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Membrane fatty acids, glutathione-peroxidase activity, and cation transport systems of erythrocytes and malondialdehyde production by platelets in Laurence Moon Barter Biedl Syndrome

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Abstract

The fatty acid composition of erythrocyte membrane, the glutathione-peroxidase activity of erythrocytes and platelets, the production of malondialdehyde by platelets and the activity of the main systems of transmembrane cation transport have been studied in 5 members of a family, 2 of whom affected by Laurence-Moon-Barter-Biedl Syndrome. A remarkable increase of polyunsaturated fatty acids (particularly arachidonic acid) and of cholesterol/phospholipid molar ratio has been noted. This pattern of membrane lipids was associated to an increment of malondialdehyde production and an increase activity of glutathione-peroxidase. Serum retinol and a-tocopherol were in the normal range, whereas serum selenium was low in 3 out of 5 members. Moreover, the alteration of membrane lipids was associated to a decrease of the maximal velocity of Li-Na countertransport. We speculate that the enrichment of polyunsaturated fatty acids on the cell membranes may represent a condition favoring the lipoperoxidation and therefore the development of the retinitis pigmentosa characteristic feature of Laurence-Moon-Barter-Biedl Syndrome.

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Authors and Affiliations

  1. Istituto di Patologia Medica, Università di Verona, 37100, Verona, Italy

    Roberto Corrocher, L. Guadagnin, M. de Gironcoli, D. Girelli, P. Guarini, O. Olivieri, S. Caffi & A. M. Stanzial

  2. Istituto di Chimica e Microscopia Clinica, Università di Verona, 37100, Verona, Italy

    S. Ferrari & L. Grigolini

Authors
  1. Roberto Corrocher
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  2. L. Guadagnin
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  3. M. de Gironcoli
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  4. D. Girelli
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  5. P. Guarini
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  6. O. Olivieri
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  7. S. Caffi
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  8. A. M. Stanzial
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  9. S. Ferrari
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  10. L. Grigolini
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Supported by grants from: Ministero della Pubblica Istruzione, Assessorato alla Sanità - Veneto.

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Corrocher, R., Guadagnin, L., de Gironcoli, M. et al. Membrane fatty acids, glutathione-peroxidase activity, and cation transport systems of erythrocytes and malondialdehyde production by platelets in Laurence Moon Barter Biedl Syndrome. J Endocrinol Invest 12, 475–481 (1989). https://doi.org/10.1007/BF03350737

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  • DOI: https://doi.org/10.1007/BF03350737

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