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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Gepubliceerd in:eLife
Hoofdauteurs: 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
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: eLife Sciences Publications, Ltd 2016
Onderwerpen:
Neuroscience
Online toegang: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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