Table 1.

Evidence for the involvement of neuroinflammation in Friedreich’s ataxia.

FRDA Model	Neuroinflammatory Features	References
Patients	Increased glial activation in cerebellum and brainstem	[34]
	Increased ferritin signals in cerebellar microglia and astrocytes	[35]
	Microglia with enlarged perikarya and thicker processes	[36]
	Astroglia intrusion into dorsal roots	[37]
	Hypertrophic cerebellar microglia positive for SOD1 enzyme	[38]
	Increased GFAP plasma levels	[39]
KIKO mice	Increased cerebellar microgliosis and astrocytosis after LPS stimulation; increased oxidative damage and DNA repair proteins	[40]
	Increased cerebellar COX2	[41]
YG8R mice	Increased cerebellar microglial activation after LPS; increased COX2	[41]
	Increased satellite cell proliferation, astrocytosis and influx of OX42 positive cells in the spinal cord and cerebellum	[42]
FGKO mice	Severe ataxia after frataxin deletion in astrocytes during development	[43]
Microglial cell lines	Increased DNA damage after frataxin knockdown	[40]
Mouse primary astrocytes	Increased ROS production after frataxin knockdown	[44]
Human astrocytes in vitro	Impaired mitochondrial activity and superoxide formation; increased release of inflammatory molecules and toxicity for neurons after frataxin knockdown	[45,46]
iPSC-derived YG8R astrocytes	Reduced aconitase and DNA repair enzymes; increased sensitivity to oxidative stress	[45]
Schwann cells in vitro	Decreased proliferation and increased inflammatory genes after frataxin knockdown	[11]
Drosophila melanogaster	Locomotor dysfunction, brain degeneration and lipid metabolism defects after frataxin knockdown in glia	[47,48]