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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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Autors principals: Bonkowsky, H L, Bloomer, J R, Ebert, P S, Mahoney, M J
Format: Artigo
Idioma:Inglês
Publicat: 1975
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC301976/
https://ncbi.nlm.nih.gov/pubmed/1184741
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