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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Publicado en:eLife
Autores principales: Chen, Kuchuan, Ho, Tammy Szu-Yu, Lin, Guang, Tan, Kai Li, Rasband, Matthew N, Bellen, Hugo J
Formato: Artigo
Lenguaje:Inglês
Publicado: eLife Sciences Publications, Ltd 2016
Materias:
Neuroscience
Acceso en línea: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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