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Activation of uroporphyrinogen decarboxylase by ferrous iron in porphyria cutanea tarda.
S Afr Med J 1979; 56(22):918-20SA

Abstract

Uroporphyrinogen decarboxylase was measured in the presence of ferrous iron, using mitochondria-free rat liver extracts as enzyme source. The activity of the enzyme was found to be increased at concentrations of ferrous iron from 0,01 mM, with maximal activity exhibited from 0,1 mM. Enzyme kinetics indicate that uroporphyrinogen decarboxylase reversibly binds ferrous iron, with a binding constant of approximately 5 x 10(4) mol-1. It is proposed that the effect of phlebotomy on patients with porphyria cutanea tarda is to mobilze storage iron in the liver to the active ferrous form, which activates hepatic uroporphyrinogen decarboxylase, the enzyme which is defective in this syndrome, with resultant clinical and biochemical remission.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

515872

Citation

Blekkenhorst, G H., et al. "Activation of Uroporphyrinogen Decarboxylase By Ferrous Iron in Porphyria Cutanea Tarda." South African Medical Journal = Suid-Afrikaanse Tydskrif Vir Geneeskunde, vol. 56, no. 22, 1979, pp. 918-20.
Blekkenhorst GH, Eales L, Pimstone NR. Activation of uroporphyrinogen decarboxylase by ferrous iron in porphyria cutanea tarda. S Afr Med J. 1979;56(22):918-20.
Blekkenhorst, G. H., Eales, L., & Pimstone, N. R. (1979). Activation of uroporphyrinogen decarboxylase by ferrous iron in porphyria cutanea tarda. South African Medical Journal = Suid-Afrikaanse Tydskrif Vir Geneeskunde, 56(22), pp. 918-20.
Blekkenhorst GH, Eales L, Pimstone NR. Activation of Uroporphyrinogen Decarboxylase By Ferrous Iron in Porphyria Cutanea Tarda. S Afr Med J. 1979 Nov 24;56(22):918-20. PubMed PMID: 515872.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Activation of uroporphyrinogen decarboxylase by ferrous iron in porphyria cutanea tarda. AU - Blekkenhorst,G H, AU - Eales,L, AU - Pimstone,N R, PY - 1979/11/24/pubmed PY - 1979/11/24/medline PY - 1979/11/24/entrez SP - 918 EP - 20 JF - South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde JO - S. Afr. Med. J. VL - 56 IS - 22 N2 - Uroporphyrinogen decarboxylase was measured in the presence of ferrous iron, using mitochondria-free rat liver extracts as enzyme source. The activity of the enzyme was found to be increased at concentrations of ferrous iron from 0,01 mM, with maximal activity exhibited from 0,1 mM. Enzyme kinetics indicate that uroporphyrinogen decarboxylase reversibly binds ferrous iron, with a binding constant of approximately 5 x 10(4) mol-1. It is proposed that the effect of phlebotomy on patients with porphyria cutanea tarda is to mobilze storage iron in the liver to the active ferrous form, which activates hepatic uroporphyrinogen decarboxylase, the enzyme which is defective in this syndrome, with resultant clinical and biochemical remission. SN - 0256-9574 UR - https://www.unboundmedicine.com/medline/citation/515872/Activation_of_uroporphyrinogen_decarboxylase_by_ferrous_iron_in_porphyria_cutanea_tarda_ L2 - http://amigo.geneontology.org/amigo/reference/PMID:515872 DB - PRIME DP - Unbound Medicine ER -