4.4 Article

Fatty liver and anti-oxidant enzyme activities along with peroxisome proliferator-activated receptors γ and α expressions in the liver of Wilson's disease

Journal

MOLECULAR GENETICS AND METABOLISM
Volume 107, Issue 3, Pages 542-547

Publisher

ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ymgme.2012.08.004

Keywords

Wilson's disease; Peroxisome proliferator-activated receptors; Catalase; Superoxide dismutase; Steatosis

Funding

  1. Grants-in-Aid for Scientific Research [23590689] Funding Source: KAKEN

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Background: The mechanisms of liver damage and steatosis in Wilson's disease (WD) presenting accumulation of copper generating oxidants remain unclear. Recent studies have shown that peroxisome proliferator-activated receptors (PPARs), in particular PPARs alpha and gamma, regulate fat content of the liver together with the anti-oxidant and anti-inflammation systems. However, such PPARs have never been studied in WD. Methods: We examined PPARs along with the liver damage and steatosis of WD using liver specimens from affected patients exhibiting mild liver damage (group I, n=5), moderate or greater liver damage (group II, n=10) and fulminant hepatic failure (group III, n=5), and from asymptomatic carriers (group H, n=4). Results: PPAR alpha expression was increased over the control levels in groups H and I but was decreased in groups II and III in parallel with the progression of liver damage (group H=I>II>III). PPAR gamma expression was inversely increased (group H

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