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Int. J. Mol. Sci. 2018, 19(7), 1989; https://doi.org/10.3390/ijms19071989

Impact of Drosophila Models in the Study and Treatment of Friedreich’s Ataxia

1
Unité de Biologie Fonctionnelle et Adaptative (BFA), Sorbonne Paris Cité, Université Paris Diderot, UMR8251 CNRS, 75013 Paris, France
2
Department of Genetics, University of Valencia, Campus of Burjassot, 96100 Valencia, Spain
3
Lehrstuhl für Entwicklungsbiologie, Universität Regensburg, 93040 Regensburg, Germany
These authors contributed equally to this work.
*
Authors to whom correspondence should be addressed.
Received: 28 May 2018 / Revised: 26 June 2018 / Accepted: 3 July 2018 / Published: 7 July 2018
(This article belongs to the Special Issue Drosophila Model and Human Disease)
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Abstract

Drosophila melanogaster has been for over a century the model of choice of several neurobiologists to decipher the formation and development of the nervous system as well as to mirror the pathophysiological conditions of many human neurodegenerative diseases. The rare disease Friedreich’s ataxia (FRDA) is not an exception. Since the isolation of the responsible gene more than two decades ago, the analysis of the fly orthologue has proven to be an excellent avenue to understand the development and progression of the disease, to unravel pivotal mechanisms underpinning the pathology and to identify genes and molecules that might well be either disease biomarkers or promising targets for therapeutic interventions. In this review, we aim to summarize the collection of findings provided by the Drosophila models but also to go one step beyond and propose the implications of these discoveries for the study and cure of this disorder. We will present the physiological, cellular and molecular phenotypes described in the fly, highlighting those that have given insight into the pathology and we will show how the ability of Drosophila to perform genetic and pharmacological screens has provided valuable information that is not easily within reach of other cellular or mammalian models. View Full-Text
Keywords: Drosophila melanogaster; Friedreich’s ataxia; frataxin; iron; oxidative stress; metal homeostasis; lipid metabolism; endoplasmic reticulum; genetic screens; drug screens Drosophila melanogaster; Friedreich’s ataxia; frataxin; iron; oxidative stress; metal homeostasis; lipid metabolism; endoplasmic reticulum; genetic screens; drug screens
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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).
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Monnier, V.; Llorens, J.V.; Navarro, J.A. Impact of Drosophila Models in the Study and Treatment of Friedreich’s Ataxia. Int. J. Mol. Sci. 2018, 19, 1989.

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