Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals

Friedreich’s ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by mutations in Frataxin (FXN). Loss of FXN causes impaired mitochondrial function and iron homeostasis. An elevated production of reactive oxygen species (ROS) was previously proposed to contribute to the pathogen...

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Bibliographic Details
Published in:eLife
Main Authors: Chen, Kuchuan, Ho, Tammy Szu-Yu, Lin, Guang, Tan, Kai Li, Rasband, Matthew N, Bellen, Hugo J
Format: Artigo
Language:Inglês
Published: eLife Sciences Publications, Ltd 2016
Subjects:
Neuroscience
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC5130293/
https://ncbi.nlm.nih.gov/pubmed/27901468
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.7554/eLife.20732
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