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Mitochondrial iron and calcium homeostasis in Friedreich ataxia
IUBMB Life ( IF 3.7 ) Pub Date : 2021-03-05 , DOI: 10.1002/iub.2457 Jordi Tamarit 1 , Elena Britti 1 , Fabien Delaspre 1 , Marta Medina-Carbonero 1 , Arabela Sanz-Alcázar 1 , Elisa Cabiscol 1 , Joaquim Ros 1
IUBMB Life ( IF 3.7 ) Pub Date : 2021-03-05 , DOI: 10.1002/iub.2457 Jordi Tamarit 1 , Elena Britti 1 , Fabien Delaspre 1 , Marta Medina-Carbonero 1 , Arabela Sanz-Alcázar 1 , Elisa Cabiscol 1 , Joaquim Ros 1
Affiliation
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.
中文翻译:
Friedreich 共济失调中的线粒体铁和钙稳态
Friedreich Ataxia 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,许多结果也强调了钙不平衡在 frataxin 缺乏引起的有害下游影响中的重要性。在这篇综述中,我们收集了这两种金属的作用,以全面了解铁和钙的动态平衡如何影响细胞功能,以及因此可以采取哪些策略来找到治疗该疾病的有效疗法。
更新日期:2021-03-15
中文翻译:
Friedreich 共济失调中的线粒体铁和钙稳态
Friedreich Ataxia 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,许多结果也强调了钙不平衡在 frataxin 缺乏引起的有害下游影响中的重要性。在这篇综述中,我们收集了这两种金属的作用,以全面了解铁和钙的动态平衡如何影响细胞功能,以及因此可以采取哪些策略来找到治疗该疾病的有效疗法。