Next Article in Journal
Role of Chemosensory TRP Channels in Lung Cancer
Next Article in Special Issue
Anemia and Iron Deficiency in Cancer Patients: Role of Iron Replacement Therapy
Previous Article in Journal
Creatinine-Based Renal Function Estimates and Dosage of Postoperative Pain Management for Elderly Acute Hip Fracture Patients
Previous Article in Special Issue
Mitochondrial Targeting in Neurodegeneration: A Heme Perspective
Article Menu
Issue 3 (September) cover image

Export Article

Open AccessReview
Pharmaceuticals 2018, 11(3), 89; https://doi.org/10.3390/ph11030089

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
*
Author to whom correspondence should be addressed.
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)
Full-Text   |   PDF [1311 KB, uploaded 20 September 2018]   |  

Abstract

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
Figures

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
SciFeed

Share & Cite This Article

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.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
Pharmaceuticals EISSN 1424-8247 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top