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Iron-responsive element-binding protein in hemochromatosis liver and intestine.

Abstract
Iron-responsive element-binding protein (IRE-BP) activity was studied in liver and intestinal samples of hemochromatosis and control patients using a short 32P-IRE-RNA probe on "retardation" nondenaturing polyacrylamide gels. IRE-BP activity was assessed in liver biopsy specimens in 36 patients--16 hemochromatosis homozygotes, 4 hemochromatosis heterozygotes, 6 patients with secondary iron overload, and 10 control patients with normal hepatic iron concentrations. Intestinal IRE-BP activity was assessed in 14 hemochromatosis homozygotes and 16 normal subjects. Endogenous IRE-BP activity was determined from 32P retarded on the gel, and total IRE-BP activity was assessed after reducing tissue samples with 2-mercaptoethanol. Hepatic endogenous IRE-BP activity was inversely related to hepatic iron concentration (r = .59, P < .0002). Mean hepatic endogenous IRE-BP activity in the hemochromatosis homozygotes, 0.25 +/- 0.04 pmol/mg protein, was significantly decreased compared with values in the normal controls, 0.45 +/- 0.06 pmol/mg protein, P < .05. Hepatic total IRE-BP was also significantly decreased in the hemochromatosis patients by gel retardation assay and Western blotting with anti-IRE-BP antibody. Intestinal endogenous IRE-BP activity, total IRE-BP activity, and iron concentration did not significantly differ between hemochromatosis patients and normal control subjects. This suggests that both endogenous IRE-BP activity and the total amount of the protein are downregulated in the liver by tissue iron. Intestinal IRE-BP activity that regulates intestinal transferrin receptor expression is normal in hemochromatosis and appropriate for the intracellular iron concentration.
AuthorsP R Flanagan, A Hajdu, P C Adams
JournalHepatology (Baltimore, Md.) (Hepatology) Vol. 22 Issue 3 Pg. 828-32 (Sep 1995) ISSN: 0270-9139 [Print] United States
PMID7657289 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Iron-Regulatory Proteins
  • RNA-Binding Proteins
  • Receptors, Transferrin
Topics
  • Adult
  • Aged
  • Blotting, Western
  • Female
  • Hemochromatosis (metabolism)
  • Humans
  • Intestinal Mucosa (metabolism)
  • Iron-Regulatory Proteins
  • Liver (metabolism)
  • Male
  • Middle Aged
  • RNA-Binding Proteins (metabolism)
  • Receptors, Transferrin (metabolism)
  • Reference Values

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