TY - JOUR
T1 - A Panel of Slow-Channel Syndrome Mice Reveals a Unique Locomotor Behavioral Signature
AU - Grajales-Reyes, José G.
AU - García-González, Aurian
AU - María-Ríos, José C.
AU - Grajales-Reyes, Gary E.
AU - Delgado-Vélez, Manuel
AU - Báez-Pagán, Carlos A.
AU - Quesada, Orestes
AU - Gómez, Christopher M.
AU - Lasalde-Dominicci, José A.
N1 - Funding Information:
This work was supported by National Institutes of Health (NIH) Grants 1P20GM103642 (J.R. and J.A.L.D.), NIH Grant R01 NS033202-15A2 (C.M.G.), Research Initiative for Scientific Enhancement (RISE) Program Grant 5R25GM061151-16 (A.G.G., M.D.V. & OQ), the Minority Access to Research Careers (MARC) Program Grant 5T34GM 007821-38 (J.G.G.R.; G.E. G.R. & OQ) and NIGMS P20GM103642 (J.A.L.D).
Publisher Copyright:
© 2017 - IOS Press and the authors. All rights reserved.
PY - 2017
Y1 - 2017
N2 - 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.
AB - 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.
KW - acetylcholine
KW - Congenital myasthenia
KW - locomotor activity
KW - mice
KW - motor endplate
KW - myalgia
KW - neuromuscular junction (NMJ)
KW - nicotinic acetylcholine receptor (nAChR)
KW - running
UR - http://www.scopus.com/inward/record.url?scp=85041164832&partnerID=8YFLogxK
U2 - 10.3233/JND-170226
DO - 10.3233/JND-170226
M3 - Article
C2 - 29036836
AN - SCOPUS:85041164832
SN - 2214-3599
VL - 4
SP - 341
EP - 347
JO - Journal of Neuromuscular Diseases
JF - Journal of Neuromuscular Diseases
IS - 4
ER -