The Porphyrias

  • J. Prieto
  • J. Camps


The porphyrias represent a group of diseases which have in common an alteration in one of the steps of the biosynthesis of heme. Porphyrinogens and porphyrins are intermediates in this pathway. Heme or Fe-protopor- phyrin IX is produced in all cells, but principally in the liver and bone marrow. In the liver it combines with apoproteins to form cytochrome P-450 and other heme proteins. In the bone marrow it binds to the globin moiety, giving rise to hemoglobin. Free porphyrins lack physiologic functions and in humans they are normally produced in only very low amounts as byproducts of the biosynthesis of heme.


Porphyria Cutanea Tarda Intermittent Acute Porphyria Acute Intermittent Porphyria Hepatic Porphyria Heme Biosynthetic Pathway 
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© Springer-Verlag Berlin Heidelberg 1992

Authors and Affiliations

  • J. Prieto
  • J. Camps

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