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- Title
Neuroinflammation in Friedreich's Ataxia.
- Authors
Apolloni, Savina; Milani, Martina; D'Ambrosi, Nadia
- Abstract
Friedreich's ataxia (FRDA) is a rare genetic disorder caused by mutations in the gene frataxin, encoding for a mitochondrial protein involved in iron handling and in the biogenesis of iron−sulphur clusters, and leading to progressive nervous system damage. Although the overt manifestations of FRDA in the nervous system are mainly observed in the neurons, alterations in non-neuronal cells may also contribute to the pathogenesis of the disease, as recently suggested for other neurodegenerative disorders. In FRDA, the involvement of glial cells can be ascribed to direct effects caused by frataxin loss, eliciting different aberrant mechanisms. Iron accumulation, mitochondria dysfunction, and reactive species overproduction, mechanisms identified as etiopathogenic in neurons in FRDA, can similarly affect glial cells, leading them to assume phenotypes that can concur to and exacerbate neuron loss. Recent findings obtained in FRDA patients and cellular and animal models of the disease have suggested that neuroinflammation can accompany and contribute to the neuropathology. In this review article, we discuss evidence about the involvement of neuroinflammatory-related mechanisms in models of FRDA and provide clues for the modulation of glial-related mechanisms as a possible strategy to improve disease features.
- Subjects
FRIEDREICH'S ataxia; NEUROINFLAMMATION; NEUROGLIA; NERVOUS system; FRATAXIN
- Publication
International Journal of Molecular Sciences, 2022, Vol 23, Issue 11, p6297
- ISSN
1661-6596
- Publication type
Article
- DOI
10.3390/ijms23116297