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Free radicals involvement in neurological porphyrias and lead poisoning

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Abstract

Porphyrias are inherited and acquired diseases of erythroid or hepatic origin, in which there are defects in specific enzymes of the heme biosynthetic pathway. In patients with intermittent acute porphyria and lead poisoning the erythrocytic activities of superoxide dismutase and glutathione peroxidase are reported to be increased. Our studies demonstrated that d-aminolevulinic acid, a heme precursor accumulated in both diseases, undergoes enolization at pH < 7.0 before it autoxidizes. The autoxidation of d-aminolevulinic acid, in the presence or absence of oxyhemoglobin has been proposed as a source of oxy and carbon-centred radicals in the cells of intermittent acute porphyria and saturnism carriers. Thus, the increased levels of antioxidant enzymes can be viewed as an intracellular response against the deleterious effects of these extremely reactive species.

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  1. Hugo P. Monteiro

    Present address: New York University Medical Center, 550-560, 1st Ave., 10016, New York, N. Y.

Authors and Affiliations

  1. Fundaçao Hemocentro de Sao Paulo-FIHSP A v. Dr. Eneas de Carvalho Aguiar, 255, PAMB, B1.12 e 6, CEP: 05403, Sao Paulo-SP, Brazil

    Hugo P. Monteiro

  2. Departamento de Bioquimica, Instituto de Quimica da Universidade de Sao Paulo, Cx. Postal 20780, Sao Paulo-SP, Brazil

    Etelvino J. H. Bechara

  3. Faculdade de Ciências Farmacêuticas da Universidade de Sao Paulo, Cx. Postal 30786, Sao Paulo-SP, Brazil

    Dulcineia S. P. Abdalla

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  1. Hugo P. Monteiro
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Monteiro, H.P., Bechara, E.J.H. & Abdalla, D.S.P. Free radicals involvement in neurological porphyrias and lead poisoning. Mol Cell Biochem 103, 73–83 (1991). https://doi.org/10.1007/BF00229595

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