iPSc
Causes de la SLA et mécanismes de la dégénérescence motoneuronale
Domaine de recherche principal:
Neurological and psychiatric diseases
Mots clefs:
Amyotrophic Lateral Sclerosis (ALS)
genetics
motor neurons degeneration
neuroimmunology
iPSc
Labelisation ENP:
2016
Centre de recherche / Institut:
Institut du Cerveau et de la Moelle épinière
Code unité de recherche:
UMRS 1127 UMR 7225
Amyotrophic Lateral Sclerosis (ALS) is the most common adult-onset motor neuron disease. Although mostly sporadic, major geneticcauses are nucleotide expansions in C9ORF72 and mutations in SOD1, TARDBP and FUS. We have shown that motor neuron deathis non-cell autonomous and that reducing mutant SOD1 in CNS microglia/peripheral macrophages slows disease progression. This suggests that in ALS, mutant gene expression in microglia/macrophages as well as the natural activation response of these cellsbecome deleterious.