Heme synthetase deficiency in human protoporphyria. Demonstration of the defect in liver and cultured skin fibroblasts.

The final step in heme biosynthesis is chelation of porphyrin with Fe++ catalyzed by the mitochondrial enzyme heme synthetase. We have employed a sensitive radiochemical assay for this enzyme, using 59Fe and deuteroporphyrin or protoporphyrin as substrates. In this method iron is maintained in the f...

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Publié dans:	J Clin Invest
Auteurs principaux:	Bonkowsky, H L, Bloomer, J R, Ebert, P S, Mahoney, M J
Format:	Artigo
Langue:	Inglês
Publié:	American Society for Clinical Investigation 1975
Sujets:	Research Article
Accès en ligne:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC301976/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/1184741/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI108189
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