Oxidative stress and pro-apoptotic conditions in a rodent model of Wilson's disease

Alberta Samuele, Anna Mangiagalli, Marie Thérèse Armentero, Roberto Fancellu, Eleonora Bazzini, Mariapia Vairetti, Andrea Ferrigno, Plinio Richelmi, Giuseppe Nappi, Fabio Blandini

Research output: Contribution to journalArticlepeer-review


Wilson's disease (WD) is an inherited disorder, characterized by selective copper deposition in liver and brain, chronic hepatitis and extra-pyramidal signs. In this study, we investigated changes of biochemical markers of oxidative stress and apoptosis in liver, striatum and cerebral cortex homogenates from Long-Evans Cinnamon (LEC) rats, a mutant strain isolated from Long Evans (LE) rats, in whom spontaneous hepatitis develops shortly after birth. LEC and control (LE) rats at 11 and 14 weeks of age were used. We determined tissue levels of glutathione (GSH/GSSG ratio), lipid peroxides, protein-thiols (P-SH), nitric oxide metabolites, activities of caspase-3 and total superoxide-dismutase (SOD), striatal levels of monoamines and serum levels of hepatic amino-transferases. We observed a decrease of protein-thiols, GSH/GSSG ratio and nitrogen species associated to increased lipid peroxidation in the liver and striatum - but not in the cerebral cortex - of LEC rats, accompanied by dramatic increase in serum amino-transferases and decrease of striatal catecholamines. Conversely, SOD and caspase-3 activity increased consistently only in the cortex of LEC rats. Hence, we assume that enhanced oxidative stress may play a central role in the cell degeneration in WD, at the main sites of copper deposition, with discrete pro-apoptotic conditions developing in distal areas.

Original languageEnglish
Pages (from-to)325-330
Number of pages6
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Issue number3
Publication statusPublished - Sep 25 2005


  • Apoptosis
  • Cell degeneration
  • Copper-toxicosis
  • Long Evans Cinnamon
  • Peroxidation

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine
  • Biophysics


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