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Updated: Jun 17, 2026

Engineering and Characterization of an Optogenetic Model of the Human Neuromuscular Junction

Engineering and Characterization of an Optogenetic Model of the Human Neuromuscular Junction

Published on: April 14, 2022

Neuromuscular disease models and analysis.

Robert W Burgess¹, Gregory A Cox, Kevin L Seburn

¹The Jackson Laboratory, Bar Harbor, ME, USA.

Methods in Molecular Biology (Clifton, N.J.)

|December 17, 2009

Summary

Assessing mouse models of neuromuscular diseases requires integrated histological, anatomical, and electrophysiological methods. Understanding these complex phenotypes is crucial for developing effective treatments for conditions like amyotrophic lateral sclerosis and muscular dystrophies.

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Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

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Last Updated: Jun 17, 2026

Engineering and Characterization of an Optogenetic Model of the Human Neuromuscular Junction

Engineering and Characterization of an Optogenetic Model of the Human Neuromuscular Junction

Published on: April 14, 2022

Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles

Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles

Published on: September 25, 2015

Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

Published on: July 18, 2019

Area of Science:

Neuroscience
Pathology
Animal Models

Background:

Neuromuscular diseases impact peripheral neurons, axons, synapses, or muscle targets.
Examples include amyotrophic lateral sclerosis, Charcot-Marie-Tooth diseases, myasthenias, and muscular dystrophies.
Characterizing these complex conditions in mouse models is essential for research.

Purpose of the Study:

To describe methods for assessing neuromuscular disease models in mice.
To highlight the integrated approach needed for phenotype characterization.
To explain how inter-related phenotypes complicate test interpretation.

Main Methods:

Histological and anatomical examination of nerve and muscle.
Functional assessment using electrophysiology.
Evaluation of gross motor performance (gait, grip strength) as quality-of-life measures.

Main Results:

Integrated approaches reveal nerve and muscle defects.
Electrophysiology provides functional insights.
Motor performance tests correlate with disease onset, severity, and progression.

Conclusions:

A multi-faceted approach is necessary for accurate neuromuscular disease modeling in mice.
Interconnectedness of phenotypes requires careful interpretation of assessment data.
This chapter provides a framework for evaluating mouse models of these debilitating diseases.