Mitochondrial fatty acid β-oxidation disorders (FAODs) are a group of about 20 diseases which are caused by specific mutations in genes that codify proteins or enzymes involved in the fatty acid transport and mitochondrial β-oxidation. As a consequence of these inherited metabolic defects, fatty acids can not be used as an appropriate energetic source during special conditions, such as prolonged fasting, exercise or other catabolic states. Therefore, patients usually present hepatopathy, cardiomyopathy, severe skeletal myopathy and neuropathy, besides biochemical features like hypoketotic hypoglycemia, metabolic acidosis, hypotony and hyperammonemia. This set of symptoms seems to be related not only with the energy deficiency, but also with toxic effects provoked by fatty acids and carnitine derivatives accumulated in the tissues of the patients. The understanding of the mechanisms by which these metabolites provoke tissue injury in FAODs is crucial for the developmental of novel therapeutic strategies that promote increased life expectancy, as well as improved life quality for patients. In this sense, the objective of this review is to present evidence from the scientific literature on the role of oxidative damage and mitochondrial dysfunction in the pathogenesis of the most prevalent FAODs: medium-chain acyl-CoA dehydrogenase (MCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and very long-chain acyl-CoA dehydrogenase (VLCAD) deficiencies. It is expected that the findings presented in this review, obtained from both animal model and patients studies, may contribute to a better comprehension of the pathophysiology of these diseases.

线粒体脂肪酸 β-氧化障碍 (FAOD) 是一组约 20 种疾病，由编码参与脂肪酸转运和线粒体 β-氧化的蛋白质或酶的基因的特定突变引起。由于这些遗传性代谢缺陷，脂肪酸不能在特殊条件下用作适当的能量来源，例如长时间禁食、运动或其他分解代谢状态。因此，除了低酮性低血糖、代谢性酸中毒、低血压和高氨血症等生化特征外，患者通常会出现肝病、心肌病、严重骨骼肌病和神经病。这组症状似乎不仅与能量缺乏有关，还与积累在患者组织中的脂肪酸和肉碱衍生物引起的毒性作用有关。了解这些代谢物在FAODs中引起组织损伤的机制对于开发新的治疗策略至关重要，这些策略可促进延长预期寿命以及改善患者的生活质量。从这个意义上说，本综述的目的是从科学文献中提供关于氧化损伤和线粒体功能障碍在最普遍的FAOD发病机制中的作用的证据：中链酰基辅酶A脱氢酶（MCAD），长链3 -羟酰基辅酶A脱氢酶（LCHAD）和超长链酰基辅酶A脱氢酶（VLCAD）缺陷。预计本综述中提出的从动物模型和患者研究中获得的发现可能有助于更好地理解这些疾病的病理生理学。