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

J Neuromuscul Dis. 2017;4(4):341-347. doi: 10.3233/JND-170226.


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.

MeSH terms

  • Animals
  • Disease Models, Animal
  • Gait Analysis
  • Locomotion / genetics*
  • Locomotion / physiology*
  • Male
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Movement Disorders / genetics
  • Movement Disorders / metabolism
  • Multivariate Analysis
  • Mutation*
  • Phenotype
  • Receptors, Nicotinic / genetics*
  • Receptors, Nicotinic / metabolism*
  • Species Specificity
  • Syndrome
  • Volition


  • Receptors, Nicotinic