Paraoxonase-1 status in patients with hereditary hemochromatosis

Nicola Martinelli, Anabel García-Heredia, Helena Roca, Núria Aranda, Victoria Arija, Bharti Mackness, Michael Mackness, Fabiana Busti, Gerard Aragonés, Juan Pedro-Botet, Federica Pedica, Ivana Cataldo, Judit Marsillach, Jorge Joven, Domenico Girelli, Jordi Camps

  • 11 Citations

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.

Original languageEnglish
Pages (from-to)1484-1492
Number of pages9
JournalJournal of Lipid Research
Volume54
Issue number5
DOIs
StatePublished - May 2013
Externally publishedYes

Fingerprint

Aryldialkylphosphatase
Hemochromatosis
Liver
Serum
Iron
Oxidative Stress
Proteins
Isoprostanes
Lipid Peroxides
Ferritins
Case-Control Studies
Hepatocytes
Antioxidants
Staining and Labeling
Biopsy
Enzymes
Genes

Keywords

  • Antioxidants
  • Inflammation
  • Iron metabolism
  • Oxidative stress

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Endocrinology

Cite this

Martinelli, N., García-Heredia, A., Roca, H., Aranda, N., Arija, V., Mackness, B., ... Camps, J. (2013). Paraoxonase-1 status in patients with hereditary hemochromatosis. Journal of Lipid Research, 54(5), 1484-1492. DOI: 10.1194/jlr.P028977

Paraoxonase-1 status in patients with hereditary hemochromatosis. / Martinelli, Nicola; García-Heredia, Anabel; Roca, Helena; Aranda, Núria; Arija, Victoria; Mackness, Bharti; Mackness, Michael; Busti, Fabiana; Aragonés, Gerard; Pedro-Botet, Juan; Pedica, Federica; Cataldo, Ivana; Marsillach, Judit; Joven, Jorge; Girelli, Domenico; Camps, Jordi.

In: Journal of Lipid Research, Vol. 54, No. 5, 05.2013, p. 1484-1492.

Research output: Contribution to journalArticle

Martinelli, N, García-Heredia, A, Roca, H, Aranda, N, Arija, V, Mackness, B, Mackness, M, Busti, F, Aragonés, G, Pedro-Botet, J, Pedica, F, Cataldo, I, Marsillach, J, Joven, J, Girelli, D & Camps, J 2013, 'Paraoxonase-1 status in patients with hereditary hemochromatosis' Journal of Lipid Research, vol 54, no. 5, pp. 1484-1492. DOI: 10.1194/jlr.P028977
Martinelli N, García-Heredia A, Roca H, Aranda N, Arija V, Mackness B et al. Paraoxonase-1 status in patients with hereditary hemochromatosis. Journal of Lipid Research. 2013 May;54(5):1484-1492. Available from, DOI: 10.1194/jlr.P028977

Martinelli, Nicola; García-Heredia, Anabel; Roca, Helena; Aranda, Núria; Arija, Victoria; Mackness, Bharti; Mackness, Michael; Busti, Fabiana; Aragonés, Gerard; Pedro-Botet, Juan; Pedica, Federica; Cataldo, Ivana; Marsillach, Judit; Joven, Jorge; Girelli, Domenico; Camps, Jordi / Paraoxonase-1 status in patients with hereditary hemochromatosis.

In: Journal of Lipid Research, Vol. 54, No. 5, 05.2013, p. 1484-1492.

Research output: Contribution to journalArticle

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AU - Martinelli,Nicola

AU - García-Heredia,Anabel

AU - Roca,Helena

AU - Aranda,Núria

AU - Arija,Victoria

AU - Mackness,Bharti

AU - Mackness,Michael

AU - Busti,Fabiana

AU - Aragonés,Gerard

AU - Pedro-Botet,Juan

AU - Pedica,Federica

AU - Cataldo,Ivana

AU - Marsillach,Judit

AU - Joven,Jorge

AU - Girelli,Domenico

AU - Camps,Jordi

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N2 - 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.

AB - 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.

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