Frataxin deficiency in proprioceptive neurons is causal to inflammatory and glial responses in dorsal root ganglia
Pauline Meriau,
Laure Weill,
Hélène Puccio
et al.
Abstract:Friedreich ataxia (FA), the most common recessive hereditary ataxia, is an early-onset neurodegenerative disease characterized by pathological changes occurring first in the peripheral dorsal root ganglia (DRG), with loss of the large sensory proprioceptive neurons, leading to ganglionopathy and proprioceptive deficits. FA is caused by a mutation in frataxin gene (Fxn), leading to reduced expression of frataxin protein (FXN), an essential ubiquitous mitochondrial protein. Most research has focused on the patho… Show more
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