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.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cells, Cultured
  • Chemistry Techniques, Synthetic / methods
  • Disease Models, Animal
  • Mice
  • Photoreceptor Cells, Vertebrate / drug effects*
  • Provitamins / chemical synthesis
  • Provitamins / chemistry
  • Provitamins / pharmacology*
  • Retinal Degeneration / drug therapy
  • Retinal Diseases / drug therapy
  • Retinitis Pigmentosa / drug therapy
  • beta Carotene / chemical synthesis
  • beta Carotene / chemistry
  • beta Carotene / pharmacology*

Substances

  • Provitamins
  • beta Carotene

Supplementary concepts

  • Fundus Albipunctatus