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

José G. Grajales-Reyes, Aurian García-González, José C. María-Ríos, Gary E. Grajales-Reyes, Manuel Delgado-Vélez, Carlos A. Báez-Pagán, Orestes Quesada, Christopher M. Gómez, José A. Lasalde-Dominicci

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

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 ϵL269F 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.

Original languageEnglish
Pages (from-to)341-347
Number of pages7
JournalJournal of neuromuscular diseases
Volume4
Issue number4
DOIs
StatePublished - 2017

Keywords

  • acetylcholine
  • Congenital myasthenia
  • locomotor activity
  • mice
  • motor endplate
  • myalgia
  • neuromuscular junction (NMJ)
  • nicotinic acetylcholine receptor (nAChR)
  • running

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