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Metal homeostasis regulators suppress FRDA phenotypes in a drosophila model of the disease

AuthorsSoriano, Sirena; Calap-Quintana, Pablo; Llorens, José V. ; Al-Ramahi, Ismael; Gutiérrez, Lucía ; Martínez-Sebastián, María José; Botas, Juan; Moltó, María Dolores
Issue Date19-Jul-2016
PublisherPublic Library of Science
CitationPLoS ONE 11(7): e0159209 (2016)
AbstractFriedreich's ataxia (FRDA), the most commonly inherited ataxia in populations of European origin, is a neurodegenerative disorder caused by a decrease in frataxin levels. One of the hallmarks of the disease is the accumulation of iron in several tissues including the brain, and frataxin has been proposed to play a key role in iron homeostasis. We found that the levels of zinc, copper, manganese and aluminum were also increased in a Drosophila model of FRDA, and that copper and zinc chelation improve their impaired motor performance. By means of a candidate genetic screen, we identified that genes implicated in iron, zinc and copper transport and metal detoxification can restore frataxin deficiency-induced phenotypes. Taken together, these results demonstrate that the metal dysregulation in FRDA includes other metals besides iron, therefore providing a new set of potential therapeutic targets.
Publisher version (URL)https://doi.org/10.1371/journal.pone.0159209
Identifiersdoi: 10.1371/journal.pone.0159209
e-issn: 1932-6203
Appears in Collections:(ICMM) Artículos
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