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...

Descripción completa

Detalles Bibliográficos
Autores: Marchetti, Desirèe Padilha, Donida, Bruna, Deon, Marion, Jacques, Carlos Eduardo, Jardim, Laura Bannach, Vargas, Carmen Regla
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2017
País:Brasil
Institución:Universidade Federal do Rio Grande do Sul (UFRGS)
Repositorio:Clinical and Biomedical Research
Idioma:inglés
OAI Identifier:oai:seer.ufrgs.br:article/69868
Acceso en línea:https://seer.ufrgs.br/index.php/hcpa/article/view/69868
Access Level:acceso abierto
Palabra clave:X- linked adrenoleukodystrophy
N-acetyl-L-cysteine
glutathione
sulfhydryl
Descripción
Sumario: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