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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...
Autores principales: | Chen, Kuchuan, Ho, Tammy Szu-Yu, Lin, Guang, Tan, Kai Li, Rasband, Matthew N, Bellen, Hugo J |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
eLife Sciences Publications, Ltd
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5130293/ https://www.ncbi.nlm.nih.gov/pubmed/27901468 http://dx.doi.org/10.7554/eLife.20732 |
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