Objectives. Hereditary hemochromatosis is the most common congenital metabolic disease in the Caucasian population. One of the mechanisms by which tissue damage occurs in these patients is increased oxidative stress. Ceruloplasmin belongs to the most important extracellular antioxidants.

Material and methods. We investigated ceruloplasmin and its enzyme activity in patients with hemochromatosis, who were divided into homozygotes for the C282Y point mutation and heterozygotes (allele with the C282Y mutation/1 other allele).

Results. In patients with hemochromatosis, we found a significant decrease in ferrooxidase activity of ceruloplasmin, which was more pronounced in homozygotes than in heterozygotes. Ferrooxidase activity of ceruloplasmin correlated better with changes in copper levels (correl.coeff. 0,58) than with apoceruloplasmin levels (correl. coeff. 0.48).

Conclusion. Decreased ceruloplasmin activity in patients with hemochromatosis may exacerbate oxidative stress and potentiate hepatocyte damage. One of the possible explanations for the decrease in the enzymatic activity of ceruloplasmin could be a disorder in the incorporation of copper atoms into the apoceruloplasmin molecule (Tab. 2, Fig. 4, Ref. 17). Text in PDF www.lekarskyobzor.sk.

KEY WORDS: haemochromatosis, ceruloplasmin, ferrooxidase, oxidative stress, alpha-2-macroglobulin.

Lek Obz 2022, 71 (11): 438-442

Ladislav TURECKÝ 1, Viera KUPČOVÁ 2, Monika ĎURFINOVÁ 1, Milada LAŠŠÁNOVÁ 1, Eva UHLÍKOVÁ 1

1 Ústav lekárskej chémie, biochémie a klinickej biochémie LF UK v Bratislave, prednosta prof. MUDr. L. Turecký, CSc.
2 III. interná klinika LF UK v Bratislave, prednosta doc. MUDr. V. Mojto, CSc., MPH

CITE
TURECKÝ L., KUPČOVÁ V., URFINOVÁ M. et al.: Ceruloplasmin and its ferrooxidase activity in sera of patients with hereditary haemochromatosis. Lek Obz 2022, 71 (11): 438-442