Roy Maimon, Lior Ankol, Tal Gradus Pery, Topaz Altman, Ariel Ionescu, Romana Weissova, Michael Ostrovsky, Elizabeth Tank, Gayster Alexandra, Natalia Shelestovich, Yarden Opatowsky, Amir Dori, Sami Barmada, Martin Balastik, Eran Perlson
Amyotrophic lateral sclerosis (ALS) is a fatal non-cell-autonomous neurodegenerative disease characterized by the loss of motor neurons (MNs). Mutations in CRMP4 are associated with ALS in patients, and elevated levels of CRMP4 are suggested to affect MN health in the SOD1G93A -ALS mouse model. However, the mechanism by which CRMP4 mediates toxicity in ALS MNs is poorly understood. Here, by using tissue from human patients with sporadic ALS, MNs derived from C9orf72-mutant patients, and the SOD1G93A -ALS mouse model, we demonstrate that subcellular changes in CRMP4 levels promote MN loss in ALS...
June 30, 2021: EMBO Journal