A Panel of Slow-Channel Syndrome Mice Reveals a Unique Locomotor Behavioral Signature

Abstract

Muscle nicotinic acetylcholine receptor (nAChR) mutations can lead to altered channel kinetics and neuromuscular junction degeneration, a neurodegenerative disorder collectively known as slow-channel syndrome (SCS). A multivariate analysis using running wheels was used to generate activity profiles for a variety of SCS models, uncovering unique locomotor patterns for the different nAChR mutants. Particularly, the αL251T and ɛL269F mutations exhibit decreased event distance, duration, and velocity over a period of 24 hours. Our approach suggests a robust relationship between the pathophysiology of SCS and locomotor activity.

Keywords: Congenital myasthenia; acetylcholine; locomotor activity; mice; motor endplate; myalgia; neuromuscular junction (NMJ); nicotinic acetylcholine receptor (nAChR); running.