Mitochondrial diseases affect one in 2,000 individuals; they can present at any age and they can manifest in any organ. How defects in mitochondria can cause such a diverse range of human diseases remains poorly understood. Insight into this diversity is emerging from recent research that investigated defects in mitochondrial protein synthesis and mitochondrial DNA maintenance, which showed that many cell-specific stress responses are induced in response to mitochondrial dysfunction. Studying the molecular regulation of these stress responses might increase our understanding of the pathogenesis and variability of human mitochondrial diseases.

中文翻译：

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线粒体疾病：细胞器应激反应对病理的贡献

线粒体疾病影响每2,000个人中的一位；它们可以出现在任何年龄，也可以出现在任何器官中。线粒体的缺陷如何导致如此广泛的人类疾病仍然知之甚少。从最近研究线粒体蛋白质合成和线粒体DNA维持缺陷的研究中可以洞悉这种多样性，这表明许多细胞特异性应激反应是响应线粒体功能障碍而引起的。研究这些应激反应的分子调控可能会增加我们对人类线粒体疾病的发病机理和变异性的了解。