Pathological overload of iron in the mitochondrial matrix has been observed in numerous diseases, including sideroblastic anemias, which have many causes, and in genetic diseases that affect iron-sulfur cluster biogenesis, heme synthesis, and mitochondrial protein translation and its products. Although high expression of the mitochondrial iron importer, mitoferrin, appears to be an underlying common feature, it is unclear what drives high mitoferrin expression and what other proteins are involved in trapping excess toxic iron in the mitochondrial matrix. Numerous examples of human diseases and model systems suggest that mitochondrial iron homeostasis is coordinated through transcriptional remodeling. A cytosolic/nuclear molecule may affect a transcriptional factor to coordinate the events that lead to iron accumulation, but no candidates for this role have yet been identified.

中文翻译：

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线粒体铁超负荷：原因和后果。

线粒体基质中铁的病理性超载已在许多疾病中观察到，其中包括具有多种原因的铁粒幼细胞性贫血，以及在影响铁硫簇生物发生，血红素合成以及线粒体蛋白质翻译及其产物的遗传疾病中。尽管线粒体铁输入物线粒体铁蛋白的高表达似乎是一个潜在的共同特征，但不清楚是什么导致线粒体铁蛋白的高表达以及哪些其他蛋白质参与了线粒体基质中过量有毒铁的捕集。人类疾病和模型系统的许多例子表明，线粒体铁稳态是通过转录重塑来协调的。胞质/核分子可能会影响转录因子以协调导致铁蓄积的事件，