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. 1977 Oct 1;2(8040):699-701.
doi: 10.1016/s0140-6736(77)90507-4.

Enzyme abnormalities in the porphyrias

M J Brodie, M R Moore, A Goldberg

Enzyme abnormalities in the porphyrias

M J Brodie et al. Lancet. .

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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