Oumayma Aousji, Simone Feldengut, Stefano Antonucci, Michael Schön, Tobias M Boeckers, Jakob Matschke, Christian Mawrin, Albert C Ludolph, Kelly Del Tredici, Francesco Roselli, Heiko Braak
Amyotrophic Lateral Sclerosis (ALS) is mainly characterized by the degeneration of corticospinal neurons and spinal α-motoneurons; vulnerable cells display prominent pTDP-43 inclusions. Evidence gathered from genetics, murine models, and iPSC-derived neurons point to the early involvement of synapses in the disease course and their crucial role in the pathogenic cascade. However, pathology studies, with specimens from large post-mortem cohorts, mapping the pattern of synaptic disturbances over clinical and neuropathological hallmarks of disease progression, are currently not available...
July 25, 2023: Acta Neuropathologica Communications