Skip to main content
SpringerLink
Log in

Inherited retinal dystrophy in RCS rats: a deficiency in vitamin A esterification in pigment epithelium

  • Letter
  • Published:

From Nature

View current issue Submit your manuscript

Abstract

The RCS (Royal College of Surgeons) mutant strain of rats1 has often served as a model for the study of inherited retinal degenerations. Lamellar outer segment debris accumulates early in the course of the dystrophy, probably as a consequence of a markedly diminished ability of the retinal pigment epithelium (RPE) to phagocytose photoreceptor disks2–4. The RPE seems to be the primary site of the genetic defect5, and faulty recognition mechanisms in the RPE cell membranes may be involved in the disease process. The observation of an unusually active retinol esterifying enzyme in the microsomal fraction of cattle RPE6, that may form part of a vitamin A cycle in this cell layer7, prompted us to investigate retinol metabolism in the RPE of the RCS rat. We have now found that in dystrophic animals, a pronounced defect of retinol esterifying enzyme is apparent as early as the 10th postnatal day, and is accompanied by a small excess of retinol in the RPE.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Bourne, M. D., Campbell, D. A. & Tansley, K. Br. J. Ophthal. 22, 613–623 (1938).

    Article  CAS  Google Scholar 

  2. Dowling, J. E. & Sidman, R. L. J. Cell Biol. 14, 73–109 (1962).

    Article  CAS  Google Scholar 

  3. Herron, W. L. Jr, Riegel, B. W., Myers, O. E. & Rubin, M. L. Invest. Opthal. 8, 595–604 (1969).

    CAS  Google Scholar 

  4. Bok, D. & Hall, M. O. J. Cell Biol. 49, 664–682 (1971).

    Article  CAS  Google Scholar 

  5. Mullen, R. J. & LaVail, M. M. Science 192, 799–801 (1976).

    Article  ADS  CAS  Google Scholar 

  6. Berman, E. R., Horowitz, J., Segal, N., Fisher, S. & Feeney-Burns, L. Biochim. biophys. Acta 630, 36–46 (1980).

    Article  CAS  Google Scholar 

  7. Berman, E. R., Segal, N., Schneider, A. & Feeny, L. Neurochemistry 1, 113–122 (1980).

    Article  CAS  Google Scholar 

  8. Kuwabara, T. in The Retinal Pigment Epithelium (eds Zinn, K. M. & Marmor, M. F) 293–313 (Harvard University Press, 1979).

    Google Scholar 

  9. Reading, H. W. J. med. Genet. 7, 277–284 (1970).

    Article  CAS  Google Scholar 

  10. Dowling, J. E. Nature 188, 114–118 (1960).

    Article  ADS  CAS  Google Scholar 

  11. Chaitin, M. H. & Williams, T. P. Expl Eye Res. 24, 553–558 (1977).

    Article  CAS  Google Scholar 

  12. Robison, W. G. Jr. & Kuwabara, T. Invest. Opthal. vis. Sci. 16, 1110–1117 (1977)

    CAS  Google Scholar 

  13. La Vail, M. M. & Battelle, B. A. Expl Eye Res. 21, 167–192 (1975).

    Article  CAS  Google Scholar 

  14. Delmelle, M., Noell, W. K. & Organisciak, D. T. Expl Eye Res. 21, 369–380 (1975)

    Article  CAS  Google Scholar 

  15. Wiggert, B. O., Bergsma, D. R. & Chader, G. J. Expl Eye Res. 22, 411–418 (1976).

    Article  CAS  Google Scholar 

  16. Bok, D., Heller, J. & Invest. Opthal. vis. Sci. 19, 1405–1414 (1980).

    CAS  Google Scholar 

  17. Essner, E., Gorrin, G. M. & Griewski, R. A. Invest. Opthal. vis. Sci 17, 278–288 (1978).

    CAS  Google Scholar 

  18. Rothman, H., Feeney, L. & Berman, E. R. Expl Eye Res. 22, 519–532 (1976).

    Article  CAS  Google Scholar 

  19. La Vail, M. M. in The Retinal Pigment Epithelium (eds Zinn, K. M. & Marmor M. F.) 357–380 (Harvard University Press, 1979).

    Google Scholar 

  20. Goodall, A. H., Fisher, D. & Lucy, J. A. Biochim. biophys. Acta 595, 9–14 (1980).

    Article  CAS  Google Scholar 

  21. Berman, E. R., Segal, N. & Feeney, L. Biochim. biophys. Acta 572, 167–177 (1979).

    Article  CAS  Google Scholar 

  22. Alvarez, R. A., Bridges, C. D. B. & Fong, S.-L. Invest. Opthal. vis. Sci. 20, 304–313 (1981).

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Opthalmic Biochemistry Unit, Hadassah University Hospital, Jerusalem, Israel

    E. R. Berman, N. Segal, S. Photiou & H. Rothman

  2. Department of Opthalmology, University of Missouri School of Medicine, Columbia, Missouri, 65201, USA

    L. Feeney-Burns

Authors
  1. E. R. Berman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. N. Segal
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. S. Photiou
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. H. Rothman
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. L. Feeney-Burns
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Berman, E., Segal, N., Photiou, S. et al. Inherited retinal dystrophy in RCS rats: a deficiency in vitamin A esterification in pigment epithelium. Nature 293, 217–220 (1981). https://doi.org/10.1038/293217a0

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/293217a0

  • Springer Nature Limited

This article is cited by

Search

Navigation