[Porphyrin-synthesizing enzymes in erythrocyte]

There are 4 different porphyrin-synthesizing enzymes, i.e. delta-aminolevulinic acid dehydratase, porphobilinogen deaminase, uroporphyrinogen III synthase and uroporphyrinogen decarboxylase, in red blood cells. Determination of activities of these enzymes is of great help in clarifying the hemopoietic mechanism operating in bone marrow erythroblasts, as well as, in establishing the diagnosis of or early detection of acute intermittent porphyria, congenital erythropoietic porphyria, delta-aminolevulinic acid dehydratase deficiency porphyria, porphyria cutanea tarda and lead poisoning. This paper presents a summary of the techniques for the measuring, diagnostic criteria for and clinical implications of activities of these 4 enzymes in red blood cells.