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  • Title: Enzyme abnormalities in the porphyrias.
    Author: Brodie MJ, Moore MR, Goldberg A.
    Journal: Lancet; 1977 Oct 01; 2(8040):699-701. PubMed ID: 71507.
    Abstract:
    Evidence is presented that each of the porphyrias represents a different inborn error of metabolism in haem biosynthesis. Control of the pathway takes place by feedback repression and inhibition by haem of delta-aminolaevulinic-acid synthase. It is suggested that insituations where the activity of this enzyme is derepressed, prophobilinogen deaminase represents a secondary control step.
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