Article
Early nuclear phenotypes and reactive transformation in human iPSC-derived astrocytes from ALS patients with SOD1 mutations.
Glia - 1 Nov 2024
Soubannier Vincent, Chaineau Mathilde, Gursu Lale, Lépine Sarah, Kalaydjian David, Sirois Julien, Haghi Ghazal, Rouleau Guy, Durcan Thomas M, Stifani Stefano
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive death of motor neurons (MNs). Glial cells play roles in MN degeneration in ALS. More specifically, astrocytes with mutations in the ALS-associated gene Cu/Zn superoxide dismutase 1 (SOD1) promote MN death. The mechanisms by which SOD1-mutated astrocytes reduce MN survival are incompletely understood. To...
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