Choosing the right ALS mouse model depends on your research goals, target pathways, your compound’s mechanism of action (MoA), and therapeutic development strategy. For example, if your compound targets SOD1, one of the most common mutations, accounting for ~20% of familial ALS cases (5–10% of total ALS), the well-established and most frequently used SOD1-G93A may the most suitable model for your research.  

InnoSer currently performs research in transgenic ALS mouse models focusing on different aspects of ALS disease pathophysiology, including the SOD1-G93A and TDP-43Q331K. While the majority of preclinical ALS studies have historically relied on SOD1-based models, translation to the clinic has been limited, and there are still no widely used models for other genetic subtypes of ALS (e.g., C9orf72, FUS, PFN1, or TBK1). To help address this need, at InnoSer, we frequently collaborate with our partners on the co-development and validation of novel, mutation-specific ALS models to enable more precise and translational preclinical testing.  

Reach out to us to discuss the most suitable ALS mouse model for your research.  

In preclinical ALS mouse models, motor unit degeneration can be evaluated using a combination of functional (behavioral), electrophysiological, and histological readouts.  

One of the most translationally relevant methods to assess motor unit integrity is the nerve conduction study (NCS), an electromyography (EMG) tool used as a standard diagnostic and disease monitoring tool in ALS patients. In mice, electrophysiological recordings are performed on the sciatic nerve using needle electrodes. Compound muscle action potential (CMAP) measures the number and integrity of functional motor units, whereby a lower CMAP response indicates fewer functional motor units. Nerve conduction velocity (NCV) measures the speed and nerve signal propagation, serving as a proxy marker for nerve myelination. Slower NCV indicates peripheral nerve damage. 

Complementary assessments include behavioral assays (e.g., Rotarod, balance beam, CatWalk, and spontaneous behavior recorded via automated home-cage monitoring), which track progressive motor impairments.

Histological analyses (e.g., spinal cord motor neuron counts, neuromuscular junction integrity, and muscle fiber morphology) provide anatomical confirmation of functional decline.  

Contact our team to discuss which combination of functional, electrophysiological, and biomarker endpoints best captures your compound’s mechanism of action in ALS mouse models. 

As a preclinical ALS CRO, InnoSer combines scientific expertise, innovation, and tailored services to support your studies. Key benefits of partnering with InnoSer for your neurodegenerative research include: 

• Access to an innovative research neuroscience platform, including behavioural assays, including classical behavioural assays and advanced assays (such as 24/7 behavioural assessment of 115 types of spontaneous mouse behaviour in an automated home-cage without human disruption), combined with robust statistical data analysis 

• Focus on translational ALS readouts, including nerve conduction studies and biomarker plasma NfL quantification  

• Flexible study start-up timelines to fit your budget and research needs 

• Collaborative study design and hands-on support from dedicated neurology study directors 

• Co-development of new ALS models tailored to your specific research requirements 

Contact us to discuss how partnering with InnoSer can accelerate your preclinical ALS program now.