Characterization of Deficient Heme Synthase Activity in Protoporphyria with Cultured Skin Fibroblasts
AUTOR(ES)
Bloomer, Joseph R.
RESUMO
Heme synthase (ferrochelatase) activity, as determined by the chelation of ferrous iron to protoporphyrin or deuteroporphyrin, is reduced to 10-25% of normal in tissues of patients with protoporphyria. With cultured skin fibroblasts from seven patients with protoporphyria and six normal individuals, the present studies examined the enzymatic defect.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=371370Documentos Relacionados
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