Action Potentials in Neuromuscular Disease Models

This collaborative whitepaper from iWorx Systems and Scantox showcases how action potential–based electrophysiological metrics can be used to better understand and evaluate neuromuscular dysfunction in preclinical models. By combining innovative technology with translational research expertise, this work illustrates how these tools can accelerate discovery in neurodegenerative diseases, such as Amyotrophic Lateral Sclerosis (ALS).

Electrophysiological techniques—including Compound Muscle Action Potential (CMAP), Motor Unit Number Estimation (MUNE), and repeated nerve stimulation—offer powerful insights into disease progression and treatment response. Using the SOD1 mouse, a commonly used ALS model, allows researchers to observe how motor neuron degeneration influences muscle function as the disease progresses.

The iWorx Small Animal CMAP System enables precise assessment of neuromuscular performance by recording muscle responses to nerve stimulation. Coupled with the LabScribe Action Potentials Module, researchers can perform detailed analysis of CMAPs. Calculations include, but are not limited to, latencies, amplitudes, and durations.

Through this collaboration, iWorx Systems and Scantox demonstrate how cutting-edge electrophysiological tools can enhance the sensitivity, reproducibility, and translational relevance of preclinical research.

Images, techniques, and procedures are courtesy of Aaron Fantina-Woblistin with Scantox.