Modulation of the Acetylcholine Receptor Clustering Pathway Improves Neuromuscular Junction Structure and Muscle Strength in a Mouse Model of Congenital Myasthenic Syndrome

Modulation of the Acetylcholine Receptor Clustering Pathway Improves Neuromuscular Junction Structure and Muscle Strength in a Mouse Model of Congenital Myasthenic Syndrome

Introduction: Congenital myasthenic syndromes (CMS) are a diverse group of inherited neuromuscular disorders characterized by a failure of synaptic transmission at the neuromuscular junction (NMJ). CMS often present early with fatigable weakness and can be fatal through respiratory complications. Th...

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Bibliographic Details
Main Authors: Sally Spendiff, Rachel Howarth, Grace McMacken, Tracey Davey, Kaitlyn Quinlan, Emily O'Connor, Clarke Slater, Stefan Hettwer, Armin Mäder, Andreas Roos, Rita Horvath, Hanns Lochmüller
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-12-01
Series:Frontiers in Molecular Neuroscience
Subjects:
neuromuscular junction
congenital myasthenia syndromes (CMS)
therapies
neuromuscular disease (NMD)
preclinical “in vivo” study
animal model
Online Access:https://www.frontiersin.org/articles/10.3389/fnmol.2020.594220/full

Internet

https://www.frontiersin.org/articles/10.3389/fnmol.2020.594220/full

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