Disease of Ornithine-Proline Pathway: A △1-pyrroline-5-carboxylate Reductase Deficiency in the Retina of Retinal Degeneration Mice

  • Takeo Matsuzawa
  • Koichi Iwasaki
  • Noriko Hiraiwa
  • Etsuko Inagaki
  • Isao Ishiguro
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

Abstract

The discovery of a genetic defect in ornithine oxoacid aminotransferase (L-ornithine: 2-oxoacid aminotransferase, EC 2.6.1.13) in gyrate atrophy of the choroid and retina1,2,3, prompted us to study the ornithine metabolism of the eye4. We soon found high 361 activities of ornithine oxoacid aminotransferase and △1-pyrroline-5-carboxylate reductase (L-proline:NAD(P)+5-oxidoreductase, EC 1.5.1.2) (P5C reductase) in the retina and retinal pigment epithelium4,5. Gyrate atrophy of the choroid and retina is a disease related to the category of choroideremia and retinitis pigmentosa (primary, hereditary, pigmentary retinopathy), these are much frequent cause of the human hereditary blindness, however the biochemical pathogenesis of these diseases remains to be solved. In this paper we describe an intracellular proline synthetic pathway from ornithine uncovered in the bovine cornea and retinal outer layers(including the choroid) which presumably participates in the biosynthesis of proline-rich proteins such as collagen and glycoproteins, and the characteristic postnatal changes and the deficiency of this pathway in C3H retinal degeneration mice.

Keywords

Retinitis Pigmentosa Ocular Tissue Arginase Activity Retinal Outer Layer Pigmentary Retinopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    J. M. F. Trijbels, R. C. A. Sengers, J. A. J. M. Bakkeren, A. F. M. DeKort and A. F. Deutman, L-Ornithine-ketoacid-transaminase deficiency in cultured fibroblasts of a patient with hyperornithinemia and gyrate atrophy of the choroid and retina, Clin. Chim. Acta 79: 371 (1977).CrossRefGoogle Scholar
  2. 2.
    M. I. Kaiser-Kupfer, D. Valle and L. A. Del Valle, A specific enzyme defect in gyrate atrophy, Am. J. Ophthal. 85: 200 (1978).PubMedGoogle Scholar
  3. 3.
    S. Hayasaka, T. Shono, K. Mizuno, T. Saito and K. Tada, Ornithine ketoacid aminotransferase activity in gyrate atrophy patients and bovine eye, Invest. Ophthal. Visual Sci.(Suppl). ARVO abstract 19: 185 (1980).Google Scholar
  4. 4.
    T. Matsuzawa, I. Ishiguro, S. Hayasaka, T. Shiono, H. Nakajima and K. Mizuno, Hyperornithinemia with gyrate atrophy and enzymes involved in ornithine metabolism of the eye, Biochem. International 1: 179 (1980).Google Scholar
  5. 5.
    S. Hayasaka, T. Shono, Y. Takaku and K. Mizuno, Ornithine ketoacid aminotransferase in the bovine eye, Invest. Ophthal. Visual Sci. 19: 1457 (1980).Google Scholar
  6. 6.
    R. J. Smith, S. J. Downing, J. M. Phang, R. F. Lodato and T. T. Aoki, Pyrroline-5-carboxylate synthase activity in mammalian cells, Proc. Natl. Acad. Sci. USA 77: 5221 (1980).PubMedCrossRefGoogle Scholar
  7. 7.
    J. J. OtDonnell, R. P. Sandman and S. R. Martin, Assay of Orni-thine aminotransferase by high-performance liquid chromatography, Anal. Biochem. 90: 41 (1978).Google Scholar
  8. 8.
    M. M. Bradford, A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding, Anal. Biochem. 72: 248 (1976).PubMedCrossRefGoogle Scholar
  9. 9.
    T. Matsuzawa and I. Ishiguro, Ornithine metabolism in relation to stimulation of urea cycle, induced by high protein diet, Arch. Biochem. Biophys. 208: 101 (1981).CrossRefGoogle Scholar
  10. 10.
    C. McCulloch and E. B. Marliss, Gyrate atrophy of the choroid and retina with hyperornithinemia, Am. J. Ophthal. 80: 1047 (1975).PubMedGoogle Scholar
  11. 11.
    K. Mizuno, Plenary lecture: Pathology and clinic of hereditary chorioretinal degeneration, Acta Soc. Ophthal. Jap. (Suppl). 85: 3 (Abstract) (1981).Google Scholar
  12. 12.
    K. Tansley, Hereditary degeneration of the mouse retina, Brit. J. Ophthal. 35: 573 (1952).Google Scholar
  13. 13.
    T. Collins, Abiotrophy of the retinal neuroepithelium, or retinitis pigmentosa, Arch. Ophthal., N.Y. 48: 517 (1919).Google Scholar

Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • Takeo Matsuzawa
    • 1
  • Koichi Iwasaki
    • 1
  • Noriko Hiraiwa
    • 2
  • Etsuko Inagaki
    • 1
  • Isao Ishiguro
    • 1
  1. 1.School of MedicineFujita-Gakuen UniversityToyoake, AichiJapan
  2. 2.Department of Biochemistry and OphthalmologyFujita-Gakuen UniversityToyoake, AichiJapan

Personalised recommendations