Next Article in Journal
NADPH Oxidases and Mitochondria in Vascular Senescence
Next Article in Special Issue
The Neuromuscular Junction and Wide Heterogeneity of Congenital Myasthenic Syndromes
Previous Article in Journal
Fish Oil Ameliorates High-Fat Diet Induced Male Mouse Reproductive Dysfunction via Modifying the Rhythmic Expression of Testosterone Synthesis Related Genes
Previous Article in Special Issue
Neurophysiological Assessment of Abnormalities of the Neuromuscular Junction in Children
Article Menu
Issue 5 (May) cover image

Export Article

Open AccessReview
Int. J. Mol. Sci. 2018, 19(5), 1326; https://doi.org/10.3390/ijms19051326

Animal Models of the Neuromuscular Junction, Vitally Informative for Understanding Function and the Molecular Mechanisms of Congenital Myasthenic Syndromes

Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford OX3 9DS, UK
Received: 13 April 2018 / Revised: 24 April 2018 / Accepted: 25 April 2018 / Published: 29 April 2018
(This article belongs to the Special Issue The Neuromuscular Synapse in Health and Disease)
Full-Text   |   PDF [592 KB, uploaded 29 April 2018]   |  

Abstract

The neuromuscular junction is the point of contact between motor nerve and skeletal muscle, its vital role in muscle function is reliant on the precise location and function of many proteins. Congenital myasthenic syndromes (CMS) are a heterogeneous group of disorders of neuromuscular transmission with 30 or more implicated proteins. The use of animal models has been instrumental in determining the specific role of many CMS-related proteins. The mouse neuromuscular junction (NMJ) has been extensively studied in animal models of CMS due to its amenability for detailed electrophysiological and histological investigations and relative similarity to human NMJ. As well as their use to determine the precise molecular mechanisms of CMS variants, where an animal model accurately reflects the human phenotype they become useful tools for study of therapeutic interventions. Many of the animal models that have been important in deconvolving the complexities of neuromuscular transmission and revealing the molecular mechanisms of disease are highlighted. View Full-Text
Keywords: neuromuscular junction; animal model; myasthenia; congenital neuromuscular junction; animal model; myasthenia; congenital
Figures

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
SciFeed

Share & Cite This Article

MDPI and ACS Style

Webster, R.G. Animal Models of the Neuromuscular Junction, Vitally Informative for Understanding Function and the Molecular Mechanisms of Congenital Myasthenic Syndromes. Int. J. Mol. Sci. 2018, 19, 1326.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top