Exciting Complexity: The Role of Motor Circuit Elements in ALS Pathophysiology.

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a fatal disease, characterized by the degeneration of both upper and lower motor neurons. Despite decades of research, we still to date lack a cure or disease modifying treatment, emphasizing the need for a much-improved insight into disease mechanisms and cell type vulnerability. Altered neuronal excitability is a common phenomenon reported in ALS patients, as well as in animal models of the disease, but the cellular and circuit processes involved, as well as the causal relevance of those observations to molecular alterations and final cell death, remain poorly understood. Here, we review evidence from clinical studies, cell type-specific electrophysiology, genetic manipulations and molecular characterizations in animal models and culture experiments

SUBMITTER: Gunes ZI 

PROVIDER: S-EPMC7311855 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature