Loss of homeostatic functions in microglia from a murine model of Friedreich's ataxia

Ilaria Della Valle, Martina Milani, Simona Rossi, Riccardo Turchi, Flavia Tortolici, Valentina Nesci, Alberto Ferri, Cristiana Valle, Daniele Lettieri-Barbato, Katia Aquilano, Mauro Cozzolino, Savina Apolloni, Nadia D'Ambrosi, Loss of homeostatic functions in microglia from a murine model of Friedreich's ataxia, Genes & Diseases, 2023, 101178, doi:10.1016/j.gendis.2023.101178. 

 Although several reports correlated microglia morphology to cerebellar degeneration in FRDA in vivo, this work is the first to provide multilayer evidence (phenomics, transcriptomics, and metabolic analysis) that FRDA microglia are dysfunctional, suggesting a contribution of non-cell autonomous mechanisms in FRDA pathogenesis.