Therapeutic strategy for handling inherited retinal degenerations in a gene-independent manner using rod-derived cone viability factors

Thierry Léveillard, Ram Fridlich, Emmanuelle Clérin, Najate Aït-Ali, Géraldine Millet-Puel, Céline Jaillard, Ying Yang, Donald Zack, Alain Van-Dorsselaer, José Alain Sahel

Research output: Contribution to journalShort surveypeer-review

8 Scopus citations

Abstract

The most common hereditary retinal degeneration, retinitis pigmentosa (RP), leads to blindness by degeneration of cone photoreceptors. Meanwhile, genetic studies have shown that a significant proportion of RP genes is expressed only by rods, which raises the question of the mechanism leading to the degeneration of cones. Following the concept of sustainability factor cones, rods secrete survival factors that are necessary to maintain the cones, named Rod-derived Cone Viability Factors (RdCVFs). In patients suffering from RP, loss of rods results in the loss of RdCVFs expression and followed by cone degeneration. We have identified the bifunctional genes nucleoredoxin-like 1 and 2 that encode for, by differential splicing, a thioredoxin enzyme and a cone survival factor, respectively RdCVF and RdCVF2. The administration of these survival factors would maintain cones and central vision in most patients suffering from RP.

Original languageEnglish (US)
Pages (from-to)207-213
Number of pages7
JournalComptes Rendus - Biologies
Volume337
Issue number3
DOIs
StatePublished - Mar 2014

Keywords

  • Cone photoreceptors
  • Nucleoredoxin-like
  • Redox signalling
  • TAU
  • Thioredoxin
  • Vision

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Microbiology(all)
  • Agricultural and Biological Sciences(all)

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