The incorporation of [4-14C] delta-aminolaevulic acid into urinary porphyrins in symptomatic porphyria.

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1. The specific activities of urinary uroporphyrin and coproporphyrin were measured as functions of time following the administration of a single oral dose of [4-14C] δ-aminolaevulic acid (ALA) to six patients with symptomatic porphyria and one control subject. 2. The peak specific activity of coproporphyrin preceded that of uroporphyrin in all subjects studied and exceeded that of uroporphyrin in the patients with symptomatic porphyria. 3. These results are interpreted as indicating the existence of two distinct metabolic pathways in the liver for the disposal of ALA, rather than as contradicting the generally accepted role of uroporphyrinogen as a precursor of coproporphyrinogen.