STUDIES IN PORPHYRIA
Open Access
- 1 October 1973
- journal article
- Published by Rockefeller University Press in The Journal of Experimental Medicine
- Vol. 138 (4) , 754-763
- https://doi.org/10.1084/jem.138.4.754
Abstract
Patients with the genetic liver disease, acute intermittent porphyria (AIP), have a defect in the reductive transformation of steroid hormones that is manifest by the disproportionate generation of 5ß-steroid metabolites from precursor hormones. 5ß-steroid metabolites were earlier shown to be potent inducers experimentally of δ-aminolevulinate synthetase (ALAS), the mitochondrial enzyme that is rate-limiting in porphyrin synthesis, and that is found at high levels of activity in the livers of AIP patients.Keywords
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