Clinical and Biomedical Research (Apr 2017)

In vitro effect of N-acetyl-L-cysteine on glutathione and sulfhydryl levels in X-linked adrenoleukodystrophy patients

  • Desirèe Padilha Marchetti,
  • Bruna Donida,
  • Marion Deon,
  • Carlos Eduardo Jacques,
  • Laura Bannach Jardim,
  • Carmen Regla Vargas

Journal volume & issue
Vol. 37, no. 1

Abstract

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Introduction: Recent evidence shows that oxidative stress seems to be related with the pathophysiology of X-linked adrenoleukodystrophy (X-ALD), a neurodegenerative disorder. Methods: In the present study, the in vitro effect of N-acetyl-L-cysteine (NAC) on glutathione (GSH) and sulfhydryl levels in X-ALD patients was evaluated. Results: A significant reduction of GSH and sulfhydryl content was observed in X-ALD patients compared to the control group. Furthermore, 5 mM of NAC, in vitro, led to an increase in GSH content and sulfhydryl groups in these patients. Conclusion: These data probably indicate that an adjuvant therapy with the antioxidant NAC could improve the oxidative imbalance in X-ALD patients. Keywords: X- linked adrenoleukodystrophy; N-acetyl-L-cysteine; glutathione; sulfhydryl

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