Amyotrophic Lateral Sclerosis – ALS Mouse Models
Explore our advanced preclinical ALS mouse models, crucial for preclinical efficacy studies and understanding disease mechanisms
Your Preclinical Amyotrophic Lateral Sclerosis Research with InnoSer
✓ Behavioral & Cognitive Tests
✓ Smart Pathological Assessments
✓ Fast Study Initiation
✓ Tailored Study Designs
✓ Dedicated Research Co-oridnators
As a European preclinical neurology contract research organization (CRO), InnoSer provides you with a portfolio of Amyothropic Lateral Sclerosis (ALS) mouse models relevant for performing preclinical efficacy research studies. By choosing InnoSer as your partner CRO, you will work alongside our expert study directors who take a collaborative approach for your study, accommodating your study timelines and budget needs. InnoSer’s neurology expert team possesses relevant experience in working with multiple therapy types ranging from small molecules, peptides, enzymes, oligonucleotides, gene therapy (viral vectors – e.g.. AAVs) and immunotherapies (antibody/vaccine immunotherapies).
InnoSer offers multiple ALS mouse models relevant for preclinical research, including the SOD1-G93A transgenic ALS mouse model, TDP-43 transgenic mouse models and TDP-43 seeding models using recombinant or patient-derived seeds. However, as each model is unique, modelling distinct pathophysiological aspects of ALS, we recommend you discuss the most appropriate model with our neurology study directors.
Your ALS Research Starts Here.
View study timelines, recommended readouts, and example data featuring behavioral across different ALS mouse models.
InnoSer’s Available ALS Mouse Models
In Vitro Neurology Assays
Screen your lead candidate compounds using InnoSer’s in vitro neurology assays to progress to preclinical in vivo studies with confidence
SOD1-G93A Transgenic ALS Mouse Model
InnoSer offers unique behavioral research services with the SOD1-G93A transgenic mouse model.
TDP-43 Transgenic Mouse Model
InnoSer offers services with several different transgenic TDP-43 mouse models that replicate the TDP-43 proteinopathy in ALS and FTD patients.
Don’t See Your Model? We Can Help!
Our experts specialize in tailored solutions to meet your unique research needs. Let’s discuss how we can support your project.
The People Behind Your Research
Sofie Carmans, PhD
Principal Scientist Neurology
Thomas Vogels, PhD
Principal Scientist Neurology
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Translational Neuroscience: Comprehensive longitudinal profiling of the Tau[P301S] female vs male mice
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Proven Compound Susceptible Tau[P301S] Mouse Model for Preclinical Proof of Concept
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Home-Cage Behavior Discriminates Neurodegeneration Models from Healthy Aging Mice
Distinguishing pathological neurodegeneration from normal biological aging is a fundamental challenge in preclinical neuroscience. Presented at AD/PD 2023 in Gothenburg, this poster — developed in collaboration with Sylics and VU University Amsterdam — establishes a...
Your ALS Research Starts Here.
This poster provides longitudinal phenotypic characterization of female TDP-43(Q331K) mice from 4 weeks to 8 months of age.
Frequently Asked Questions
How are ALS mouse models used in preclinical research?
ALS mouse models are essential tools for evaluating the efficacy of potential therapeutics before they advance to clinical trials. Briefly, following lead compound identification, promising candidates typically undergo in vitro testing to evaluate and confirm their mechanism of action, potency, target engagement, and potential off-target effects.
Compounds that demonstrate favourable activity in vitro then typically progress onto in vivo studies, where the tolerability, efficacy, and safety of the compounds are further evaluated. In vivo studies typically involve transgenic mouse models that carry mutations that have been previously linked to be causative of human ALS pathophysiology, such as the SOD1-G93A mouse model or the TDP-43Q331K mouse model. These models, combined with translational readouts, help reproduce key aspects of human ALS disease pathophysiology, making them highly relevant for preclinical efficacy studies.
In turn, preclinical efficacy studies in ALS mouse models provide you with critical translational data to help inform clinical trial design, optimize therapeutic dosing and administration routes, and de-risk your drug development pipeline.
As a preclinical neurology CRO specialized in multiple neurodegenerative disorders, including ALS, InnoSer frequently validates new disease models. Subscribe to our newsletter to stay up to date with our preclinical ALS research.
What ALS mouse model should I choose for my research?
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.
How is motor unit degeneration assessed in preclinical ALS mouse models?
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.
Why should I choose InnoSer as my partner CRO for preclinical ALS mouse model research?
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.
info@innoserlaboratories.com