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Iron‐responsive element‐binding protein in hemochromatosis liver and intestine
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 h...
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Published in: | Hepatology (Baltimore, Md.) Md.), 1995-09, Vol.22 (3), p.828-832 |
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Main Authors: | , , |
Format: | Article |
Language: | English |
Subjects: | |
Citations: | Items that this one cites Items that cite this one |
Online Access: | Get full text |
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Summary: | 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 also 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. (Hepatology 1995; 22:828–832.) |
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ISSN: | 0270-9139 1527-3350 |
DOI: | 10.1002/hep.1840220321 |