Skip to main content

Advertisement

Log in

Functional rescue of cone photoreceptors in retinitis pigmentosa

  • Club Jules Gonin
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9

References

  1. Bessant DA, Ali RR, Bhattacharya SS (2001) Molecular genetics and prospects for therapy of the inherited retinal dystrophies. Curr Opin Genet Dev 11:307–316

    Article  PubMed  CAS  Google Scholar 

  2. Berson EL (1993) Retinitis pigmentosa. The friedenwald lecture. Investig Ophthalmol Vis Sci 34:1659–1676

    CAS  Google Scholar 

  3. Sahel and Roska (in press) Gene therapy for blindness. Annu Rev Neurosci

  4. Geller AM, Sieving PA (1993) Assessment of foveal cone photoreceptors in Stargardt’s macular dystrophy using a small dot detection task. Vis Res 33:1509–1524

    Article  PubMed  CAS  Google Scholar 

  5. Mohand-Said S, Hicks D, Dreyfus H, Sahel JA (2000) Selective transplantation of rods delays cone loss in a retinitis pigmentosa model. Arch Ophthalmol 118:807–811

    PubMed  CAS  Google Scholar 

  6. Mohand-Said S, Deudon-Combe A, Hicks D, Simonutti M, Forster V, Fintz AC, Leveillard T, Dreyfus H, Sahel JA (1998) Normal retina releases a diffusible factor stimulating cone survival in the retinal degeneration mouse. Proc Natl Acad Sci U S A 95:8357–8362

    Article  PubMed  CAS  Google Scholar 

  7. Mohand-Said S, Hicks D, Simonutti M, Tran-Minh D, Deudon-Combe A, Dreyfus H, Silverman MS, Ogilvie JM, Tenkova T, Sahel J (1997) Photoreceptor transplants increase host cone survival in the retinal degeneration (rd) mouse. Ophthalmic Res 29:290–297

    Article  PubMed  CAS  Google Scholar 

  8. Leveillard T, Mohand-Said S, Lorentz O, Hicks D, Fintz AC, Clerin E, Simonutti M, Forster V, Cavusoglu N, Chalmel F, Dolle P, Poch O, Lambrou G, Sahel JA (2004) Identification and characterization of rod-derived cone viability factor. Nat Genet 36:755–759

    Article  PubMed  CAS  Google Scholar 

  9. Chalmel F, Leveillard T, Jaillard C, Lardenois A, Berdugo N, Morel E, Koehl P, Lambrou G, Holmgren A, Sahel JA, Poch O (2007) Rod-derived cone viability factor-2 is a novel bifunctional-thioredoxin-like protein with therapeutic potential. BMC Mol Biol 8:74

    Article  PubMed  Google Scholar 

  10. Yang Y, Mohand-Said S, Danan A, Simonutti M, Fontaine V, Clerin E, Picaud S, Leveillard T, Sahel JA (2009) Functional cone rescue by RdCVF protein in a dominant model of retinitis pigmentosa. Mol Ther 17:787–795

    Article  PubMed  CAS  Google Scholar 

  11. Leveillard T, Sahel JA (2010) Rod-derived cone viability factor for treating blinding diseases: from clinic to redox signaling. Sci Transl Med 2:26ps16

    Article  PubMed  Google Scholar 

  12. Cingolani C, Rogers B, Lu L, Kachi S, Shen J, Campochiaro PA (2006) Retinal degeneration from oxidative damage. Free Radic Biol Med 40:660–669

    Article  PubMed  CAS  Google Scholar 

  13. Komeima K, Rogers BS, Lu L, Campochiaro PA (2006) Antioxidants reduce cone cell death in a model of retinitis pigmentosa. Proc Natl Acad Sci U S A 103:11300–11305

    Article  PubMed  CAS  Google Scholar 

  14. Fridlich R, Delalande F, Jaillard C, Lu J, Poidevin L, Cronin T, Perrocheau L, Millet-Puel G, Niepon ML, Poch O, Holmgren A, Van Dorsselaer A, Sahel JA, Leveillard T (2009) The thioredoxin-like protein rod-derived cone viability factor (RdCVFL) interacts with TAU and inhibits its phosphorylation in the retina. Mol Cell Proteomics 8:1206–1218

    Article  PubMed  CAS  Google Scholar 

  15. Cronin T, Raffelsberger W, Lee-Rivera I, Jaillard C, Niepon ML, Kinzel B, Clerin E, Petrosian A, Picaud S, Poch O, Sahel JA, Leveillard T (2010) The disruption of the rod-derived cone viability gene leads to photoreceptor dysfunction and susceptibility to oxidative stress. Cell Death Differ 17:1199–1210

    Article  PubMed  CAS  Google Scholar 

  16. Jaillard C, Mouret A, Niepon ML, Clerin E, Yang Y, Lee-Rivera I, Ait-Ali N, Millet-Puel G, Cronin T, Sedmak T, Raffelsberger W, Kinzel B, Trembleau A, Poch O, Bennett J, Wolfrum U, Lledo PM, Sahel JA, Leveillard T (2012) Nxnl2 splicing results in dual functions in neuronal cell survival and maintenance of cell integrity. Hum Mol Genet 21:2298–2311

    Article  PubMed  CAS  Google Scholar 

  17. Byrne LC, Clérin E, Lee-Rivera I, Luna G, Fisher SK, Bennett J, Sahel JA, Flannery JG and Leveillard TD (2011) AAV-mediated Delivery of Rod-derived Cone Viability Factor in a Mouse Model of Retinal Degeneration. ARVO ABSTRACT 1395/A544

  18. Milam AH, Li ZY, Fariss RN (1998) Histopathology of the human retina in retinitis pigmentosa. Prog Retin Eye Res 17:175–205

    Article  PubMed  CAS  Google Scholar 

  19. Li ZY, Kljavin IJ, Milam AH (1995) Rod photoreceptor neurite sprouting in retinitis pigmentosa. J Neurosci 15:5429–5438

    PubMed  CAS  Google Scholar 

  20. Lin B, Masland RH, Strettoi E (2009) Remodeling of cone photoreceptor cells after rod degeneration in rd mice. Exp Eye Res 88:589–599

    Article  PubMed  CAS  Google Scholar 

  21. Nagel G, Ollig D, Fuhrmann M, Kateriya S, Musti AM, Bamberg E, Hegemann P (2002) Channelrhodopsin-1: a light-gated proton channel in green algae. Science 296:2395–2398

    Article  PubMed  CAS  Google Scholar 

  22. Boyden ES, Zhang F, Bamberg E, Nagel G, Deisseroth K (2005) Millisecond-timescale, genetically targeted optical control of neural activity. Nat Neurosci 8:1263–1268

    Article  PubMed  CAS  Google Scholar 

  23. Zhang F, Wang LP, Brauner M, Liewald JF, Kay K, Watzke N, Wood PG, Bamberg E, Nagel G, Gottschalk A, Deisseroth K (2007) Multimodal fast optical interrogation of neural circuitry. Nature 446:633–639

    Article  PubMed  CAS  Google Scholar 

  24. Bi A, Cui J, Ma YP, Olshevskaya E, Pu M, Dizhoor AM, Pan ZH (2006) Ectopic expression of a microbial-type rhodopsin restores visual responses in mice with photoreceptor degeneration. Neuron 50:23–33

    Article  PubMed  CAS  Google Scholar 

  25. Lagali PS, Balya D, Awatramani GB, Munch TA, Kim DS, Busskamp V, Cepko CL, Roska B (2008) Light-activated channels targeted to ON bipolar cells restore visual function in retinal degeneration. Nat Neurosci 11:667–675

    Article  PubMed  CAS  Google Scholar 

  26. Masland RH (2001) The fundamental plan of the retina. Nat Neurosci 4:877–886

    Article  PubMed  CAS  Google Scholar 

  27. Wassle H (2004) Parallel processing in the mammalian retina. Nat Rev Neurosci 5:747–757

    Article  PubMed  Google Scholar 

  28. Busskamp V, Picaud S, Sahel JA, Roska B (2011) Optogenetic therapy for retinitis pigmentosa. Gene Ther 19:169–175

    Article  PubMed  Google Scholar 

  29. Busskamp V, Duebel J, Balya D, Fradot M, Viney TJ, Siegert S, Groner AC, Cabuy E, Forster V, Seeliger M, Biel M, Humphries P, Paques M, Mohand-Said S, Trono D, Deisseroth K, Sahel JA, Picaud S, Roska B (2010) Genetic reactivation of cone photoreceptors restores visual responses in retinitis pigmentosa. Science 329:413–417

    Article  PubMed  CAS  Google Scholar 

  30. Fradot M, Busskamp V, Forster V, Cronin T, Leveillard T, Bennett J, Sahel JA, Roska B, Picaud S (2011) Gene therapy in ophthalmology: validation on cultured retinal cells and explants from postmortem human eyes. Hum Gene Ther 22:587–593

    Article  PubMed  CAS  Google Scholar 

Download references

Disclosure

J-A Sahel is a patent co-inventor of RdCVF and consultant for Sanofi-Fovea

Funding

NOVARTIS Pharma, Sanofi-Fovea, INSERM, Ministère de la Recherche, European 5th, 6th and 7th Framework Program, GIP AVENTIS, Agence Nationale de la Recherche, A.F.M. Téléthon, RETINA France, Fédération des Aveugles de France, Fondation pour la Recherche Medicale, Bettencourt-Schuller Award, Alcon Research Institute Award, Foundation Fighting Blindness/NNRI ,NRJ Neuroscience Award, Association Française contre les Myopathies, LABEX LIFESENSES

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to José-Alain Sahel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sahel, JA., Léveillard, T., Picaud, S. et al. Functional rescue of cone photoreceptors in retinitis pigmentosa. Graefes Arch Clin Exp Ophthalmol 251, 1669–1677 (2013). https://doi.org/10.1007/s00417-013-2314-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00417-013-2314-7

Keywords

Navigation