ALS (Amyotrophic Lateral Sclerosis) disease models and services 

ALS, also known as motor neurone disease (MND), Lou Gehrig’s disease, and Charcot disease, is a fatal motor neuron disease marked by progressive weakness and muscle wasting.

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Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurological disorder primarily impacting motor neurons in the brain and spinal cord. Early stages manifest as spasticity and muscle twitching, evolving into escalating weakness and muscle wasting as the disease progresses. Despite its heterogeneous clinical presentation, the majority of ALS patients face a life expectancy of only a few years following diagnosis. While there are therapies to address symptoms, a disease-modifying treatment with substantial efficacy remains unavailable.

ALS Disease model options

Transgenic SOD1 models

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

Transgenic TDP-43 Models

InnoSer offers services with several different transgenic tau models, which recapitulate the neurofibrilary tangle pathology of AD.

TDP-43 Seeding Models

InnoSer offers extensive preclinical research experience with TDP-43 seeds using patient-derived brain extracts.
Preclinical behavioral test for drug development

View our comprehensive range of standardized and customized behavioural tests

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
European based preclinical CRO offering LRRK2 - Parkinson's Disease mouse models for drug development

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.

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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

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