Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease.
Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease.
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DOI：
10.1371/journal.pone.0159209
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发表时间：
2016
期刊：
影响因子：
3.7
通讯作者：
Moltó MD
中科院分区：
文献类型：
--
作者：
Soriano S;Calap-Quintana P;Llorens JV;Al-Ramahi I;Gutiérrez L;Martínez-Sebastián MJ;Botas J;Moltó MD
Friedreich’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.
