Uroporphyrinogen III cosynthetase activity in fibroblasts from patients with congenital erythropoietic porphyria |
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| Authors: | Giovanni Romeo Michael M. Kaback Ephraim Y. Levin |
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| Affiliation: | (1) Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland |
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| Abstract: | The activity of uroporphyrinogen III cosynthetase is lower in extracts of fibroblasts from patients with congenital erythropoietic porphyria than in extracts of fibroblasts from control subjects. The porphyric extracts do not inhibit the cosynthetase activity of control extracts. The genetically determined enzymatic defect in this disease can thus occur in other tissues besides bone marrow.Supported in part by a National Institutes of Health Grant (NB-05367). |
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