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

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

Author information

1
Department of Biology, University of Puerto Rico, Río Piedras Campus, San Juan, PR, USA.
2
Department of Physical Sciences, University of Puerto Rico, Río Piedras Campus, San Juan, PR, USA.
3
Department of Neurology, The University of Chicago, Chicago, IL, USA.
4
Department of Chemistry, University of Puerto Rico, Río Piedras Campus, San Juan, PR, USA.

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

PMID:
29036836
DOI:
10.3233/JND-170226

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