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Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?

Departament de Ciències Mèdiques Bàsiques, IRBLleida, Universitat de Lleida, 25198 Lleida, Spain
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Pharmaceuticals 2018, 11(3), 89; https://0-doi-org.brum.beds.ac.uk/10.3390/ph11030089
Received: 10 August 2018 / Revised: 15 September 2018 / Accepted: 17 September 2018 / Published: 19 September 2018
(This article belongs to the Special Issue Iron as Therapeutic Targets in Human Diseases)
Friedreich ataxia is a neurodegenerative disease with an autosomal recessive inheritance. In most patients, the disease is caused by the presence of trinucleotide GAA expansions in the first intron of the frataxin gene. These expansions cause the decreased expression of this mitochondrial protein. Many evidences indicate that frataxin deficiency causes the deregulation of cellular iron homeostasis. In this review, we will discuss several hypotheses proposed for frataxin function, their caveats, and how they could provide an explanation for the deregulation of iron homeostasis found in frataxin-deficient cells. We will also focus on the potential mechanisms causing cellular dysfunction in Friedreich Ataxia and on the potential use of the iron chelator deferiprone as a therapeutic agent for this disease. View Full-Text
Keywords: Iron-sulfur; Friedreich Ataxia; Oxidative stress; Iron chelators Iron-sulfur; Friedreich Ataxia; Oxidative stress; Iron chelators
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MDPI and ACS Style

Alsina, D.; Purroy, R.; Ros, J.; Tamarit, J. Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon? Pharmaceuticals 2018, 11, 89. https://0-doi-org.brum.beds.ac.uk/10.3390/ph11030089

AMA Style

Alsina D, Purroy R, Ros J, Tamarit J. Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon? Pharmaceuticals. 2018; 11(3):89. https://0-doi-org.brum.beds.ac.uk/10.3390/ph11030089

Chicago/Turabian Style

Alsina, David, Rosa Purroy, Joaquim Ros, and Jordi Tamarit. 2018. "Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?" Pharmaceuticals 11, no. 3: 89. https://0-doi-org.brum.beds.ac.uk/10.3390/ph11030089

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