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. 1970 Nov;67(3):1315–1320. doi: 10.1073/pnas.67.3.1315

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

L James Strand *,‡,†, Bertram F Felsher ‡, Allan G Redeker *,‡, Harvey S Marver *,‡,†
PMCID: PMC283354  PMID: 5274461

Abstract

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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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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