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

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...

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Bibliographic Details
Authors: Marchetti, Desirèe Padilha, Donida, Bruna, Deon, Marion, Jacques, Carlos Eduardo Diaz, Jardim, Laura Bannach, Vargas, Carmen Regla
Format: article
Status:Published version
Publication Date:2017
Country:Brasil
Institution:Universidade Federal do Rio Grande do Sul (UFRGS)
Repository:Repositório Institucional da UFRGS
Language:English
OAI Identifier:oai:www.lume.ufrgs.br:10183/174872
Online Access:http://hdl.handle.net/10183/174872
Access Level:Open access
Keyword:N-Acetil-L-Cisteína
X- linked adrenoleukodystrophy
N-acetyl-L-cysteine
Glutathione
Sulfhydryl
Description
Summary: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.

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