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Neuromuscular Disease Models and Analysis

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Book cover Mouse Models for Drug Discovery

Part of the book series: Methods in Molecular Biology ((MIMB,volume 1438))

Abstract

Neuromuscular diseases can affect the survival of peripheral neurons, their axons extending to peripheral targets, their synaptic connections onto those targets, or the targets themselves. Examples include motor neuron diseases such as Amyotrophic Lateral Sclerosis, peripheral neuropathies such as Charcot-Marie-Tooth diseases, myasthenias, and muscular dystrophies. Characterizing these phenotypes in mouse models requires an integrated approach, examining both the nerve and muscle histologically, anatomically, and functionally by electrophysiology. Defects observed at these levels can be related back to onset, severity, and progression, as assessed by “Quality of life measures” including tests of gross motor performance such as gait or grip strength. This chapter describes methods for assessing neuromuscular disease models in mice, and how interpretation of these tests can be complicated by the inter-relatedness of the phenotypes.

This chapter is reproduced from the previous edition. The methods and approaches described have not changed and therefore the chapter remains relevant; however, additional mouse models are emerging constantly and the lists provided should not be considered comprehensive in 2016

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  1. The Jackson Laboratory, 600 Main Street, Bar Harbor, ME, 04609, USA

    Robert W. Burgess, Gregory A. Cox & Kevin L. Seburn

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  1. Robert W. Burgess
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  2. Gregory A. Cox
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Correspondence to Robert W. Burgess .

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  1. Takeda Pharmaceutical Company, Cambridge, Massachusetts, USA

    Gabriele Proetzel

  2. The Jackson Laboratory, Bar Harbor, Maine, USA

    Michael V. Wiles

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Burgess, R.W., Cox, G.A., Seburn, K.L. (2016). Neuromuscular Disease Models and Analysis. In: Proetzel, G., Wiles, M. (eds) Mouse Models for Drug Discovery. Methods in Molecular Biology, vol 1438. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-3661-8_19

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