As many other neurodegenerative diseases, ARSACS is also a matter of inflammation. Recent researches clarified that high levels of Sacsin protein are present in astrocytes and microglia, the cell populations of the brain that act like principal mediators of neuroinflammation. Our lab also discovered that these processes could be involved in retinal defective development and degeneration observed in ARSACS mutant zebrafish. In this project, we will study the possible role of neuroinflammation in cerebellar degeneration, using a new ARSACS zebrafish model in which we will be monitor the interactions between Purkinje cells and microglia in the pathological condition. In addition, we will also study the characteristics of human mutant microglial cells, generated from stem cells directly derived from ARSACS patients, searching for a possible link with the human disease. The results of our experiments will put the base for new therapeutic approaches, that will be screened in our fish model to open new avenues for treatment of this rare neurodegenerative disease.
Financement: 100 000$
Durée : un an
Dr Devid Damiani
Via dei Giacinti 2, 56128
Calambrone (PI)
Italy
Coordonnées: devid.damiani@gmail.com