis-beta-carotene inhibits photoreceptor degeneration in cultures of eye cups from rpe65rd12 mouse model of retinoid cycle defect

Synthetic 9-cis-beta-carotene inhibits photoreceptor degeneration in cultures of eye cups from rpe65rd12 mouse model of retinoid cycle defect

  • Sci Rep. 2018 Apr 17;8(1):6130. doi: 10.1038/s41598-018-24439-3.
Ifat Sher 1,  Adi Tzameret 1 2,  Sara Peri-Chen 1,  Victoria Edelshtain 1 2,  Michael Ioffe 3,  Alon Sayer 3,  Ludmila Buzhansky 3 4,  Ehud Gazit 3 5 4,  Ygal Rotenstreich 6 7
Affiliations

Affiliations

  • 1 Goldschleger Eye Institute, Sheba Medical Center, Tel-Hashomer, Israel.
  • 2 Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
  • 3 Department of Molecular Microbiology and Biotechnology, George S. Wise Faculty of Life Sciences, Tel Aviv University, Tel Aviv, Israel.
  • 4 BLAVATNIK CENTER for Drug Discovery, Tel Aviv University, Tel Aviv, Israel.
  • 5 Department of Materials Science and Engineering, Iby and Aladar Fleischman Faculty of Engineering, Tel Aviv University, Tel Aviv, Israel.
  • 6 Goldschleger Eye Institute, Sheba Medical Center, Tel-Hashomer, Israel. Ygal.Rotenstreich@sheba.health.gov.il.
  • 7 Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Ygal.Rotenstreich@sheba.health.gov.il.
PMID: 29666392 DOI: 10.1038/s41598-018-24439-3
Abstract

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.

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