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    ALS (Amyotrophic Lateral Sclerosis) Mouse Models

    Explore our advanced preclinical ALS mouse models, crucial for preclinical efficacy studies and understanding disease mechanisms

    Home » Neurology CRO Services » ALS models and services

    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. 

    ALS Mouse Models Options

    SOD1-G93A Transgenic ALS Mouse Model

    InnoSer offers unique behavioural research services with several different transgenic SOD1 mouse models. 

    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. 

    TDP-43 Seeding Mouse Models

    InnoSer offers extensive preclinical research experience with TDP-43 seeds using patient-derived brain extracts.

    Learn more about the different ALS disease models by downloading a leaflet containing models, readouts, and example data. 

    ALS sample data leaflet download preclinical mouse models of ALS

    ALS motor neuron disease

    Pathology in the brain and spinal cord

    Several ALS mouse models mimic the progressive loss of motor neurons in the motor cortex, brain stem, and spinal cord. ALS also exists on a continuum with frontotemporal dementia (FTD), which also affects the frontal and temporal lobes. Virtually all patients have cytoplasmic inclusions of aggregated nuclear protein TDP-43. Patients with mutations in the SOD1 or FUS genes additionally have toxic cellular accumulation of aggregates of these proteins. Abnormal repeats in the C9orf72 gene are also associated with both ALS and FTD. ALS is therefore a heterogeneous disorder, which underlines the need for modeling the different aspects of the disease in distinct mouse models.
    brown mouse for mouse models in vivo facility Leiden
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    Risk factors: C9orf72, SOD1, FUS, and TARDBP

    Four genes are responsible for the majority of familial ALS cases, with C9orf72 accounting for 40%, SOD1 for 20%, FUS for 1–5%, and TARDBP for 1–5%. The TARDBP gene encodes the TDP-43 protein, which accumulates in motor neurons in nearly all cases of both sporadic and familial ALS, as well as in 50% of FTD cases.

    Consequently, mice expressing the human protein with these mutations serve as highly promising models for evaluating therapeutics targeted at ALS.

    The people behind the models

    Jolien Beekens, PhD

    Jolien Beekens, PhD, Neurology Study Director

    With in vitro screening methods available as a suited first step in your development process our team of experts have the experience to optimise your study design process.

    Thomas Vogels, PhD Neurology study director InnoSer

    Thomas Vogels, PhD, Neurology Study Director

    Leads an expert team of scientists with vast experience in our Neurology models to help you choose the right model and guide your optimal study design. We provide the solution to accelerating your drug development.

    AAALAC Accreditation

    InnoSer has earned the AAALAC accreditation, demonstrating our commitment to responsible animal care and use. AAALAC International is a nonprofit organization that promotes the humane treatment of animals in science through voluntary accreditation and assessment programs. Our accreditation is valid for three years, incl. 2023. Read more about the AAALAC accreditation programme here.

    AAALAC logo

    Animal Welfare

    The 3Rs impact everything from policy and regulatory change to the development and uptake of new technologies and approaches. This is why Innoser has ongoing commitment and monitoring of these processes. The steps we practice maximize our ability to replace, reduce and refine animal involvement and facilitate our commitment to these principles when it comes to research and drug development.

    Related Resources

    View our in vivo neurology models overview with sample data

    Accelerating ALS Drug Development: InnoSer’s Available In Vivo Models for Preclinical Research

    Neurology research models and services overview page