Raffaella Gozzelino, and Paolo Arosio; Review: Int. J. Mol. Sci. 2016, 17(1), 130; doi:10.3390/ijms17010130
OPEN ACESS
It is established the involvement of this metal in Friedrich’s ataxia, a
disease characterized by progressive degeneration of sensory neurons in
the dorsal root ganglia caused by a mutation in the frataxin gene.
Frataxin plays an essential role in delivering Fe to mitochondrial
pathways involved in ISC biogenesis and when its functionality is reduced below a critical threshold, Fe accumulates in mitochondria.
Reduced mitochondrial functioning and impaired electron transport chain
results in Fe-mediated ROS, which was shown to positively correlate
with the severity of Friedrich’s ataxia.
Thursday, March 31, 2016
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