Protective effect of n-acetyl-L-cysteine and rosuvastatin against oxidative stress in fibroblasts from asymptomatic patients with X-ALD: a preliminary study

Authors

  • Desiree Marchetti Programa de Pós-Graduação em Ciências Biológicas, Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre
  • Graziela S Ribas Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre
  • Bruna Donida Programa de Pós-Graduação em Ciências Biológicas, Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre
  • Verônica Brito Universidade Federal de Ciências de Saúde de Porto Alegre, UFCSPA
  • Dinara J Moura Universidade Federal de Ciências de Saúde de Porto Alegre, UFCSPA
  • Daniella M Coelho Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre
  • Andryele Z Machado Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre
  • Rejane G Kessler Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre
  • Adriana S Coitinho Departamento de Microbiologia, Imunologia e Parasitologia, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. Programa de Pós-Graduação em Farmacologia e Terapêutica, Universidade Federal do Rio Grande do Sul, Instituto de Ciências Básicas e da Saúde, Porto Alegre, RS, Brazil.
  • Carmen R Vargasa Programa de Pós-Graduação em Ciências Biológicas, Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

Keywords:

X-linked adrenoleukodistrophy, fibroblasts, n-acetyl-L-cysteine, rosuvastatin, oxidative stress

Abstract

Introduction: Several studies in the literature have evaluated the role of oxidative stress and adjuvant therapies for X-linked adrenoleukodystrophy (X-ALD). Here, we investigated whether n-acetyl-L-cysteine (NAC) and rosuvastatin (RSV) could influence the generation of reactive species, redox status and nitrative stress in fibroblasts from asymptomatic patients with X-ALD.

Methods: Skin biopsy samples were cultured and treated for 2 hours (37 °C) with NAC and RSV.

Results: X-ALD fibroblasts generated high levels of reactive oxygen species. These levels were significantly lower in fibroblasts treated with NAC and RSV relative to untreated samples. The X-ALD fibroblasts from asymptomatic patients also had higher catalase activity, and only NAC was able to increase enzyme activity in the samples.

Conclusion: Our results indicated that NAC and RSV were able to improve oxidative stress parameters in fibroblasts from asymptomatic patients with X-ALD, showing that adjuvant antioxidant therapy may be a promising treatment strategy for asymptomatic patients with this disease.

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Published

2021-06-28

How to Cite

1.
Marchetti D, Ribas GS, Donida B, Brito V, Moura DJ, Coelho DM, Machado AZ, Kessler RG, Coitinho AS, Vargasa CR. Protective effect of n-acetyl-L-cysteine and rosuvastatin against oxidative stress in fibroblasts from asymptomatic patients with X-ALD: a preliminary study. Clin Biomed Res [Internet]. 2021 Jun. 28 [cited 2025 Jun. 24];41(1). Available from: https://seer.ufrgs.br/index.php/hcpa/article/view/106453

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