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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Detalles Bibliográficos
Autores principales: 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: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2016
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4956409/
https://www.ncbi.nlm.nih.gov/pubmed/27343351
http://dx.doi.org/10.7554/eLife.16043

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