Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

Mutations in Frataxin (FXN) cause Friedreich’s ataxia (FRDA), a recessive neurodegenerative disorder. Previous studies have proposed that loss of FXN causes mitochondrial dysfunction, which triggers elevated reactive oxygen species (ROS) and leads to the demise of neurons. Here we describe a ROS ind...

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Detalhes bibliográficos
Publicado no:eLife
Main Authors: Chen, Kuchuan, Lin, Guang, Haelterman, Nele A, Ho, Tammy Szu-Yu, Li, Tongchao, Li, Zhihong, Duraine, Lita, Graham, Brett H, Jaiswal, Manish, Yamamoto, Shinya, Rasband, Matthew N, Bellen, Hugo J
Formato: Artigo
Idioma:Inglês
Publicado em: eLife Sciences Publications, Ltd 2016
Assuntos:
Neuroscience
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4956409/
https://ncbi.nlm.nih.gov/pubmed/27343351
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.7554/eLife.16043
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