Heme Biosynthesis in Intermittent Acute Porphyria: Decreased Hepatic Conversion of Porphobilinogen to Porphyrins and Increased Delta Aminolevulinic Acid Synthetase Activity*

AUTOR(ES)

Strand, L. James

RESUMO

Hepatic conversion of porphobilinogen to porphyrins was less than 50% of control levels in human subjects with the genetic disease, intermittent acute porphyria. This relative block in heme biosynthesis may be relevant to a concomitant 6- to 10-fold elevation in δ-aminolevulinic acid synthetase activity, since this first and rate-controlling enzyme in the biosynthetic pathway is subject to negative feedback regulation by the end product, heme. A micro-radio-chemical assay of δ-aminolevulinic acid synthetase, and some of its applications, are described.

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