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  • Title: Diminished erythroid ferrochelatase activity in protoporphyria.
    Author: Bottomley SS, Tanaka M, Everett MA.
    Journal: J Lab Clin Med; 1975 Jul; 86(1):126-31. PubMed ID: 1151134.
    Abstract:
    In two patients with protoporphyria the enzymatic synthesis of aminolevulinic acid and prophobilinogen in erythroid tissue was normal. Boine marrow ferrochelatase activity was less than one-fourth of the mean activity in normal control subjects. Ferrochelatase activity in peripheral blood reticulocytes was less than 10% of controls. This metabolic abnormality provides one biochemical explanation for the increased concentrations of blood protoporphyrin in protoporphyria and clarifies the apparent minimally impaired hemoglobin synthesis in the two case studies.
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