Patterns of synaptic loss in human amyotrophic lateral sclerosis spinal cord: a clinicopathological study.

ABSTRACT: 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. Thus, the appearance and progression of synaptic degeneration in human ALS patients are currently not known, preventing a full validation of the murine and in vitro models. Here, we investigated the loss of

SUBMITTER: Aousji O 

PROVIDER: S-EPMC10367350 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature