Redox active plasma iron in C282Y/C282Y hemochromatosis.
Labile plasma iron (LPI) represents the redox active component of non-transferrin-bound iron (NTBI). Its presence in thalassemic patients has been recently reported. The aim of the present study was to quantify LPI in HFE genetic hemochromatosis (GH) and to characterize the mechanisms accounting for its appearance. We studied 159 subjects subdivided into the following groups: (1) 23 with iron overloaded GH; (2) 14 with iron-depleted GH; (3) 26 with dysmetabolic hepatosiderosis; (4) 33 with alcoholic cirrhosis; (5) 63 healthy controls. Both NTBI and LPI were substantially higher in patients with iron-overloaded GH than in those with iron-depleted GH or in healthy controls. LPI was significantly correlated with serum transaminase increase in this group. LPI was elevated in the alcoholic cirrhosis subgroup of severely affected patients. LPI was found essentially when transferrin saturation exceeded 75%, regardless of the etiologic condition. Transferrin saturation above 75% was related to iron overload in GH and to liver failure in alcoholic cirrhosis. LPI is present in C282Y/C282Y hemochromatosis and may be a marker of toxicity due to its potential for catalyzing the generation of reactive oxygen radicals in vivo.
Le Lan
Caroline
C
Service des Maladies du Foie, INSERM U-522 and Laboratoire de Biochimie Générale et Enzymologie, University Hospital Pontchaillou, Rennes 35033, France.
Loréal
Olivier
O
Cohen
Tally
T
Ropert
Martine
M
Glickstein
Hava
H
Lainé
Fabrice
F
Pouchard
Michel
M
Deugnier
Yves
Y
Le Treut
André
A
Breuer
William
W
Cabantchik
Z Ioav
ZI
Brissot
Pierre
P
D000328:Adult; D016022:Case-Control Studies; D005838:Genotype; D006432:Hemochromatosis / Q000097:blood / Q000235:genetics*; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I / Q000235:genetics; D006801:Humans; D007501:Iron / Q000097:blood* / Q000378:metabolism; D000090463:Iron Deficiencies; D019190:Iron Overload / Q000097:blood / Q000235:genetics; D008104:Liver Cirrhosis, Alcoholic / Q000097:blood / Q000235:genetics; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D008875:Middle Aged; D020125:Mutation, Missense; D010084:Oxidation-Reduction; D011446:Prospective Studies; D000637:Transaminases / Q000097:blood; D014168:Transferrin / Q000378:metabolism
C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D014168:Transferrin; D007501:Iron; D000637:Transaminases