期刊
IUBMB LIFE
卷 73, 期 3, 页码 543-553出版社
WILEY
DOI: 10.1002/iub.2457
关键词
mitochondrial disorders; Friedreich ataxia; iron; calcium
This review discusses how iron and calcium dyshomeostasis impacts cellular functions, and suggests strategies for finding an effective therapy for Friedreich Ataxia, a neuro-cardiodegenerative disease caused by frataxin deficiency.
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.
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