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Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda.
N Engl J Med 1978; 299(6):274-8NEJM

Abstract

To investigate the role of uroporphyrinogen decarboxylase in the pathogenesis of the sporadic form of porphyria cutanea tarda, we measured this enzyme in liver, erythrocytes and cultured skin fibroblasts, and also measured coproporphyrinogen oxidase and the total iron concentration in liver. The mean uroporphyrinogen decarboxylase activity was lower in liver from seven male patients (9.0 pmol of coproporphyrin per minute per milligram of protein) than in 12 controls, including seven with alcoholic liver disease (22.3 pmol per minute per milligram; P less than 0.05). Coproporphyrinogen oxidase activities were the same in each group. Liver iron concentrations were lower during remission, but uroporphyrinogen decarboxylase activities were not related to clinical activity for uroporphyrin excretion. Erythrocyte and fibroblast enzyme activities were the same as in normal subjects. A hepatic uroporphyrinogen decarboxylase defect is a prerequisite for the development of porphyria cutanea tarda, but other factors, which probably do not alter uroporphyrinogen decarboxylase activity, determine the clinical onset. In sporadic porphyria cutaneous tarda, the enzyme defect appears to be restricted to the liver.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

661926

Citation

Elder, G H., et al. "Decreased Activity of Hepatic Uroporphyrinogen Decarboxylase in Sporadic Porphyria Cutanea Tarda." The New England Journal of Medicine, vol. 299, no. 6, 1978, pp. 274-8.
Elder GH, Lee GB, Tovey JA. Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda. N Engl J Med. 1978;299(6):274-8.
Elder, G. H., Lee, G. B., & Tovey, J. A. (1978). Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda. The New England Journal of Medicine, 299(6), pp. 274-8.
Elder GH, Lee GB, Tovey JA. Decreased Activity of Hepatic Uroporphyrinogen Decarboxylase in Sporadic Porphyria Cutanea Tarda. N Engl J Med. 1978 Aug 10;299(6):274-8. PubMed PMID: 661926.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda. AU - Elder,G H, AU - Lee,G B, AU - Tovey,J A, PY - 1978/8/10/pubmed PY - 1978/8/10/medline PY - 1978/8/10/entrez SP - 274 EP - 8 JF - The New England journal of medicine JO - N. Engl. J. Med. VL - 299 IS - 6 N2 - To investigate the role of uroporphyrinogen decarboxylase in the pathogenesis of the sporadic form of porphyria cutanea tarda, we measured this enzyme in liver, erythrocytes and cultured skin fibroblasts, and also measured coproporphyrinogen oxidase and the total iron concentration in liver. The mean uroporphyrinogen decarboxylase activity was lower in liver from seven male patients (9.0 pmol of coproporphyrin per minute per milligram of protein) than in 12 controls, including seven with alcoholic liver disease (22.3 pmol per minute per milligram; P less than 0.05). Coproporphyrinogen oxidase activities were the same in each group. Liver iron concentrations were lower during remission, but uroporphyrinogen decarboxylase activities were not related to clinical activity for uroporphyrin excretion. Erythrocyte and fibroblast enzyme activities were the same as in normal subjects. A hepatic uroporphyrinogen decarboxylase defect is a prerequisite for the development of porphyria cutanea tarda, but other factors, which probably do not alter uroporphyrinogen decarboxylase activity, determine the clinical onset. In sporadic porphyria cutaneous tarda, the enzyme defect appears to be restricted to the liver. SN - 0028-4793 UR - https://www.unboundmedicine.com/medline/citation/661926/Decreased_activity_of_hepatic_uroporphyrinogen_decarboxylase_in_sporadic_porphyria_cutanea_tarda_ L2 - http://www.nejm.org/doi/full/10.1056/NEJM197808102990603?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -