Tamarit Jordi, Britti Elena, Delaspre Fabien, Medina-Carbonero Marta, Sanz-Alcázar Arabela, Cabiscol Elisa, Ros Joaquim
Dept. Ciències Mèdiques Bàsiques, Universitat de Lleida, IRBLleida, Lleida, Spain.
IUBMB Life. 2021 Mar;73(3):543-553. doi: 10.1002/iub.2457. Epub 2021 Mar 5.
Friedreich Ataxia is a neuro-cardiodegenerative disease caused by the deficiency of frataxin, a mitochondrial protein. Many evidences indicate that frataxin deficiency causes an unbalance of iron homeostasis. Nevertheless, in the last decade many results also highlighted the importance of calcium unbalance in the deleterious downstream effects caused by frataxin deficiency. In this review, the role of these two metals has been gathered to give a whole view of how iron and calcium dyshomeostasys impacts on cellular functions and, as a result, which strategies can be followed to find an effective therapy for the disease.
弗里德赖希共济失调是一种由线粒体蛋白frataxin缺乏引起的神经心脏退行性疾病。许多证据表明，frataxin缺乏会导致铁稳态失衡。然而，在过去十年中，许多研究结果也强调了钙失衡在frataxin缺乏引起的有害下游效应中的重要性。在这篇综述中，我们总结了这两种金属的作用，以全面了解铁和钙的动态平衡失调如何影响细胞功能，以及由此可以采取哪些策略来寻找针对该疾病的有效治疗方法。
