Serum ceruloplasmin and ferroxidase activity are decreased in HFE C282Y homozygote male iron-overloaded patients.
OBJECTIVE
A body of evidence suggests that ceruloplasmin (Cp), the major serum copper-containing protein, acts in iron metabolism due to its ferroxidase activity which appears essential for iron movements and exchanges.


METHODS
The present study investigated the serum levels of Cp and its ferroxidase activity in 53 C282Y homozygote genetic hemochromatosis (38 iron overloaded, 15 iron depleted) patients as compared to age and sex-matched healthy volunteers.


RESULTS
Serum levels of Cp were significantly decreased in iron-overloaded male hemochromatotic patients vs. the control group (P=0.02). Furthermore, serum ferroxidase activity was strongly and significantly lower in iron-overloaded male hemochromatotic patients (P<0.001). In contrast, in iron-depleted male hemochromatotic patients, who were under maintenance therapy by regular phlebotomies, serum levels of Cp and ferroxidase activity were not statistically different from those observed in controls.


CONCLUSIONS
These data: (i) show that serum Cp and ferroxidase activity are decreased when C282Y homozygote men are iron overloaded and normal when iron depleted; (ii) suggest that iron may modulate the Cp gene expression; and (iii) raise the issue of the putative role of decreased serum ferroxidase activity in the phenotypic expression of HFE-1 hereditary hemochromatosis.
Lainé
Fabrice
F
Service des Maladies du Foie, CHRU Pontchaillou, Rue Henri le Guilloux-35033, Cedex, Rennes, France.
Ropert
Martine
M
Lan
Caroline Le
CL
Loréal
Olivier
O
Bellissant
Eric
E
Jard
Christine
C
Pouchard
Michel
M
Le Treut
André
A
Brissot
Pierre
P
D000328:Adult; D000368:Aged; D002570:Ceruloplasmin / Q000378:metabolism*; D003300:Copper / Q000097:blood; D005260:Female; D006432:Hemochromatosis / Q000235:genetics / Q000378:metabolism*; D006720:Homozygote; D006801:Humans; D007501:Iron / Q000097:blood; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D014168:Transferrin / Q000378:metabolism
D014168:Transferrin; D003300:Copper; D007501:Iron; D002570:Ceruloplasmin