Advertisement

Experimental protoporphyria in hairless mice: Photodermatitis

  • H. Baart De La Faille
  • R. A. Woutersen
  • H. Van Weelden
  • E. H. Baart De La Faille-Kuyper
Part of the Inflammation: Mechanisms and Treatment book series (FTIN, volume 4)

Abstract

Erythropoietic protoporphyria (EPP) is an inheritable inborn error of human metabolism giving rise to an excess production and accumulation of the haem precursor protoporphyrin1–3. Increased concentrations of protoporphyrin can be demonstrated in, for example, erythrocytes, and to a smaller extent also in plasma. The excretion of the substance in faecal matter is often elevated; urinary porphyrins are always normal.

Keywords

Skin Thickness Hairless Mouse Violet Light Urinary Porphyrin Erythropoietic Protoporphyria 
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.
    Magnus, I. A., Jarrett, A., Prankerd, T. A. J. and Rimington, C. (1961). Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. Lancet, 2, 448PubMedCrossRefGoogle Scholar
  2. 2.
    DeLeo, V. A., Poh-Fitzpatrick, M. Mathews-Roth, M and Harber, L. C. (1976). Erythropoietic protoporphyria. 10 years experience. Am. J. Med., 60, 8Google Scholar
  3. 3.
    Baart de la Faille, H. (1979). Erythropoetische Protoporphyrie. In Körting, G. (ed.) Dermatologie in Praxis und Klinik, pp. 38.69–38.82 (Stuttgart: Georg Thieme Verlag)Google Scholar
  4. 4.
    Baart de la Faille, H. (1975). Erythropoietic protoporphyria. A photodermatosis. Microscopical and electron microscopical investigations of irradiated skin and a clinical study. (Utrecht: Oosthoek, Scheltema and Holkema)Google Scholar
  5. 5.
    Gschnait, F., Wolff, K. and Konrad, K. (1975). Erythropoietic protoporphyria-submicroscopic events during the acute photosensitivity flare. Br. J. Dermatol., 92, 545CrossRefGoogle Scholar
  6. 6.
    Allison, A. C., Magnus, I. A. and Young, M. R. (1966). Role of lysosomes and cell membranes in photosensitization. Nature (London), 209, 874CrossRefGoogle Scholar
  7. 7.
    Schothorst, A. A., Suurmond, D. and Ploem, J.S. (1977). In vitro studies on the protoporphyrin uptake and photosensitivity of normal skin fibroblasts and fibroblasts from patients with erythropoietic protoporphyria. J. Invest. Dermatol., 69, 551PubMedCrossRefGoogle Scholar
  8. 8.
    Huber, J. D., Parker, F. and Odland, G. F. (1968). A basic fuchsine and alkalinized methylene blue rapid stain for epoxy-embedded tissue. Stain Technol., 43, 83PubMedGoogle Scholar
  9. 9.
    Berg, N. O. (1951). Histological studies of masked lipids. Stainability, distribution and functional variations. Acta Pathol. Scand. Suppl.,90,Google Scholar
  10. 10.
    Lillie, R. D. and Ashburn, L. L. (1943). Laboratory methods and technical notes. Supersaturated solutions of fat stains in dilute isopropanol for demonstration of acute fatty degenerations not shown by Herxheimer technic. Arch. Pathol, 36,432Google Scholar
  11. 11.
    Romeis, B. (1968). Mikroskopische Technik, 16th Edn. (München, R. Oldenbourg Verlag)Google Scholar
  12. 12.
    Gomori, G. (1951). Alkaline phosphatase of cell nuclei. J. Lab. Clin. Med., 37, 526PubMedGoogle Scholar
  13. 13.
    Burstone, M. S. (1958). Histochemical comparison of naphtol AS-phosphates for the demonstration of phosphatases. J. Natl. Cancer Inst., 20, 601PubMedGoogle Scholar
  14. 14.
    Woutersen, R. A., Holsteijn van, C. W. M. and Baart de la Faille, H. (1980). Experimental protoporphyria in hairless mice. II. Photohaemolysis versus Photodermatitis. This volume, Chap. 88Google Scholar
  15. 15.
    Konrad, K., Hönigsmann, H., Gschnait, F. and Wolff, K. (1975). Mouse model for protoporphyria. II. Cellular and subcellular events in the photosensitivity flare of the skin. J. Invest. Dermatol, 65, 300PubMedCrossRefGoogle Scholar
  16. 16.
    Cannistraro, S., van der Vorst, A. and Jori, G. (1978). EPR studies on singlet oxygen production by porphyrins. Photochem. Photobiol., 28, 257CrossRefGoogle Scholar
  17. 17.
    Buettner, G. and Oberley, L. W. (1979). Superoxide formation by protoporphyrin as seen by spin trapping. FEBS Lett., 98,18PubMedCrossRefGoogle Scholar

Copyright information

© MTP Press Limited 1980

Authors and Affiliations

  • H. Baart De La Faille
    • 1
  • R. A. Woutersen
    • 1
  • H. Van Weelden
    • 1
  • E. H. Baart De La Faille-Kuyper
    • 1
  1. 1.Netherlands

Personalised recommendations