Hereditary hemochromatosis (HH) is characterized by accumulation of iron, oxidative stress, infl ammation, and fi brogenesis in liver tissue. In this setting, research on the protection afforded by intracellular antioxidants is of clinical relevance. Paraoxonase-1 (PON1) is an enzyme that degrades lipid peroxides. This study investigates the alterations in serum PON1 status, PON1 gene polymorphisms, and PON1 hepatic expression in patients with HH. We performed a case-control study in 77 patients with HH (80.5% men, 22-70 years of age) and 408 healthy individuals (43.1% men, 26-74 years of age). Serum PON1 activities against different substrates and PON1 192 and PON1 55 polymorphisms were analyzed. PON1 protein expression was investigated in 20 liver biopsies. HH patients had signifi cantly lower serum PON1 activity, which was inversely correlated with ferritin (marker of iron stores) and serum 8-isoprostane concentrations (index of oxidative stress). PON1 protein expression in liver tissue was higher in patients and showed stronger staining in hepatocytes surrounding the areas of infl ammation. Our study provides preliminary evidence that PON1 may play a role in protecting against iron-induced oxi dative stress in hereditary hemochromatosis. Copyright © 2013 by the American Society for Biochemistry and Molecular Biology, Inc.
Paraoxonase-1 status in patients with hereditary hemochromatosis / Martinelli, N.; Garcia-Heredia, A.; Roca, H.; Aranda, N.; Arija, V.; Mackness, B.; Mackness, M.; Busti, F.; Aragones, G.; Pedro-Botet, J.; Pedica, F.; Cataldo, I.; Marsillach, J.; Joven, J.; Girelli, D.; Camps, J.. - In: JOURNAL OF LIPID RESEARCH. - ISSN 0022-2275. - 54:5(2013), pp. 1484-1492. [10.1194/jlr.P028977]
Paraoxonase-1 status in patients with hereditary hemochromatosis
Pedica F.;
2013-01-01
Abstract
Hereditary hemochromatosis (HH) is characterized by accumulation of iron, oxidative stress, infl ammation, and fi brogenesis in liver tissue. In this setting, research on the protection afforded by intracellular antioxidants is of clinical relevance. Paraoxonase-1 (PON1) is an enzyme that degrades lipid peroxides. This study investigates the alterations in serum PON1 status, PON1 gene polymorphisms, and PON1 hepatic expression in patients with HH. We performed a case-control study in 77 patients with HH (80.5% men, 22-70 years of age) and 408 healthy individuals (43.1% men, 26-74 years of age). Serum PON1 activities against different substrates and PON1 192 and PON1 55 polymorphisms were analyzed. PON1 protein expression was investigated in 20 liver biopsies. HH patients had signifi cantly lower serum PON1 activity, which was inversely correlated with ferritin (marker of iron stores) and serum 8-isoprostane concentrations (index of oxidative stress). PON1 protein expression in liver tissue was higher in patients and showed stronger staining in hepatocytes surrounding the areas of infl ammation. Our study provides preliminary evidence that PON1 may play a role in protecting against iron-induced oxi dative stress in hereditary hemochromatosis. Copyright © 2013 by the American Society for Biochemistry and Molecular Biology, Inc.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
