Immune-mediated myogenesis and acetylcholine receptor clustering promote a slow disease progression in ALS mouse models.

ABSTRACT:

Background

Amyotrophic lateral sclerosis (ALS) is a heterogeneous disease in terms of onset and progression rate. This may account for therapeutic clinical trial failure. Transgenic SOD1G93A mice on C57 or 129Sv background have a slow and fast disease progression rate, mimicking the variability observed in patients. Based on evidence inferring the active influence of skeletal muscle on ALS pathogenesis, we explored whether dysregulation in hindlimb skeletal muscle reflects the phenotypic difference between the two mouse models.

Methods

Ex vivo immunohistochemical, biochemical, and biomolecular methodologies, together with in vivo electrophysiology and in vitro approaches on primary cells, were used to afford a comparative and longitudinal analysis of gastrocnemius medialis b

SUBMITTER: Margotta C 

PROVIDER: S-EPMC9996869 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature