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.
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References
Bourne, M. D., Campbell, D. A. & Tansley, K. Br. J. Ophthal. 22, 613–623 (1938).
Dowling, J. E. & Sidman, R. L. J. Cell Biol. 14, 73–109 (1962).
Herron, W. L. Jr, Riegel, B. W., Myers, O. E. & Rubin, M. L. Invest. Opthal. 8, 595–604 (1969).
Bok, D. & Hall, M. O. J. Cell Biol. 49, 664–682 (1971).
Mullen, R. J. & LaVail, M. M. Science 192, 799–801 (1976).
Berman, E. R., Horowitz, J., Segal, N., Fisher, S. & Feeney-Burns, L. Biochim. biophys. Acta 630, 36–46 (1980).
Berman, E. R., Segal, N., Schneider, A. & Feeny, L. Neurochemistry 1, 113–122 (1980).
Kuwabara, T. in The Retinal Pigment Epithelium (eds Zinn, K. M. & Marmor, M. F) 293–313 (Harvard University Press, 1979).
Reading, H. W. J. med. Genet. 7, 277–284 (1970).
Dowling, J. E. Nature 188, 114–118 (1960).
Chaitin, M. H. & Williams, T. P. Expl Eye Res. 24, 553–558 (1977).
Robison, W. G. Jr. & Kuwabara, T. Invest. Opthal. vis. Sci. 16, 1110–1117 (1977)
La Vail, M. M. & Battelle, B. A. Expl Eye Res. 21, 167–192 (1975).
Delmelle, M., Noell, W. K. & Organisciak, D. T. Expl Eye Res. 21, 369–380 (1975)
Wiggert, B. O., Bergsma, D. R. & Chader, G. J. Expl Eye Res. 22, 411–418 (1976).
Bok, D., Heller, J. & Invest. Opthal. vis. Sci. 19, 1405–1414 (1980).
Essner, E., Gorrin, G. M. & Griewski, R. A. Invest. Opthal. vis. Sci 17, 278–288 (1978).
Rothman, H., Feeney, L. & Berman, E. R. Expl Eye Res. 22, 519–532 (1976).
La Vail, M. M. in The Retinal Pigment Epithelium (eds Zinn, K. M. & Marmor M. F.) 357–380 (Harvard University Press, 1979).
Goodall, A. H., Fisher, D. & Lucy, J. A. Biochim. biophys. Acta 595, 9–14 (1980).
Berman, E. R., Segal, N. & Feeney, L. Biochim. biophys. Acta 572, 167–177 (1979).
Alvarez, R. A., Bridges, C. D. B. & Fong, S.-L. Invest. Opthal. vis. Sci. 20, 304–313 (1981).
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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
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DOI: https://doi.org/10.1038/293217a0
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