Monnier, Veronique and Vicente Llorens, Jose and Navarro, Juan Antonio (2018) Impact of Drosophila Models in the Study and Treatment of Friedreich's Ataxia. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, 19 (7): 1989. ISSN 1422-0067
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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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | FRATAXIN DEFICIENCY LEADS; ALTERED LIPID-METABOLISM; DORSAL-ROOT GANGLIA; TRIGGERS CELL-DEATH; LIFE-SPAN EXTENSION; OXIDATIVE STRESS; IRON-SULFUR; YEAST FRATAXIN; GENE-EXPRESSION; ENDOPLASMIC-RETICULUM; Drosophila melanogaster; Friedreich's ataxia; frataxin; iron; oxidative stress; metal homeostasis; lipid metabolism; endoplasmic reticulum; genetic screens; drug screens |
| Subjects: | 500 Science > 570 Life sciences 500 Science > 590 Zoological sciences |
| Divisions: | Biology, Preclinical Medicine > Institut für Zoologie > Entwicklungsbiologie (Prof. Dr. Stephan Schneuwly) |
| Depositing User: | Petra Gürster |
| Date Deposited: | 02 Jul 2020 12:08 |
| Last Modified: | 02 Jul 2020 12:08 |
| URI: | https://pred.uni-regensburg.de/id/eprint/14314 |
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