Friedreich´s ataxia is the commonest autosomal recessive ataxia among population of European descent. Despite the huge advances performed in the last decades, a cure still remains elusive. One of the most studied hallmarks of the disease is the increased production of oxidative stress markers in patients and models. This feature has been the motivation to develop treatments that aim to counteract such boost of free radicals and to enhance the production of antioxidant defenses. In this work, we present and critically review those “antioxidant” drugs that went beyond the disease´s models and were approved for its application in clinical trials. The evaluation of these trials highlights some crucial aspects of the FRDA research. On the one hand, the analysis contributes to elucidate whether oxidative stress plays a central role or whether it is only an epiphenomenon. On the other hand, it comments on some limitations in the current trials that complicate the analysis and interpretation of their outcome. We also include some suggestions that will be interesting to implement in future studies and clinical trials.

中文翻译：

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弗里德赖希共济失调的抗氧化疗法和氧化应激：正确的道路还是只是一种消遣？

弗里德赖希共济失调是欧洲血统人群中最常见的常染色体隐性共济失调。尽管过去几十年取得了巨大进步，但治愈方法仍然难以实现。该疾病研究最多的特征之一是患者和模型中氧化应激标记物的产生增加。这一特征一直是开发治疗方法的动力，旨在抵消自由基的增加并增强抗氧化防御的产生。在这项工作中，我们提出并严格审查了那些超越疾病模型并被批准在临床试验中应用的“抗氧化”药物。这些试验的评估突出了 FRDA 研究的一些关键方面。一方面，该分析有助于阐明氧化应激是否发挥核心作用，或者是否只是一种附带现象。另一方面，它评论了当前试验中的一些局限性，这些局限性使结果的分析和解释变得复杂。我们还提出了一些在未来的研究和临床试验中实施的有趣建议。