Article
Glutathione imbalance in patients with X-linked adrenoleukodystrophy.
Molecular genetics and metabolism - 1 Aug 2013
Petrillo Sara, Piemonte Fiorella, Pastore Anna, Tozzi Giulia, Aiello Chiara, Pujol Aurora, Cappa Marco, Bertini Enrico
Abstract excerpt
BACKGROUND: X-linked adrenoleukodystrophy (X-ALD) is a genetic disorder of X-linked inheritance caused by a mutation in the ABCD1 gene which determines an accumulation of long-chain fatty acids in plasma and tissues. Recent evidence shows that oxidative stress may be a hallmark in the pathogenesis of X-ALD and glutathione plays an important role in the defense against free radicals. In this study we have analyzed...
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