title: Redox active plasma iron in C282Y/C282Y hemochromatosis.
abstract: 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.
authors.affiliation: Service des Maladies du Foie, INSERM U-522 and Laboratoire de Biochimie Générale et Enzymologie, University Hospital Pontchaillou, Rennes 35033, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Le Lan
authors.forename: Olivier
authors.initials: O
authors.lastname: Loréal
authors.forename: Tally
authors.initials: T
authors.lastname: Cohen
authors.forename: Martine
authors.initials: M
authors.lastname: Ropert
authors.forename: Hava
authors.initials: H
authors.lastname: Glickstein
authors.forename: Fabrice
authors.initials: F
authors.lastname: Lainé
authors.forename: Michel
authors.initials: M
authors.lastname: Pouchard
authors.forename: Yves
authors.initials: Y
authors.lastname: Deugnier
authors.forename: André
authors.initials: A
authors.lastname: Le Treut
authors.forename: William
authors.initials: W
authors.lastname: Breuer
authors.forename: Z Ioav
authors.initials: ZI
authors.lastname: Cabantchik
authors.forename: Pierre
authors.initials: P
authors.lastname: Brissot
mesh_terms: 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
chemical_list: C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D014168:Transferrin; D007501:Iron; D000637:Transaminases